Wegener's Granulomatosis: Evolving Concepts in Treatment

Joseph P. Lynch; Eric White; Henry Tazelaar; Carol A. Langford

doi:10.1055/s-2004-836143

Seminars in Respiratory and Critical Care Medicine, Table of Contents

Semin Respir Crit Care Med 2004; 25(5): 491-521
DOI: 10.1055/s-2004-836143

Wegener's Granulomatosis: Evolving Concepts in Treatment

Joseph P. Lynch¹ III , Eric White² , Henry Tazelaar³ , Carol A. Langford⁴

¹Department of Medicine, Division of Pulmonary, Critical Care Medicine, and Hospitalists, The David Geffen School of Medicine at UCLA, Los Angeles, California
²Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Michigan Medical Center, Ann Arbor, Michigan
³Department of Laboratory Medicine and Pathology, Mayo Clinic Foundation, Rochester, Minnesota
⁴Center for Vasculitis Care and Research, Department of Rheumatic and Immunologic Diseases, The Cleveland Clinic Foundation, Cleveland, Ohio

Abstract

Wegener's granulomatosis (WG), the most common of the pulmonary granulomatous vasculitides, typically involves the upper respiratory tract, lower respiratory tract (bronchi and lung), and kidney, with varying degrees of disseminated vasculitis. Major histological features include a necrotizing vasculitis involving small vessels, extensive “geographic” necrosis, and granulomatous inflammation. Clinical manifestations of WG are protean; virtually any organ can be involved. Further, the spectrum and severity of the disease is heterogeneous, ranging from indolent disease involving only one site to fulminant, multiorgan vasculitis leading to death. The pathogenesis of WG has not been elucidated, but both cellular and humoral components are involved. Circulating antineutrophil cytoplasmic antibodies (cANCA) likely play a role in the pathogenesis and often correlate with activity of the disease. Treatment strategies are evolving. Cyclophosphamide (CYC) plus corticosteroids (CS) is the mainstay of therapy for generalized, multisystemic WG. Historically, the combination of CYC plus CS was used for a minimum of 12 months, but concern about late toxicities associated with CYC has led to novel treatment approaches. Currently, short-course (3-6 months) induction treatment with CYC plus CS, followed by maintenance therapy with less toxic agents (e.g., methotrexate, azathioprine) is recommended. Further, recent studies suggest that methotrexate combined with CS may be adequate for limited, non-life threatening WG. The role of other immunomodulatory agents (including trimethoprim-sulfamethoxazole) is also explored.

KEYWORDS

Wegener's granulomatosis - granulomatous vasculitis - pulmonary vasculitis - capillaritis - antineutrophil cytoplasmic antibodies - geographic necrosis

