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Semin Respir Crit Care Med 2003; 24(4): 427-436
DOI: 10.1055/s-2003-42377
Copyright © 2003 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA. Tel.: +1(212) 584-4662

Pulmonary Vasculitis: CT Features

James G. Ravenel1 , H. Page McAdams2
  • 1Department of Radiology, Medical University of South Carolina, Charleston, South Carolina
  • 2Department of Radiology, Duke University Medical Center, Durham, North Carolina
Further Information

Publication History

Publication Date:
18 October 2003 (online)

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ABSTRACT

The pulmonary vasculitides are a heterogeneous group of inflammatory disorders that may be primarily localized to the pulmonary vasculature or be systemic in nature. The primary pulmonary vasculitides usually affect small vessels, whereas the systemic vasculitides can involve any size pulmonary vessels. For all types of vasculitis, there is a high degree of overlap within groups in regard to their appearance at computed tomography (CT). The most common CT findings are the result of either diffuse or focal pulmonary hemorrhage, are nonspecific, and include ground-glass opacity, consolidation, and small centrilobular nodules. Therefore, the CT findings must be considered in concert with the history, physical examination, and laboratory examination when a specific diagnosis is sought. This review will discuss the typical CT features of both the systemic and the primary pulmonary vasculitides, drawing a distinction where CT is helpful in differentiating among the various causes.

KEYWORDS

Vasculitis - ANCA positive vasculitis - pulmonary angiitis - granulomatosis

REFERENCES

  • 1 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
    CrossrefPubMedSearch in Google Scholar
  • 2 Hoffman G S. Classification of the systemic vasculitides: antineutrophil cytoplasmic antibodies, consensus and controversy.  Clin Exp Rheumatol . 1998;  16 111-115
    PubMedSearch in Google Scholar
  • 3 Rao J K, Allen N B, Pincus T. Limitations of the 1990 American College of Rheumatology classification criteria in the diagnosis of vasculitis.  Ann Intern Med . 1998;  129 345-352
    CrossrefPubMedSearch in Google Scholar
  • 4 Sorensen S F, Slot O, Tvede N, Petersen J. A prospective study of vasculitis patients collected in a five-year period: evaluation of the Chapel Hill nomenclature.  Ann Rheum Dis . 2000;  59 478-482
    PubMedSearch in Google Scholar
  • 5 Kerr G S, Hallahan C W, Giordano J. et al . Takayasu arteritis.  Ann Intern Med . 1994;  120 919-929
    CrossrefPubMedSearch in Google Scholar
  • 6 Arend W P, Michel B A, Bloch D A. et al . The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis.  Arthritis Rheum . 1990;  33 1129-1134
    PubMedSearch in Google Scholar
  • 7 Weidner N. Giant-cell vasculitides.  Semin Diagn Pathol . 2001;  18 24-33
    PubMedSearch in Google Scholar
  • 8 Park J H, Chung J W, Im J G, Kim S K, Park Y B, Han M C. Takayasu arteritis: evaluation of mural changes in the aorta and pulmonary artery with CT angiography.  Radiology . 1995;  196 89-93
    PubMedSearch in Google Scholar
  • 9 Yamada I, Nakagawa T, Himeno Y, Numano F, Shibuya H. Takayasu arteritis: evaluation of the thoracic aorta with CT angiography.  Radiology . 1998;  209 103-109
    PubMedSearch in Google Scholar
  • 10 Takahashi K, Honda M, Furuse M, Yanagisawa M, Saitoh K. CT findings of pulmonary parenchyma in Takayasu arteritis.  J Comput Assist Tomogr . 1996;  20 742-748
    CrossrefPubMedSearch in Google Scholar
  • 11 Hunder G G, Bloch D A, Michel B A. et al . The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis.  Arthritis Rheum . 1990;  33 1122-1128
    CrossrefPubMedSearch in Google Scholar
  • 12 Hunder G G. Clinical features of GCA/PMR.  Clin Exp Rheumatol . 2000;  18 (suppl 20) S6-S8
    PubMedSearch in Google Scholar
  • 13 Doyle L, McWilliam L, Hasleton P S. Giant cell arteritis with pulmonary involvement.  Br J Dis Chest . 1988;  82 88-92
    CrossrefPubMedSearch in Google Scholar
  • 14 Zenone T, Souquet P J, Bohas C