Full Text

References

REFERENCES

1 Fauci A S, Haynes B F, Katz P, Wolff S M. Wegener's granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med. 1983; 98 76-85
2 Hoffman G S, Kerr G S, Leavitt R Y, Hallahan C W. Wegener's granulomatosis: an analysis of 158 patients. Ann Intern Med. 1992; 116 488-498
3 Luqmani R A, Bacon P A, Beaman M et al.. Classical versus nonrenal Wegener's granulomatosis. Q J Med. 1994; 87 161-167
4 Reinhold-Keller E, Beuge N, Latza U et al.. An interdisciplinary approach to the care of patients with Wegener's granulomatosis: long-term outcome in 155 patients. Arthritis Rheum. 2000; 43 1021-1032
5 Leavitt R Y, Fauci A S, Bloch D A et al.. The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis. Arthritis Rheum. 1990; 33 1101-1107
6 Langford C A, Hoffman G S. Rare diseases, III: Wegener's granulomatosis. Thorax. 1999; 54 629-637
7 Abdou N I, Kullman G J, Hoffman G S et al.. Wegener's granulomatosis: survey of 701 patients in North America: changes in outcome in the 1990s. J Rheumatol. 2002; 29 309-316
8 Lynch J PI, Hoffman G S. Wegener's granulomatosis: controversies and current concepts. Compr Ther. 1998; 24 421-440
9 Mark E J, Matsubara O, Tan-Liu N S, Fienberg R. The pulmonary biopsy in the early diagnosis of Wegener's (pathergic) granulomatosis: a study based on 35 open lung biopsies. Hum Pathol. 1988; 19 1065-1071
10 Travis W D, Hoffman G S, Leavitt R Y, Pass H I, Fauci A S. Surgical pathology of the lung in Wegener's granulomatosis: review of 87 open lung biopsies from 67 patients. Am J Surg Pathol. 1991; 15 315-333
11 Devaney K, Travis W, Hoffman G, Leavitt R, Lebovics R, Fauci A. Interpretation of head and neck biopsies in Wegener's granulomatosis: a pathologic study of 126 biopsis in 70 patients. Am J Surg Pathol. 1990; 14 555-564
12 Cotch M F, Hoffman G S, Yerg D E, Kaufman G I, Targonski P, Kaslow R A. The epidemiology of Wegener's granulomatosis: estimates of the five-year period prevalence, annual mortality, and geographic disease distribution from population-based data sources. Arthritis Rheum. 1996; 39 87-92
13 Watts R A, Gonzales-Gay M, Garcia-Porrua C, Lane S E, Bentham G, Scott D G. ANCA-associated vasculitis in two European regions. Clin Exp Immunol. 2000; 120 (suppl 1) 60
14 Koldingsnes W, Nossent H. Epidemiology of Wegener's granulomatosis in northern Norway. Arthritis Rheum. 2000; 43 2481-2487
15 Rottem M, Fauci A S, Hallahan C W et al.. Wegener granulomatosis in children and adolescents: clinical presentation and outcome. J Pediatr. 1993; 122 26-31
16 Stegmayr B G, Gothefors L, Malmer B, Muller Wiefel D E, Nilsson K, Sundelin B. Wegener granulomatosis in children and young adults: a case study of ten patients. Pediatr Nephrol. 2000; 14 208-213
17 Yalcindag A, Sundel R. Vasculitis in childhood. Curr Opin Rheumatol. 2001; 13 422-427
18 Wardyn K A, Ycinska K, Matuszkiewicz-Rowinska J, Chipczynska M. Pseudotumour orbitae as the initial manifestation in Wegener's granulomatosis in a 7-year-old girl. Clin Rheumatol. 2003; 22 472-474
19 Belostotsky V M, Shah V, Dillon M J. Clinical features in 17 paediatric patients with Wegener granulomatosis. Pediatr Nephrol. 2002; 17 754-761
20 Roberti I, Reisman L, Churg J. Vasculitis in childhood. Pediatr Nephrol. 1993; 7 479-489
21 Klinger H. Grenzformen der Periarteritis Nodosa. Pathology. 1931; 42 455-480
22 Wegener F. Uber generarlisisierte, seeptische Gefaesserkrankungen. Verh Dtsch Ges Pathol. 1936; 29 202-210
23 Godman G. J. C. Wegener's granulomatosis: pathology and review of the literature. Arch Pathol. 1954; 58 533-553
24 Carrington C B, Liebow A. Limited forms of angiitis and granulomatosis of Wegener's type. Am J Med. 1966; 41 497-527
25 Cassan S M, Coles D T, Harrison Jr E G. The concept of limited forms of Wegener's granulomatosis. Am J Med. 1970; 49 366-379
26 Israel H L, Patchefsky A S, Saldana M J. Wegener's granulomatosis, lymphomatoid granulomatosis, and benign lymphocytic angiitis and granulomatosis of lung: recognition and treatment. Ann Intern Med. 1977; 87 691-699
27 DeRemee R A, McDonald T J, Harrison Jr E G, Coles D T. Wegener's granulomatosis: anatomic correlates, a proposed classification. Mayo Clin Proc. 1976; 51 777-781
28 Reinhold-Keller E, De Groot K, Rudert H, Nolle B, Heller M, Gross W L. Response to trimethoprim/sulfamethoxazole in Wegener's granulomatosis depends on the phase of disease. Q J Med. 1996; 89 15-23
29 Ahmad I, Lee W C, Nagendran V, Wilson F, Shortridge R T. Localised Wegener's granulomatosis in otolaryngology: a review of six cases. ORL J Otorhinolaryngol Relat Spec. 2000; 62 149-155
30 Fauci A, Wolff S. Wegener's granulomatosis: studies in 18 patients and a review of the literature. Medicine. 1973; 52 535-561
31 Jennette J C, Falk R J, Andrassy K et al.. Nomenclature of systemic vasculitides: proposal of an international consensus conference. Arthritis Rheum. 1994; 37 187-192
32 Jayne D R, Rasmussen N. Treatment of antineutrophil cytoplasm autoantibody-associated systemic vasculitis: initiatives of the European Community Systemic Vasculitis Clinical Trials Study Group. Mayo Clin Proc. 1997; 72 737-747
33 Rao J K, Weinberger M, Oddone E Z, Allen N B, Landsman P, Feussner J R. The role of antineutrophil cytoplasmic antibody (c-ANCA) testing in the diagnosis of Wegener granulomatosis: a literature review and meta- analysis. Ann Intern Med. 1995; 123 925-932
34 Jayne D R, Gaskin G, Pusey C D, Lockwood C M. ANCA and predicting relapse in systemic vasculitis. Q J Med. 1995; 88 127-133
35 Nolle B, Specks U, Ludemann J, Rohrbach M S, DeRemee R A, Gross W L. Anticytoplasmic autoantibodies: their immunodiagnostic value in Wegener granulomatosis. Ann Intern Med. 1989; 111 28-40
36 Hoffman G S, Specks U. Antineutrophil cytoplasmic antibodies. Arthritis Rheum. 1998; 41 1521-1537
37 Gaudin P B, Askin F B, Falk R J, Jennette J C. The pathologic spectrum of pulmonary lesions in patients with anti-neutrophil cytoplasmic autoantibodies specific for anti-proteinase 3 and anti-myeloperoxidase. Am J Clin Pathol. 1995; 104 7-16
38 Gaskin G, Savage C O, Ryan J J et al.. Anti-neutrophil cytoplasmic antibodies and disease activity during long-term follow-up of 70 patients with systemic vasculitis. Nephrol Dial Transplant. 1991; 6 689-694
39 Hauschild S, Schmitt W H, Csernok E, Flesch B K, Rautmann A, Gross W L. ANCA in systemic vasculitides, collagen vascular diseases, rheumatic disorders and inflammatory bowel diseases. Adv Exp Med Biol. 1993; 336 245-251
40 Matteson E L, Gold K N, Bloch D A, Hunder G G. Long-term survival of patients with Wegener's granulomatosis from the American College of Rheumatology Wegener's Granulomatosis Classification Criteria Cohort. Am J Med. 1996; 101 129-134
41 McDonald T J, Neel III H B, DeRemee R A. Wegener's granulomatosis of the subglottis and the upper portion of the trachea. Ann Otol Rhinol Laryngol. 1982; 91(6 pt 1) 588-592
42 Rasmussen N. Management of the ear, nose, and throat manifestations of Wegener granulomatosis: an otorhinolaryngologist's perspective. Curr Opin Rheumatol. 2001; 13 3-11
43 Gubbels S P, Barkhuizen A, Hwang P H. Head and neck manifestations of Wegener's granulomatosis. Otolaryngol Clin North Am. 2003; 36 685-705
44 Yang C, Talbot J M, Hwang P H. Bony abnormalities of the paranasal sinuses in patients with Wegener's granulomatosis. Am J Rhinol. 2001; 15 121-125
45 Muhle C, Reinhold-Keller E, Richter C et al.. MRI of the nasal cavity, the paranasal sinuses and orbits in Wegener's granulomatosis. Eur Radiol. 1997; 7 566-570
46 Lloyd G, Lund V J, Beale T, Howard D. Rhinologic changes in Wegener's granulomatosis. J Laryngol Otol. 2002; 116 565-569
47 Haynes B F, Fishman M L, Fauci A S, Wolff S M. The ocular manifestations of Wegener's granulomatosis: fifteen years experience and review of the literature. Am J Med. 1977; 63 131-141
48 Woo T L, Francis I C, Wilcsek G A, Coroneo M T, McNab A A, Sullivan T J. Australasian orbital and adnexal Wegener's granulomatosis. Ophthalmology. 2001; 108 1535-1543
49 Kwan A S, Rose G E. Orbital Wegener's granuloma resulting from direct extension of nasal disease through a surgical rhinostomy. Br J Ophthalmol. 1998; 82 198
50 McDonald T J, DeRemee R A. Wegener's granulomatosis. Laryngoscope. 1983; 93 220-231
51 McCaffrey T V, McDonald T J, Facer G W, DeRemee R A. Otologic manifestations of Wegener's granulomatosis. Otolaryngol Head Neck Surg. 1980; 88 586-593
52 Jacob S E, Martin L K, Kerdel F A. Cutaneous Wegener's granulomatosis (malignant pyoderma) in a patient with Crohn's disease. Int J Dermatol. 2003; 42 896-898
53 Bibas A, Fahy C, Sneddon L, Bowdler D. Facial paralysis in Wegener's granulomatosis of the middle ear. J Laryngol Otol. 2001; 115 304-306
54 Bucolo S, Torre V, Montemagno A, Beatrice F. Wegener's granulomatosis presenting with otologic and neurologic symptoms: clinical and pathological correlations. J Oral Pathol Med. 2003; 32 438-440
55 Nikolaou A C, Vlachtsis K C, Daniilidis M A, Petridis D G, Daniilidis I C. Wegener's granulomatosis presenting with bilateral facial nerve palsy. Eur Arch Otorhinolaryngol. 2001; 258 198-202
56 Dagum P, Roberson Jr J B. Otologic Wegener's granulomatosis with facial nerve palsy. Ann Otol Rhinol Laryngol. 1998; 107 555-559
57 Specks U, Colby T V, Olsen K D, DeRemee R A. Salivary gland involvement in Wegener's granulomatosis. Arch Otolaryngol Head Neck Surg. 1991; 117 218-223
58 Ah-See K W, McLaren K, Maran A G. Wegener's granulomatosis presenting as major salivary gland enlargement. J Laryngol Otol. 1996; 110 691-693
59 Murty G E, Mains B T, Bennett M K. Salivary gland involvement in Wegener's granulomatosis. J Laryngol Otol. 1990; 104 259-261
60 Benson-Mitchell R, Tolley N, Croft C B, Roberts D. Wegener's granuloma-presenting as a unilateral parotid swelling. J Laryngol Otol. 1994; 108 431-432
61 Liu S Y, Vlantis A C, Lee W C. Bilateral parotid and submandibular gland enlargement: rare features of Wegener's granulomatosis. J Laryngol Otol. 2003; 117 148-150
62 Congdon D, Sherris D A, Specks U, McDonald T. Long-term follow-up of repair of external nasal deformities in patients with Wegener's granulomatosis. Laryngoscope. 2002; 112 731-737
63 Knight J M, Hayduk M J, Summerlin D J, Mirowski G W. “Strawberry” gingival hyperplasia: a pathognomonic mucocutaneous finding in Wegener granulomatosis. Arch Dermatol. 2000; 136 171-173
64 Jennings C R, Jones N S, Dugar J, Powell R J, Lowe J. Wegener's granulomatosis: a review of diagnosis and treatment in 53 subjects. Rhinology. 1998; 36 188-191
65 Del Buono E A, Flint A. Diagnostic usefulness of nasal biopsy in Wegener's granulomatosis. Hum Pathol. 1991; 22 107-110
66 Takwoingi Y M, Dempster J H. Wegener's granulomatosis: an analysis of 33 patients seen over a 10-year period. Clin Otolaryngol. 2003; 28 187-194
67 Murray A, McGarry G W. The clinical value of septal perforation biopsy. Clin Otolaryngol. 2000; 25 107-109
68 O'Devaney K, Ferlito A, Hunter B C, Devaney S L, Rinaldo A. Wegener's granulomatosis of the head and neck. Ann Otol Rhinol Laryngol. 1998; 107(5 pt 1) 439-445
69 Matsubara O, Yoshimura N, Doi Y, Tamura A, Mark E J. Nasal biopsy in the early diagnosis of Wegener's (pathergic) granulomatosis: significance of palisading granuloma and leukocytoclastic vasculitis. Virchows Arch. 1996; 428 13-19
70 Newman N J, Slamovits T L, Friedland S, Wilson W B. Neuro-ophthalmic manifestations of meningocerebral inflammation from the limited form of Wegener's granulomatosis. Am J Ophthalmol. 1995; 120 613-621
71 Bullen C L, Liesegang T J, McDonald T J, DeRemee R A. Ocular complications of Wegener's granulomatosis. Ophthalmology. 1983; 90 279-290
72 Harper S L, Letko E, Samson C M et al.. Wegener's granulomatosis: the relationship between ocular and systemic disease. J Rheumatol. 2001; 28 1025-1032
73 Valmaggia C, Neuweiler J. Orbital involvement as the first manifestation in classic Wegener's granulomatosis. Orbit. 2001; 20 231-237
74 Lamprecht P, Reinhold-Keller E, Gross W L, Reuter M. Clinical images: orbital granuloma and subglottic tracheal stenosis in Wegener's granulomatosis. Arthritis Rheum. 2000; 43 1654
75 Niskopoulou M, Du Toit N. Optic neuritis as a feature of Wegener's granulomatosis. Eye. 2002; 16 320-321
76 Messmer E M, Foster C S. Vasculitic peripheral ulcerative keratitis. Surv Ophthalmol. 1999; 43 379-396
77 Blum M, Andrassy K, Adler D, Hartmann M, Volcker H E. Early experience with intravenous immunoglobulin treatment in Wegener's granulomatosis with ocular involvement. Graefes Arch Clin Exp Ophthalmol. 1997; 235 599-602
78 Hardwig P W, Bartley G B, Garrity J A. Surgical management of nasolacrimal duct obstruction in patients with Wegener's granulomatosis. Ophthalmology. 1992; 99 133-139
79 Perry S R, Rootman J, White V A. The clinical and pathologic constellation of Wegener granulomatosis of the orbit. Ophthalmology. 1997; 104 683-694
80 Kalina P H, Lie J T, Campbell R J, Garrity J A. Diagnostic value and limitations of orbital biopsy in Wegener's granulomatosis. Ophthalmology. 1992; 99 120-124
81 Lebovics R S, Hoffman G S, Leavitt R Y et al.. The management of subglottic stenosis in patients with Wegener's granulomatosis. Laryngoscope. 1992; 102(12 pt 1) 1341-1345
82 Langford C, Sneller M, Hallahan C, Hoffman G. Clinical features and therapeutic management of subglottic stenosis in patients with Wegener's granulomatosis. Arthritis Rheum. 1996; 39 1754-1760
83 Daum T, Specks U, Colby T, Edell E. Tracheobronchial involvement in Wegener's granulomatosis. Am J Respir Crit Care Med. 1995; 151 522-526
84 Cordier J F, Valeyre D, Guillevin L, Loire R, Brechot J M. Pulmonary Wegener's granulomatosis: a clinical and imaging study of 77 cases. Chest. 1990; 97 906-912
85 Gluth M B, Shinners P A, Kasperbauer J L. Subglottic stenosis associated with Wegener's granulomatosis. Laryngoscope. 2003; 113 1304-1307
86 Screaton N J, Sivasothy P, Flower C D, Lockwood C M. Tracheal involvement in Wegener's granulomatosis: evaluation using spiral CT. Clin Radiol. 1998; 53 809-815
86a Lynch III J P, Quint L E. Tracheobronchial and esophageal manifestations of systemic diseases. In: Cummings CE Otolaryngology Head and Neck Surgery. 3rd ed. St. Louis; Mosby-Year Book 1998: 2343-2367
87 Sheehan R E, Flint J D, Muller N L. Computed tomography features of the thoracic manifestations of Wegener granulomatosis. J Thorac Imaging. 2003; 18 34-41
88 Quint L E, Whyte R I, Kazerooni E A et al.. Stenosis of the central airways: evaluation by using helical CT with multiplanar reconstructions. Radiology. 1995; 194 871-877
89 Summers R M, Aggarwal N R, Sneller M C et al.. CT virtual bronchoscopy of the central airways in patients with Wegener's granulomatosis. Chest. 2002; 121 242-250
90 Maskell G F, Lockwood C M, Flower C D. Computed tomography of the lung in Wegener's granulomatosis. Clin Radiol. 1993; 48 377-380
91 Gotway M B, Golden J A, LaBerge J M et al.. Benign tracheobronchial stenoses: changes in short-term and long-term pulmonary function testing after expandable metallic stent placement. J Comput Assist Tomogr. 2002; 26 564-572
92 Utzig M J, Warzelhan J, Wertzel H, Berwanger I, Hasse J. Role of thoracic surgery and interventional bronchoscopy in Wegener's granulomatosis. Ann Thorac Surg. 2002; 74 1948-1952
93 Hoffman G S, Thomas-Golbanov C K, Chan J, Akst L M, Eliachar I. Treatment of subglottic stenosis, due to Wegener's granulomatosis, with intralesional corticosteroids and dilation. J Rheumatol. 2003; 30 1017-1021
94 Rosenberg D M, Weinberger S E, Fulmer J D, Flye M W, Fauci A S, Crystal R G. Functional correlates of lung involvement in Wegener's granulomatosis: use of pulmonary function tests in staging and follow-up. Am J Med. 1980; 69 387-394
95 Shin M S, Young K R, Ho K J. Wegener's granulomatosis upper respiratory tract and pulmonary radiographic manifestations in 30 cases with pathogenetic consideration. Clin Imaging. 1998; 22 99-104
96 Aberle D R, Gamsu G, Lynch D. Thoracic manifestations of Wegener granulomatosis: diagnosis and course. Radiology. 1990; 174(3 pt 1) 703-709
97 Papiris S, Manoussakis M, Drosos A, Knotogiannis D. Imaging of thoracic Wegener's granulomatosis: the computed tomographic appearance. Am J Med. 1992; 93 529-536
98 Komocsi A, Reuter M, Heller M, Murakozi H, Gross W L, Schnabel A. Active disease and residual damage in treated Wegener's granulomatosis: an observational study using pulmonary high-resolution computed tomography. Eur Radiol. 2003; 13 36-42
99 Lynch III J P, Leatherman J W. Alveolar hemorrhage syndromes. In: Fishman AE Pulmonary Diseases and Disorders. 3rd ed. New York; McGraw-Hill 1997: 1193-1210
100 Travis W D, Colby T V, Lombard C, Carpenter H A. A clinicopathologic study of 34 cases of diffuse pulmonary hemorrhage with lung biopsy confirmation. Am J Surg Pathol. 1990; 14 1112-1125
100a Lynch I J P, Matteson E, McCune J W. Wegener's granulomatosis: evolving concepts. Medical Rounds. 1989; 2 67-80
101 George T, Cash J, Farver G et al.. Mediastinal mass and hilar adenopathy: rare thoracic manifestations of Wegener's granulomatosis. Arthritis Rheum. 1997; 40 1992-1997
102 Lee K S, Kim T S, Fujimoto K et al.. Thoracic manifestation of Wegener's granulomatosis: CT findings in 30 patients. Eur Radiol. 2003; 13 43-51
102a Lynch III J P, Raghu G. Major disease syndromes of unknown etiology. In: Baum G, Crapo J Textbook of Pulmonary Disease. 6th ed. Boston; Little, Brown 1998: 431-476
103 Reuter M, Schnabel A, Wesner F et al.. Pulmonary Wegener's granulomatosis: correlation between high-resolution CT findings and clinical scoring of disease activity. Chest. 1998; 114 500-506
104 Kuhlman J E, Hruban R H, Fishman E K. Wegener granulomatosis: CT features of parenchymal lung disease. J Comput Assist Tomogr. 1991; 15 948-952
105 Attali P, Begum R, Ban Romdhane H, Valeyre D, Guillevin L, Brauner M W. Pulmonary Wegener's granulomatosis: changes at follow-up CT. Eur Radiol. 1998; 8 1009-1013
106 Beggs A D, Hain S F. F-18 FDG-positron emission tomographic scanning and Wegener's granulomatosis. Clin Nucl Med. 2002; 27 705-706
107 Uner A, Rozum-Slota B, Katzenstein A. Bronchiolitis obliterans organizing pneumonia (BOOP)-like variant of Wegener's granulomatosis: a clinicopathologic study of 16 cases. Am J Surg Pathol. 1996; 20 794-801
108 Katzenstein A L, Locke W K. Solitary lung lesions in Wegener's granulomatosis: pathologic findings and clinical significance in 25 cases. Am J Surg Pathol. 1995; 19 545-552
109 Yousem S. Bronchocentric injury in Wegener's granulomatosis: a clinicopathologic study of 16 cases. Am J Surg Pathol. 1991; 20 794-801
110 Mark E J, Flieder D B, Matsubara O. Treated Wegener's granulomatosis: distinctive pathological findings in the lungs of 20 patients and what they tell us about the natural history of the disease. Hum Pathol. 1997; 28 450-458
111 Lombard C, Duncan S, Rizk N, Colby T. The diagnosis of Wegener's granulomatosis from transbronchial lung biopsy specimens. Hum Pathol. 1990; 21 838-842
112 Michael C W, Flint A. The cytologic features of Wegener's granulomatosis. Am J Clin Pathol. 1998; 110 10-15
113 Hoffman G S, Sechler J M, Gallin J I et al.. Bronchoalveolar lavage analysis in Wegener's granulomatosis: a method to study disease pathogenesis. Am Rev Respir Dis. 1991; 143 401-407
114 Schnabel A, Csernok E, Braun J, Gross W L. Activation of neutrophils, eosinophils, and lymphocytes in the lower respiratory tract in Wegener's granulomatosis. Am J Respir Crit Care Med. 2000; 161(2 pt 1) 399-405
115 Baltaro R J, Hoffman G S, Sechler J M et al.. Immunoglobulin G antineutrophil cytoplasmic antibodies are produced in the respiratory tract of patients with Wegener's granulomatosis. Am Rev Respir Dis. 1991; 143 275-278
116 Carruthers D M, Connor S, Howie A J et al.. Percutaneous image-guided biopsy of lung nodules in the assessment of disease activity in Wegener's granulomatosis. Rheumatology (Oxford). 2000; 39 776-782
117 Haworth S J, Savage C OS, Carr D et al.. Lung haemorrhage in patients with Wegener's granulomatosis and microscopic polyarteritis. BMJ. 1985; 290 1775-1778
118 Pinching A, Lockwood C, Pussell B et al.. Wegener's granulomatosis: observations on 18 patients with severe renal disease. QJM. 1983; 52 435-460
119 Brandwein S, Esdaile J, Danoff D, Tannenbaum H. Wegener's granulomatosis: clinical features and outcome in 13 patients. Arch Intern Med. 1983; 143 476-479
120 Leatherman J W. Autoimmune diffuse alveolar hemorrhage. Clin Pulm Med. 1994; 1 356-364
121 ten Berge I J, Wilmink J M, Meyer C J et al.. Clinical and immunological follow-up of patients with severe renal disease in Wegener's granulomatosis. Am J Nephrol. 1985; 5 21-29
122 Aasarod K, Bostad L, Hammerstrom J, Jorstad S, Iversen B M. Renal histopathology and clinical course in 94 patients with Wegener's granulomatosis. Nephrol Dial Transplant. 2001; 16 953-960
123 Horn R G, Fauci A S, Rosenthal A S, Wolff S M. Renal biopsy pathology in Wegener's granulomatosis. Am J Pathol. 1974; 74 423-440
124 Ronco P, Verroust P, Mignon F et al.. Immunopathological studies of polyarteritis nodosa and Wegener's granulomatosis: a report of 43 patients with 51 renal biopsies. Q J Med. 1983; 52 212-223
125 Kuross S, Davin T, Kjellstrand C M. Wegener's granulomatosis with severe renal failure: clinical course and results of dialysis and transplantation. Clin Nephrol. 1981; 16 172-180
126 Mekhail T M, Hoffman G S. Long-term outcome of Wegener's granulomatosis in patients with renal disease requiring dialysis. J Rheumatol. 2000; 27 1237-1240
127 Andrassy K, Erb A, Koderisch J, Waldherr R, Ritz E. Wegener's granulomatosis with renal involvement: patient survival and correlations between initial renal function, renal histology, therapy and renal outcome. Clin Nephrol. 1991; 35 139-147
128 Aasarod K, Iversen B M, Hammerstrom J, Bostad L, Vatten L, Jorstad S. Wegener's granulomatosis: clinical course in 108 patients with renal involvement. Nephrol Dial Transplant. 2000; 15 611-618
129 Bajema I M, Hagen E C, Hermans J et al.. Kidney biopsy as a predictor for renal outcome in ANCA-associated necrotizing glomerulonephritis. Kidney Int. 1999; 56 1751-1758
130 Hensle T W, Mitchell M E, Crooks K K, Robinson D. Urologic manifestations of Wegener granulomatosis. Urology. 1978; 12 553-556
131 Huong D L, Papo T, Piette J C et al.. Urogenital manifestations of Wegener granulomatosis. Medicine (Baltimore). 1995; 74 152-161
132 Baker S B, Robinson D R. Unusual renal manifestations of Wegener's granulomatosis: report of two cases. Am J Med. 1978; 64 883-889
133 Ruiz Carazo E, Medina Benitez A, Lopez Milena G, Rabaza Espigares J, Leon L, Marquez B. Multiple renal masses as initial manifestation of Wegener's granulomatosis. AJR Am J Roentgenol. 2001; 176 116-118
134 Villa-Forte A, Hoffman G S. Wegener's granulomatosis presenting with a renal mass. J Rheumatol. 1999; 26 457-458
135 Gunnarsson R, Omdal R, Kjellevold K H, Ellingsen C L. Wegener's granulomatosis of the prostate gland. Rheumatol Int 2003 24: 120-122
136 Nishino H, Rubino F, DeRemee R, Swanson J, Parisi J. Neurological involvement in Wegener's granulomatosis: an analysis of 324 consecutive patients at the Mayo Clinic. Ann Neurol. 1993; 33 4-90
137 de Groot K, Schmidt D K, Arlt A C, Gross W L, Reinhold-Keller E. Standardized neurologic evaluations of 128 patients with Wegener granulomatosis. Arch Neurol. 2001; 58 1215-1221
138 Drachman D A. Neurological complications of Wegener's granulomatosis. Arch Neurol. 1963; 8 145-155
139 Murphy J M, Gomez-Anson B, Gillard J H et al.. Wegener granulomatosis: MR imaging findings in brain and meninges. Radiology. 1999; 213 794-799
140 Weinberger L M, Cohen M L, Remler B F, Naheedy M H, Leigh R J. Intracranial Wegener's granulomatosis. Neurology. 1993; 43 1831-1834
141 Miller K S, Miller J M. Wegener's granulomatosis presenting as a primary seizure disorder with brain lesions demonstrated by magnetic resonance imaging. Chest. 1993; 103 316-318
142 Thajeb P, Tsai J J. Cerebral and oculorhinal manifestations of a limited form of Wegener's granulomatosis with c-ANCA-associated vasculitis. J Neuroimaging. 2001; 11 59-63
143 Berlis A, Petschner F, Botefur I C, Spreer J. Wegener granuloma in the fourth ventricle. AJNR Am J Neuroradiol. 2003; 24 523-525
144 Czarnecki E J, Spickler E M. MR demonstration of Wegener granulomatosis of the infundibulum, a cause of diabetes insipidus. AJNR Am J Neuroradiol. 1995; 16(suppl 4) 968-970
145 Garovic V D, Clarke B L, Chilson T S, Specks U. Diabetes insipidus and anterior pituitary insufficiency as presenting features of Wegener's granulomatosis. Am J Kidney Dis. 2001; 37 E5
146 Katzman G L, Langford C A, Sneller M C, Koby M, Patronas N J. Pituitary involvement by Wegener's granulomatosis: a report of two cases. AJNR Am J Neuroradiol. 1999; 20 519-523
147 Woywodt A, Knoblauch H, Kettritz R, Schneider W, Gobel U. Sudden death and Wegener's granulomatosis of the pituitary. Scand J Rheumatol. 2000; 29 264-266
148 Spranger M, Schwab S, Meinck H M et al.. Meningeal involvement in Wegener's granulomatosis confirmed and monitored by positive circulating antineutrophil cytoplasm in cerebrospinal fluid. Neurology. 1997; 48 263-265
149 Mattioli F, Capra R, Rovaris M et al.. Frequency and patterns of subclinical cognitive impairment in patients with ANCA-associated small vessel vasculitides. J Neurol Sci. 2002; 195 161-166
150 Specks U, Moder K G, McDonald T J. Meningeal involvement in Wegener granulomatosis. Mayo Clin Proc. 2000; 75 856-859
151 Mentzel H J, Neumann T, Fitzek C, Sauner D, Reichenbach J R, Kaiser W A. MR imaging in Wegener granulomatosis of the spinal cord. AJNR Am J Neuroradiol. 2003; 24 18-21
152 Cheng T M, O'Neill B P, Scheithauer B W, Piepgras D G. Chronic meningitis: the role of meningeal or cortical biopsy. Neurosurgery. 1994; 34 590-595 , discussion 596
153 Asmus R, Koltze H, Muhle C et al.. MRI of the head in Wegener's granulomatosis. Adv Exp Med Biol. 1993; 336 319-321
154 Provenzale J M, Allen N B. Wegener granulomatosis: CT and MR findings. AJNR Am J Neuroradiol. 1996; 17 785-792
155 Riccieri V, Spadaro A, Parisi G et al.. Imaging evidence of successful multiple immunosuppressive treatment of cerebral involvement in Wegener's granulomatosis. Clin Exp Rheumatol. 2002; 20 578-579
156 Albayram S, Kizilkilie O, Adaletli I, Erdogan N, Kocer N, Islak C. MR imaging findings of spinal dural involvement with Wegener granulomatosis. AJNR Am J Neuroradiol. 2002; 23 1603-1606
157 Daoud M S, Gibson L E, DeRemee R A, Specks U, el-Azhary R A, Su W P. Cutaneous Wegener's granulomatosis: clinical, histopathologic, and immunopathologic features of thirty patients. J Am Acad Dermatol. 1994; 31 605-612
158 Frances C, Du L T, Piette J C et al.. Wegener's granulomatosis: dermatological manifestations in 75 cases with clinicopathologic correlation. Arch Dermatol. 1994; 130 861-867
159 Norris M, Tomecki K, Bergfeld W, Wilke W. Cutaneous Wegener's granulomatosis: report of a case and review of the literature. Cleve Clin J Med. 1988; 55 181-184
160 Hu C H, O'Loughlin S, Winkelmann R K. Cutaneous manifestations of Wegener granulomatosis. Arch Dermatol. 1977; 113 175-182
161 Szocs H I, Torma K, Petrovicz E et al.. Wegener's granulomatosis presenting as pyoderma gangrenosum. Int J Dermatol. 2003; 42 898-902
162 Cone L A, Annunziata G M, Gebhart R N. Malignant pyoderma and Wegener's granulomatosis. Mayo Clin Proc. 1998; 73 390
163 Barksdale S K, Hallahan C W, Kerr G S, Fauci A S, Stern J B, Travis W D. Cutaneous pathology in Wegener's granulomatosis: a clinicopathologic study of 75 biopsies in 46 patients. Am J Surg Pathol. 1995; 19 161-172
164 Allen D C, Doherty C C, O'Reilly D P. Pathology of the heart and the cardiac conduction system in Wegener's granulomatosis. Br Heart J. 1984; 52 674-678
165 Forstot J Z, Overlie P A, Neufeld G K, Harmon C E, Forstot S L. Cardiac complications of Wegener granulomatosis: a case report of complete heart block and review of the literature. Semin Arthritis Rheum. 1980; 10 148-154
166 Morelli S, Gurgo Di Castelmenardo A M, Conti F et al.. Cardiac involvement in patients with Wegener's granulomatosis. Rheumatol Int. 2000; 19 209-212
167 Goodfield N E, Bhandari S, Plant W D, Morley-Davies A, Sutherland G R. Cardiac involvement in Wegener's granulomatosis. Br Heart J. 1995; 73 110-115
168 Grant S C, Levy R D, Venning M C, Ward C, Brooks N H. Wegener's granulomatosis and the heart. Br Heart J. 1994; 71 82-86
169 Kouba D J, Kirsch D G, Mimouni D, Nousari C H. Wegener's granulomatosis with cardiac involvement masquerading as Lyme disease. Clin Exp Rheumatol. 2003; 21 647-649
170 Suleymenlar G, Sarikaya M, Sari R, Tuncer M, Sevinc A. Complete heart block in a patient with Wegener's granulomatosis in remission: a case report. Angiology. 2002; 53 337-340
171 Parry S D, Clark D M, Campbell J. Coronary arteritis in Wegener's granulomatosis causing fatal myocardial infarction. Hosp Med. 2000; 61 284-285
172 Ohkawa S, Miyao M, Chida K et al.. Extensive involvement of the myocardium and the cardiac conduction system in a case of Wegener's granulomatosis. Jpn Heart J. 1999; 40 509-515
173 Bruno P, Le Hello C, Massetti M et al.. Necrotizing granulomata of the aortic valve in Wegener's disease. J Heart Valve Dis. 2000; 9 633-635
174 Davenport A, Goodfellow J, Goel S, Maciver A G, Walker P. Aortic valve disease in patients with Wegener's granulomatosis. Am J Kidney Dis. 1994; 24 205-208
175 Herbst A, Padilla M T, Prasad A R, Morales M C, Copeland J G. Cardiac Wegener's granulomatosis masquerading as left atrial myxoma. Ann Thorac Surg. 2003; 75 1321-1323
176 Weyand C M, Goronzy J J. Medium- and large-vessel vasculitis. N Engl J Med. 2003; 349 160-169
177 Schmidt W A, Seipelt E, Molsen H P, Poehls C, Gromnica-ihle E J. Vasculitis of the internal carotid artery in Wegener's granulomatosis: comparison of ultrasonography, angiography, and MRI. Scand J Rheumatol. 2001; 30 48-50
178 Blockmans D, Baeyens H, Van Loon R, Lauwers G, Bobbaers H. Periaortitis and aortic dissection due to Wegener's granulomatosis. Clin Rheumatol. 2000; 19 161-164
179 Chang Y J, Kerr L D. Isolated abdominal vasculitis as an atypical presentation of Wegener's granulomatosis. Am J Gastroenterol. 2000; 95 297-298
180 Haworth S J, Pusey C D. Severe intestinal involvement in Wegener's granulomatosis. Gut. 1984; 25 1296-1300
181 Camilleri M, Pusey C D, Chadwick V S, Rees A J. Gastrointestinal manifestations of systemic vasculitis. Q J Med. 1983; 52 141-149
182 Steele C, Bohra S, Broe P, Murray F E. Acute upper gastrointestinal haemorrhage and colitis: an unusual presentation of Wegener's granulomatosis. Eur J Gastroenterol Hepatol. 2001; 13 993-995
183 Sokol R J, Farrell M K, McAdams A J. An unusual presentation of Wegener's granulomatosis mimicking inflammatory bowel disease. Gastroenterology. 1984; 87 426-432
184 Zen Y, Sunagozaka H, Tsuneyama K et al.. Incomplete septal cirrhosis associated with Wegener's granulomatosis. Liver. 2002; 22 388-393
185 Yoshimoto T, Kagotani K, Hirao F, Tamai M. Wegener's granulomatosis in a woman with asymptomatic primary biliary cirrhosis. Nihon Kyobu Shikkan Gakkai Zasshi. 1989; 27 1545-1550
186 Boissy C, Bernard E, Chazal M, Fuzibet J G, Michiels J F, Saint-Paul M C. Wegener's granulomatosis disclosed by hepato-splenic involvement. Gastroenterol Clin Biol. 1997; 21 633-635
187 Fonner B T, Nemcek Jr A A, Boschman C. CT appearance of splenic infarction in Wegener's granulomatosis. AJR Am J Roentgenol. 1995; 164 353-354
188 Hafezi-Rachti S, Riess R, Weidner S, Wonka A, Rupprecht H D. The patient with Wegener's granulomatosis and an intrasplenic mass of unknown origin. Nephrol Dial Transplant. 2000; 15 906-908
189 Rentsch J, McColl G. Splenic infarction in Wegener's granulomatosis. J Rheumatol. 2000; 27 1554-1555
190 Roy D K, George A, Chattopadhyay C, Grennan D M. Splenic infarction in a patient with Wegener's granulomatosis. Rheumatology (Oxford). 1999; 38 1162-1163
191 Gal A A, Masor J J. Splenic involvement in Wegener's granulomatosis. Arch Pathol Lab Med. 1996; 120 974-977
192 Fahey J L, Leonard E, Churg J, Godman G. Wegener's granulomatosis. Am J Med. 1954; 17 168-179
193 Kalaitzoglou I, Drevelengas A, Palladas P, Asimaki A. MRI appearance of pulmonary Wegener's granulomatosis with concomitant splenic infarction. Eur Radiol. 1998; 8 367-370
194 Ulbright T M, Katzenstein A L. Solitary necrotizing granulomas of the lung: differentiating features and etiology. Am J Surg Pathol. 1980; 4 13-28
195 Pinching A, Rees A, Pussell B et al.. Relapses in Wegener's granulomatosis: the role of infection. BMJ. 1980; 281 836-838
196 Hind C, Winearis C, Lockwood C et al.. Objective monitoring of activity of Wegener's granulomatosis by measurement of serum C-reactive protein concentration. Clin Nephrol. 1984; 21 341-345
197 Kerr G, Fleisher T, Hallahan C et al.. Limited prognostic value of changes in anti-neutrophil cytoplasmic antibody titer in patients with Wegener's granulomatosis. Arthritis Rheum. 1993; 36 365-371
198 Merkel P A, Polisson R P, Chang Y, Skates S J, Niles J L. Prevalence of antineutrophil cytoplasmic antibodies in a large inception cohort of patients with connective tissue disease. Ann Intern Med. 1997; 126 866-873
199 Boomsma M M, Stegeman C A, van der Leij M J et al.. Prediction of relapses in Wegener's granulomatosis by measurement of antineutrophil cytoplasmic antibody levels: a prospective study. Arthritis Rheum. 2000; 43 2025-2033
200 Wong R C, Silvestrini R A, Savige J A, Fulcher D A, Benson E M. Diagnostic value of classical and atypical antineutrophil cytoplasmic antibody (ANCA) immunofluorescence patterns. J Clin Pathol. 1999; 52 124-128
201 Russell K A, Fass D N, Specks U. Antineutrophil cytoplasmic antibodies reacting with the pro form of proteinase 3 and disease activity in patients with Wegener's granulomatosis and microscopic polyangiitis. Arthritis Rheum. 2001; 44 463-468
202 Cohen Tervaert J W. The value of serial ANCA testing during follow-up studies in patients with ANCA-associated vasculitides: a review. J Nephrol. 1996; 9 232-240
203 Girard T, Mahr A, Noel L H et al.. Are antineutrophil cytoplasmic antibodies a marker predictive of relapse in Wegener's granulomatosis? A prospective study. Rheumatology (Oxford). 2001; 40 147-151
204 Kyndt X, Reumaux D, Bridoux F et al.. Serial measurements of antineutrophil cytoplasmic autoantibodies in patients with systemic vasculitis. Am J Med. 1999; 106 527-533
205 Slart R H, Jager P L, Poot L, Piers D A, Tervaert J W, Stegeman C A. Clinical value of gallium-67 scintigraphy in assessment of disease activity in Wegener's granulomatosis. Ann Rheum Dis. 2003; 62 659-662
206 Exley A R, Bacon P A, Luqmani R A, Kitas G D, Carruthers D M, Moots R. Examination of disease severity in systemic vasculitis from the novel perspective of damage using the vasculitis damage index (VDI). Br J Rheumatol. 1998; 37 57-63
207 Stone J H, Hoffman G S, Merkel P A et al.. A disease-specific activity index for Wegener's granulomatosis: modification of the Birmingham Vasculitis Activity Score. International Network for the Study of the Systemic Vasculitides (INSSYS). Arthritis Rheum. 2001; 44 912-920
208 Ludviksson B R, Sneller M C, Chua K S et al.. Active Wegener's granulomatosis is associated with HLA-DR+ CD4+ T cells exhibiting an unbalanced Th1-type T cell cytokine pattern: reversal with IL-10. J Immunol. 1998; 160 3602-3609
209 Jennette J C, Falk R J. Pathogenesis of the vascular and glomerular damage in ANCA-positive vasculitis. Nephrol Dial Transplant. 1998; 13(suppl 1) 16-20
210 Qasim F J, Mathieson P W, Sendo F, Thiru S, Oliveira D B. Role of neutrophils in the pathogenesis of experimental vasculitis. Am J Pathol. 1996; 149 81-89
211 Harper L, Savage C O. Pathogenesis of ANCA-associated systemic vasculitis. J Pathol. 2000; 190 349-359
212 Brons R H, de Jong M C, de Boer N K, Stegeman C A, Kallenberg C G, Cohen Tervaert J W. Detection of immune deposits in skin lesions of patients with Wegener's granulomatosis. Ann Rheum Dis. 2001; 60 1097-1102
213 Stegeman C A, Tervaert J W, Sluiter W J, Manson W L, de Jong P E, Kallenberg C G. Association of chronic nasal carriage of Staphylococcus aureus and higher relapse rates in Wegener granulomatosis. Ann Intern Med. 1994; 120 12-17
214 George J, Levy Y, Kallenberg C G, Shoenfeld Y. Infections and Wegener's granulomatosis: a cause and effect relationship?. QJM. 1997; 90 367-373
215 Jennette J C, Falk R J. Small-vessel vasculitis. N Engl J Med. 1997; 337 1512-1523
216 Schwarz M I, Brown K K. Small vessel vasculitis of the lung. Thorax. 2000; 55 502-510
217 Lhote F, Guillevin L. Polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome. Sem Respir Crit Care Med. 1998; 19 27-45
218 Gross W L, Schnabel A, Trabandt A. New perspectives in pulmonary angiitis: from pulmonary angiitis and granulomatosis to ANCA associated vasculitis. Sarcoidosis Vasc Diffuse Lung Dis. 2000; 17 33-52
219 Guillevin L, Cohen P, Gayraud M, Lhote F, Jarrousse B, Casassus P. Churg-Strauss syndrome: clinical study and long-term follow-up of 96 patients. Medicine (Baltimore). 1999; 78 26-37
220 Guillevin L, Visser H, Noel L H et al.. Antineutrophil cytoplasm antibodies in systemic polyarteritis nodosa with and without hepatitis B virus infection and Churg-Strauss syndrome: 62 patients. J Rheumatol. 1993; 20 1345-1349
221 Conron M, Beynon H L. Churg-Strauss syndrome. Thorax. 2000; 55 870-877
222 Savage C O, Pottinger B E, Gaskin G, Pusey C D, Pearson J D. Autoantibodies developing to myeloperoxidase and proteinase 3 in systemic vasculitis stimulate neutrophil cytotoxicity toward cultured endothelial cells. Am J Pathol. 1992; 141 335-342
223 Deguchi Y, Shibata N, Kishimoto S. Enhanced expression of the tumour necrosis factor/cachectin gene in peripheral blood mononuclear cells from patients with systemic vasculitis. Clin Exp Immunol. 1990; 81 311-314
224 Mayet W J, Schwarting A, Orth T, Sibelius U, Hattar K, Meyer zum Buschenfelde K H. Signal transduction pathways of membrane expression of proteinase 3 (PR-3) in human endothelial cells. Eur J Clin Invest. 1997; 27 893-899
225 Charles L A, Caldas M L, Falk R J, Terrell R S, Jennette J C. Antibodies against granule proteins activate neutrophils in vitro. J Leukoc Biol. 1991; 50 539-546
226 Casselman B L, Kilgore K S, Miller B F, Warren J S. Antibodies to neutrophil cytoplasmic antigens induce monocyte chemoattractant protein-1 secretion from human monocytes. J Lab Clin Med. 1995; 126 495-502
227 Berger S P, Seelen M A, Hiemstra P S et al.. Proteinase 3, the major autoantigen of Wegener's granulomatosis, enhances IL-8 production by endothelial cells in vitro. J Am Soc Nephrol. 1996; 7 694-701
228 Asagoe K, Yamamoto K, Takahashi A et al.. Down-regulation of CXCR2 expression on human polymorphonuclear leukocytes by TNF-alpha. J Immunol. 1998; 160 4518-4525
229 Falk R J, Terrell R S, Charles L A, Jennette J C. Anti-neutrophil cytoplasmic autoantibodies induce neutrophils to degranulate and produce oxygen radicals in vitro. Proc Natl Acad Sci U S A. 1990; 87 4115-4119
230 Porges A J, Redecha P B, Kimberly W T, Csernok E, Gross W L, Kimberly R P. Anti-neutrophil cytoplasmic antibodies engage and activate human neutrophils via Fc gamma RIIa. J Immunol. 1994; 153 1271-1280
231 Okrent D G, Lichtenstein A K, Ganz T. Direct cytotoxicity of polymorphonuclear leukocyte granule proteins to human lung-derived cells and endothelial cells. Am Rev Respir Dis. 1990; 141 179-185
232 Savage C O, Gaskin G, Pusey C D, Pearson J D. Anti-neutrophil cytoplasm antibodies can recognize vascular endothelial cell-bound anti-neutrophil cytoplasm antibody-associated autoantigens. Exp Nephrol. 1993; 1 190-195
233 Yang J J, Kettritz R, Falk R J, Jennette J C, Gaido M L. Apoptosis of endothelial cells induced by the neutrophil serine proteases proteinase 3 and elastase. Am J Pathol. 1996; 149 1617-1626
234 Gilligan H M, Bredy B, Brady H R et al.. Antineutrophil cytoplasmic autoantibodies interact with primary granule constituents on the surface of apoptotic neutrophils in the absence of neutrophil priming. J Exp Med. 1996; 184 2231-2241
235 Moosig F, Csernok E, Kumanovics G, Gross W L. Opsonization of apoptotic neutrophils by anti-neutrophil cytoplasmic antibodies (ANCA) leads to enhanced uptake by macrophages and increased release of tumour necrosis factor-alpha (TNF-alpha). Clin Exp Immunol. 2000; 122 499-503
236 Bratt J, Palmblad J. Cytokine-induced neutrophil-mediated injury of human endothelial cells. J Immunol. 1997; 159 912-918
237 Barnett Jr C C, Moore E E, Mierau G W et al.. ICAM-1-CD18 interaction mediates neutrophil cytotoxicity through protease release. Am J Physiol. 1998; 274(6 pt 1) C1634-C1644
238 Westlin W F, Kiely J M, Gimbrone Jr M A. Interleukin-8 induces changes in human neutrophil actin conformation and distribution: relationship to inhibition of adhesion to cytokine-activated endothelium. J Leukoc Biol. 1992; 52 43-51
239 de Bandt M, Ollivier V, Meyer O et al.. Induction of interleukin-1 and subsequent tissue factor expression by anti-proteinase 3 antibodies in human umbilical vein endothelial cells. Arthritis Rheum. 1997; 40 2030-2038
240 Leeuwenberg J F, Smeets E F, Neefjes J J et al.. E-selectin and intercellular adhesion molecule-1 are released by activated human endothelial cells in vitro. Immunology. 1992; 77 543-549
241 Stegeman C A, Tervaert J W, Huitema M G, de Jong P E, Kallenberg C G. Serum levels of soluble adhesion molecules intercellular adhesion molecule 1, vascular cell adhesion molecule 1, and E-selectin in patients with Wegener's granulomatosis: relationship to disease activity and relevance during followup. Arthritis Rheum. 1994; 37 1228-1235
242 Ara J, Mirapeix E, Arrizabalaga P et al.. Circulating soluble adhesion molecules in ANCA-associated vasculitis. Nephrol Dial Transplant. 2001; 16 276-285
243 Mayet W J, Csernok E, Szymkowiak C, Gross W L, Meyer zum Buschenfelde K H. Human endothelial cells express proteinase 3, the target antigen of anticytoplasmic antibodies in Wegener's granulomatosis. Blood. 1993; 82 1221-1229
244 Sibelius U, Hattar K, Schenkel A et al.. Wegener's granulomatosis: anti-proteinase 3 antibodies are potent inductors of human endothelial cell signaling and leakage response. J Exp Med. 1998; 187 497-503
245 Cockwell P, Brooks C J, Adu D, Savage C O. Interleukin-8: a pathogenetic role in antineutrophil cytoplasmic autoantibody-associated glomerulonephritis. Kidney Int. 1999; 55 852-863
246 Brouwer E, Huitema M G, Mulder A H et al.. Neutrophil activation in vitro and in vivo in Wegener's granulomatosis. Kidney Int. 1994; 45 1120-1131
247 Schwarting A, Hagen D, Odenthal M et al.. Proteinase-3 mRNA expressed by glomerular epithelial cells correlates with crescent formation in Wegener's granulomatosis. Kidney Int. 2000; 57 2412-2422
248 Taekema-Roelvink M E, van Kooten C, Janssens M C, Heemskerk E, Daha M R. Effect of anti-neutrophil cytoplasmic antibodies on proteinase 3-induced apoptosis of human endothelial cells. Scand J Immunol. 1998; 48 37-43
249 Cunningham M A, Ono T, Hewitson T D, Tipping P G, Becker G J, Holdsworth S R. Tissue factor pathway inhibitor expression in human crescentic glomerulonephritis. Kidney Int. 1999; 55 1311-1318
250 Slupsky J R, Kalbas M, Willuweit A, Henn V, Kroczek R A, Muller-Berghaus G. Activated platelets induce tissue factor expression on human umbilical vein endothelial cells by ligation of CD40. Thromb Haemost. 1998; 80 1008-1014
251 Komocsi A, Lamprecht P, Csernok E et al.. Peripheral blood and granuloma CD4(+)CD28(-) T cells are a major source of interferon-gamma and tumor necrosis factor-alpha in Wegener's granulomatosis. Am J Pathol. 2002; 160 1717-1724
252 Giscombe R, Nityanand S, Lewin N, Grunewald J, Lefvert A K. Expanded T cell populations in patients with Wegener's granulomatosis: characteristics and correlates with disease activity. J Clin Immunol. 1998; 18 404-413
253 Rasmussen N, Petersen J. Cellular immune responses and pathogenesis in c-ANCA positive vasculitides. J Autoimmun. 1993; 6 227-236
254 Moosig F, Csernok E, Wang G, Gross W L. Costimulatory molecules in Wegener's granulomatosis (WG): lack of expression of CD28 and preferential up-regulation of its ligands B7-1 (CD80) and B7-2 (CD86) on T cells. Clin Exp Immunol. 1998; 114 113-118
255 King W J, Brooks C J, Holder R, Hughes P, Adu D, Savage C O. T lymphocyte responses to anti-neutrophil cytoplasmic autoantibody (ANCA) antigens are present in patients with ANCA-associated systemic vasculitis and persist during disease remission. Clin Exp Immunol. 1998; 112 539-546
256 Griffith M E, Coulthart A, Pusey C D. T cell responses to myeloperoxidase (MPO) and proteinase 3 (PR3) in patients with systemic vasculitis. Clin Exp Immunol. 1996; 103 253-258
257 Csernok E, Trabandt A, Muller A et al.. Cytokine profiles in Wegener's granulomatosis: predominance of type 1 (Th1) in the granulomatous inflammation. Arthritis Rheum. 1999; 42 742-750
258 Muller A, Trabandt A, Gloeckner-Hofmann K et al.. Localized Wegener's granulomatosis: predominance of CD26 and IFN-gamma expression. J Pathol. 2000; 192 113-120
259 Schlaak J F, Nieder P, Meyer zum Buschenfelde K H, Fleischer B. Human T helper cells reactive with somatic bacterial antigens belong to the Th1 subset. Med Microbiol Immunol (Berl). 1994; 183 169-175
260 Gross W L, Trabandt A, Csernok E. Pathogenesis of Wegener's granulomatosis. Ann Med Interne (Paris). 1998; 149 280-286
261 Libraty D H, Airan L E, Uyemura K et al.. Interferon-gamma differentially regulates interleukin-12 and interleukin-10 production in leprosy. J Clin Invest. 1997; 99 336-341
262 Balding C E, Howie A J, Drake-Lee A B, Savage C O. Th2 dominance in nasal mucosa in patients with Wegener's granulomatosis. Clin Exp Immunol. 2001; 125 332-339
263 Coulomb-L'Hermine A, Capron F, Zou W et al.. Expression of the chemokine RANTES in pulmonary Wegener's granulomatosis. Hum Pathol. 2001; 32 320-326
264 Cockwell P, Howie A J, Adu D, Savage C O. In situ analysis of C-C chemokine mRNA in human glomerulonephritis. Kidney Int. 1998; 54 827-836
264a Ralston D R, Marsh C B, Lowe M P, Wewers M D. Antineutrophil cytoplasmic antibodies induce monocyte IL-8 release role of surface proteinase-3, alpha-1-antitrypsin, and Fc gamma receptors. J Clin Invest. 1997; 100 1416-1424
265 Yeung M M, Melgar S, Baranov V et al.. Characterisation of mucosal lymphoid aggregates in ulcerative colitis: immune cell phenotype and TcR-gammadelta expression. Gut. 2000; 47 215-227
266 Martens P B, Goronzy J J, Schaid D, Weyand C M. Expansion of unusual CD4+ T cells in severe rheumatoid arthritis. Arthritis Rheum. 1997; 40 1106-1114
267 Namekawa T, Snyder M R, Yen J H et al.. Killer cell activating receptors function as costimulatory molecules on CD4+CD28null T cells clonally expanded in rheumatoid arthritis. J Immunol. 2000; 165 1138-1145
268 Lamprecht P, Moosig F, Csernok E et al.. CD28 negative T cells are enriched in granulomatous lesions of the respiratory tract in Wegener's granulomatosis. Thorax. 2001; 56 751-757
269 Rasmussen N, Petersen J, Ralfkiaer E, Avnstom S, Wiik A. Spontaneous and induced immunoglobulin synthesis and anti-neutrophil cytoplasm antibodies in Wegener's granulomatosis: relation to leukocyte subpopulations in blood and active lesions. Rheumatol Int. 1988; 8 153-158
270 Wang G, Hansen H, Tatsis E, Csernok E, Lemke H, Gross W L. High plasma levels of the soluble form of CD30 activation molecule reflect disease activity in patients with Wegener's granulomatosis. Am J Med. 1997; 102 517-523
271 Mosmann T R, Sad S. The expanding universe of T-cell subsets: Th1, Th2 and more. Immunol Today. 1996; 17 138-146
272 Mulder A H, Stegeman C A, Kallenberg C G. Activation of granulocytes by anti-neutrophil cytoplasmic antibodies (ANCA) in Wegener's granulomatosis: a predominant role for the IgG3 subclass of ANCA. Clin Exp Immunol. 1995; 101 227-232
273 Brouwer E, Tervaert J W, Horst G et al.. Predominance of IgG1 and IgG4 subclasses of anti-neutrophil cytoplasmic autoantibodies (ANCA) in patients with Wegener's granulomatosis and clinically related disorders. Clin Exp Immunol. 1991; 83 379-386
274 Aalberse R C, van der Gaag R, van Leeuwen J. Serologic aspects of IgG4 antibodies, I: Prolonged immunization results in an IgG4-restricted response. J Immunol. 1983; 130 722-726
275 Mayet W J, Marker-Hermann E, Schlaak J, Meyer Zum Buschenfelde K H. Irregular cytokine pattern of CD4+ T lymphocytes in response to Staphylococcus aureus in patients with Wegener's granulomatosis. Scand J Immunol. 1999; 49 585-594
276 Elkon K B, Sutherland D C, Rees A J, Hughes G R, Batchelor J R. HLA antigen frequencies in systemic vasculitis: increase in HLA-DR2 in Wegener's granulomatosis. Arthritis Rheum. 1983; 26 102-105
277 Papiha S S, Murty G E, Ad'Hia A, Mains B T, Venning M. Association of Wegener's granulomatosis with HLA antigens and other genetic markers. Ann Rheum Dis. 1992; 51 246-248
278 Katz P, Alling D W, Haynes B F, Fauci A S. Association of Wegener's granulomatosis with HLA-B8. Clin Immunol Immunopathol. 1979; 14 268-270
279 Nakamaru Y, Maguchi S, Takizawa M, Fukuda S, Inuyama Y. The association between human leukocyte antigens (HLA) and cytoplasmic-antineutrophil cytoplasmic antibody (cANCA)-positive Wegener's granulomatosis in a Japanese population. Rhinology. 1996; 34 163-165
280 Beigel A, Lehmann H, Westphal E. The spectrum of histocompatibility antigens (HLA) in Wegener's granulomatosis [author's transl]. Arch Otorhinolaryngol. 1981; 233 157-160
281 Zhang L, Jayne D R, Zhao M H, Lockwood C M, Oliveira D B. Distribution of MHC class II alleles in primary systemic vasculitis. Kidney Int. 1995; 47 294-298
282 Strimlan C V, Taswell H F, Kueppers F, DeRemee R A, McDonald T J. HLA-A antigens of patients with Wegener's granulomatosis. Tissue Antigens. 1978; 11 129-131
283 Dijstelbloem H M, Scheepers R H, Oost W W et al.. Fcgamma receptor polymorphisms in Wegener's granulomatosis: risk factors for disease relapse. Arthritis Rheum. 1999; 42 1823-1827
284 Bredius R G, de Vries C E, Troelstra A et al.. Phagocytosis of Staphylococcus aureus and Haemophilus influenzae type B opsonized with polyclonal human IgG1 and IgG2 antibodies: functional hFc gamma RIIa polymorphism to IgG2. J Immunol. 1993; 151 1463-1472
285 Elzouki A N, Segelmark M, Wieslander J, Eriksson S. Strong link between the alpha 1-antitrypsin PiZ allele and Wegener's granulomatosis. J Intern Med. 1994; 236 543-548
286 Dolman K M, Stegeman C A, van de Wiel B A et al.. Relevance of classic anti-neutrophil cytoplasmic autoantibody (C-ANCA)-mediated inhibition of proteinase 3-alpha 1-antitrypsin complexation to disease activity in Wegener's granulomatosis. Clin Exp Immunol. 1993; 93 405-410
287 Savige J A, Chang L, Cook L, Burdon J, Daskalakis M, Doery J. Alpha 1-antitrypsin deficiency and anti-proteinase 3 antibodies in anti-neutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis. Clin Exp Immunol. 1995; 100 194-197
288 Borgmann S, Endisch G, Urban S, Sitter T, Fricke H. A linkage disequilibrium between genes at the serine protease inhibitor gene cluster on chromosome 14q32.1 is associated with Wegener's granulomatosis. Clin Immunol. 2001; 98 244-248
289 Langford C A. Wegener's granulomatosis: current and upcoming therapies. Arthritis Res Ther. 2003; 5 180-191
290 Walton E W. Giant cell granuloma of the respiratory tract (Wegener's granulomatosis). BMJ. 1958; 2 265-270
291 Hoffman G S. “Wegener's granulomatosis”: the path traveled since 1931. Medicine (Baltimore). 1994; 73 325-329
292 Hollander D, Manning R T. The use of alkylating agents in the treatment of Wegener's granulomatosis. Ann Intern Med. 1967; 67 393-398
293 Guillevin L, Cordier J F, Lhote F et al.. A prospective, multicenter, randomized trial comparing steroids and pulse cyclophosphamide versus steroids and oral cyclophosphamide in the treatment of generalized Wegener's granulomatosis. Arthritis Rheum. 1997; 40 2187-2198
294 Mahr A, Girard T, Agher R, GuillevinL L. Analysis of factors predictive of survival based on 49 patients with systemic Wegener's granulomatosis and prospective follow-up. Rheumatology (Oxford). 2001; 40 492-498
295 Lynch III J P, McCune W J. Immunosuppressive and cytotoxic pharmacotherapy for pulmonary disorders. Am J Respir Crit Care Med. 1997; 155 395-420
296 Talar-Williams C, Hijazi Y M, Walther M M et al.. Cyclophosphamide-induced cystitis and bladder cancer in patients with Wegener granulomatosis. Ann Intern Med. 1996; 124 477-484
297 Hoffman G S, Leavitt R Y, Fleisher T A, Minor J R, Fauci A S. Treatment of Wegener's granulomatosis with intermittent high-dose intravenous cyclophosphamide. Am J Med. 1990; 89 403-410
298 Le Thi Huong D, Papo T, Piette J C et al.. Monthly intravenous pulse cyclophosphamide therapy in Wegener's granulomatosis. Clin Exp Rheumatol. 1996; 14 9-16
299 Reinhold-Keller E, Kekow J, Schnabel A et al.. Influence of disease manifestation and antineutrophil cytoplasmic antibody titer on the response to pulse cyclophosphamide therapy in patients with Wegener's granulomatosis. Arthritis Rheum. 1994; 37 919-924
300 Haubitz M, Frei U, Rother U, Brunkhorst R, Koch K M. Cyclophosphamide pulse therapy in Wegener's granulomatosis. Nephrol Dial Transplant. 1991; 6 531-535
301 Drosos A A, Sakkas L I, Goussia A, Siamopoulos K C, Moutsopoulos H M. Pulse cyclophosphamide therapy in Wegener's granulomatosis: a pilot study. J Intern Med. 1992; 232 279-282
302 de Groot K, Adu D, Savage C O. The value of pulse cyclophosphamide in ANCA-associated vasculitis: meta-analysis and critical review. Nephrol Dial Transplant. 2001; 16 2018-2027
303 de Groot K, Muhler M, Reinhold-Keller E, Paulsen J, Gross W L. Induction of remission in Wegener's granulomatosis with low dose methotrexate. J Rheumatol. 1998; 25 492-495
304 Langford C A, Talar-Williams C, Sneller M C. Use of methotrexate and glucocorticoids in the treatment of Wegener's granulomatosis: long-term renal outcome in patients with glomerulonephritis. Arthritis Rheum. 2000; 43 1836-1840
305 Jayne D, Rasmussen N, Andrassy K et al.. A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med. 2003; 349 36-44
306 Sneller M C, Hoffman G S, Talar-Williams C, Kerr G S, Hallahan C W, Fauci A S. An analysis of forty-two Wegener's granulomatosis patients treated with methotrexate and prednisone. Arthritis Rheum. 1995; 38 608-613
307 Langford C A, Sneller M C, Hoffman G S. Methotrexate use in systemic vasculitis. Rheum Dis Clin North Am. 1997; 23 841-853
308 Langford C A, Talar-Williams C, Barron K S, Sneller M C. A staged approach to the treatment of Wegener's granulomatosis: induction of remission with glucocorticoids and daily cyclophosphamide switching to methotrexate for remission maintenance. Arthritis Rheum. 1999; 42 2666-2673
309 de Groot K, Reinhold-Keller E, Tatsis E et al.. Therapy for the maintenance of remission in sixty-five patients with generalized Wegener's granulomatosis: methotrexate versus trimethoprim/sulfamethoxazole. Arthritis Rheum. 1996; 39 2052-2061
310 Stone J H, Tun W, Hellman D B. Treatment of non-life threatening Wegener's granulomatosis with methotrexate and daily prednisone as the initial therapy of choice. J Rheumatol. 1999; 26 1134-1139
311 Furst D E. Practical clinical pharmacology and drug interactions of low-dose methotrexate therapy in rheumatoid arthritis. Br J Rheumatol. 1995; 34(suppl 2) 20-25
312 Godeau B, Mainardi J L, Roudot-Thoraval F et al.. Factors associated with Pneumocystis carinii pneumonia in Wegener's granulomatosis. Ann Rheum Dis. 1995; 54 991-994
313 Weiner S R, Paulus H E. Treatment of Wegener's granulomatosis. Semin Respir Med. 1989; 10 156-161
314 Knight A, Askling J, Ekbom A. Cancer incidence in a population-based cohort of patients with Wegener's granulomatosis. Int J Cancer. 2002; 100 82-85
315 Odeh M. Renal cell carcinoma associated with cyclophosphamide therapy for Wegener's granulomatosis. Scand J Rheumatol. 1996; 25 391-393
316 Lee K, Baglin T P, Marcus R E. Therapy-related leukaemia in Wegener's granulomatosis. Clin Lab Haematol. 1991; 13 207-209
317 Koldingsnes W, Gran J T, Omdal R, Husby G. Wegener's granulomatosis: long-term follow-up of patients treated with pulse cyclophosphamide. Br J Rheumatol. 1998; 37 659-664
318 Nowack R, Gobel U, Klooker P, Hergesell O, Andrassy K, van der Woude F J. Mycophenolate mofetil for maintenance therapy of Wegener's granulomatosis and microscopic polyangiitis: a pilot study in 11 patients with renal involvement. J Am Soc Nephrol. 1999; 10 1965-1971
319 Langford C A, Talar-Williams C, Sneller M C. Mycophenolate mofetil for remission maintenance in the treatment of Wegener's granulomatosis. Arthritis Rheum. 2004; 51 278-283
320 Stegeman C A, Cohen Tervaert J W, de Jong P E, Kallenberg C G. Trimethoprim-sulfamethoxazole (co-trimoxazole) for the prevention of relapses of Wegener's granulomatosis. Dutch Co-Trimoxazole Wegener Study Group. N Engl J Med. 1996; 335 16-20
321 Hoffman G S. Immunosuppressive therapy is always required for the treatment of limited Wegener's granulomatosis. Sarcoidosis Vasc Diffuse Lung Dis. 1996; 13 249-252
322 DeRemee R A, McDonald T J, Weiland L H. Wegener's granulomatosis: observations on treatment with antimicrobial agents. Mayo Clin Proc. 1985; 60 27-32
323 DeRemee R A. The treatment of Wegener's granulomatosis with trimethoprim/sulfamethoxazole: illusion or vision?. Arthritis Rheum. 1988; 31 1068-1074
324 Georgi J, Ulmer M, Gross W L. Cotrimoxazole in Wegener's granulomatosis: a prospective study. Immun Infekt. 1991; 19 97-98
325 Huynh-Do U, Gantenbein H, Binswanger U. Pneumocystis carinii pneumonia during immunosuppressive therapy for antineutrophil cytoplasmic autoantibody-positive vasculitis. Arch Intern Med. 1995; 155 872-874
326 Ognibene F P, Shelhamer J H, Hoffman G S et al.. Pneumocystis carinii pneumonia: a major complication of immunosuppressive therapy in patients with Wegener's granulomatosis [see comments]. Am J Respir Crit Care Med. 1995; 151(3 pt 1) 795-799
327 Jarrousse B, Guillevin L, Bindi P et al.. Increased risk of Pneumocystis carinii pneumonia in patients with Wegener's granulomatosis. Clin Exp Rheumatol. 1993; 11 615-621
328 Gross W L. New concepts in treatment protocols for severe systemic vasculitis. Curr Opin Rheumatol. 1999; 11 41-46
329 Chung J B, Armstrong K, Schwartz J S, Albert D. Cost-effectiveness of prophylaxis against Pneumocystis carinii pneumonia in patients with Wegner's granulomatosis undergoing immunosuppressive therapy. Arthritis Rheum. 2000; 43 1841-1848
330 Munoz P, Munoz R M, Palomo J, Rodriguez-Creixems M, Munoz R, Bouza E. Pneumocystis carinii infection in heart transplant recipients: efficacy of a weekend prophylaxis schedule. Medicine (Baltimore). 1997; 76 415-422
331 El-Sadr W M, Murphy R L, Yurik T M et al.. Atovaquone compared with dapsone for the prevention of Pneumocystis carinii pneumonia in patients with HIV infection who cannot tolerate trimethoprim, sulfonamides, or both. Community Program for Clinical Research on AIDS and the AIDS Clinical Trials Group. N Engl J Med. 1998; 339 1889-1895
332 Leoung G S, Feigal Jr D W, Montgomery A B et al.. Aerosolized pentamidine for prophylaxis against Pneumocystis carinii pneumonia. The San Francisco community prophylaxis trial. N Engl J Med. 1990; 323 769-775
333 Klemmer P J, Chalermskulrat W, Reif M S, Hogan S L, Henke D C, Falk R J. Plasmapheresis therapy for diffuse alveolar hemorrhage in patients with small-vessel vasculitis. Am J Kidney Dis. 2003; 42 1149-1153
334 Gaskin G, Pusey C D. Plasmapheresis in antineutrophil cytoplasmic antibody-associated systemic vasculitis. Ther Apher. 2001; 5 176-181
335 Krafcik S, Covin R, Lynch III J, Sitrin R. Wegener's granulomatosis in the elderly. Chest. 1996; 109 430-437
336 Gyetko M R, Toews G B. Immunology of the aging lung. Clin Chest Med. 1993; 14 379-391
337 Koldingsnes W, Nossent J C. Baseline features and initial treatment as predictors of remission and relapse in Wegener's granulomatosis. J Rheumatol. 2003; 30 80-88
338 Clarke A E, Bitton A, Eappen R, Danoff D S, Esdaile J M. Treatment of Wegener's granulomatosis after renal transplantation: is cyclosporine the preferred treatment?. Transplantation. 1990; 50 1047-1051
339 Allen N B, Caldwell D S, Rice J R, McCallum R M. Cyclosporin A therapy for Wegener's granulomatosis. Adv Exp Med Biol. 1993; 336 473-476
340 Ghez D, Westeel P F, Henry I, Pruna A, Fournier A, Lassoued K. Control of a relapse and induction of long-term remission of Wegener's granulomatosis by cyclosporine. Am J Kidney Dis. 2002; 40 E6
341 Birck R, Warnatz K, Lorenz H M et al.. 15-Deoxyspergualin in patients with refractory ANCA-associated systemic vasculitis: a six-month open-label trial to evaluate safety and efficacy. J Am Soc Nephrol. 2003; 14 440-447
342 Jayne D R, Chapel H, Adu D et al.. Intravenous immunoglobulin for ANCA-associated systemic vasculitis with persistent disease activity. QJM. 2000; 93 433-439
343 Jayne D R, Esnault V L, Lockwood C M. ANCA anti-idiotype antibodies and the treatment of systemic vasculitis with intravenous immunoglobulin. J Autoimmun. 1993; 6 207-219
344 Jayne D R, Davies M J, Fox C J, Black C M, Lockwood C M. Treatment of systemic vasculitis with pooled intravenous immunoglobulin. Lancet. 1991; 337 1137-1139
345 Richter C, Schnabel A, Csernok E, De Groot K, Reinhold-Keller E, Gross W L. Treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis with high-dose intravenous immunoglobulin. Clin Exp Immunol. 1995; 101 2-7
346 Taylor C T, Buring S M, Taylor K H. Treatment of Wegener's granulomatosis with immune globulin: CNS involvement in an adolescent female. Ann Pharmacother. 1999; 33 1055-1059
347 Lockwood C M, Thiru S, Isaacs J D, Hale G, Waldmann H. Long-term remission of intractable systemic vasculitis with monoclonal antibody therapy. Lancet. 1993; 341 1620-1622
348 Lockwood C M. New treatment strategies for systemic vasculitis: the role of intravenous immune globulin therapy. Clin Exp Immunol. 1996; 104(suppl 1) 77-82
349 Lockwood C M, Thiru S, Stewart S et al.. Treatment of refractory Wegener's granulomatosis with humanized monoclonal antibodies. Q J Med. 1996; 89 903-912
350 Specks U, Fervenza F C, McDonald T J, Hogan M C. Response of Wegener's granulomatosis to anti-CD20 chimeric monoclonal antibody therapy. Arthritis Rheum. 2001; 44 2836-2840
351 Dick A D, Meyer P, James T et al.. Campath-1H therapy in refractory ocular inflammatory disease. Br J Ophthalmol. 2000; 84 107-109
352 Kool J, de Keizer R J, Siegert C E. Antithymocyte globulin treatment of orbital Wegener granulomatosis: a follow-up study. Am J Ophthalmol. 1999; 127 738-739
353 Papo T, Le Thi Huong D, Wiederkehr J L et al.. Etoposide in Wegener's granulomatosis. Rheumatology (Oxford). 1999; 38 473-475
354 Stone J H, Uhlfelder M L, Hellmann D B, Crook S, Bedocs N M, Hoffman G S. Etanercept combined with conventional treatment in Wegener's granulomatosis: a six-month open-label trial to evaluate safety. Arthritis Rheum. 2001; 44 1149-1154

Joseph P Lynch IIIM.D

Division of Pulmonary and Critical Care Medicine and Hospitalists

The David Geffen School of Medicine at UCLA

10833 Le Conte Ave., Rm. 37-131 CHS

Los Angeles, CA 90095-1690

Email: jplynch@mednet.ucla.edu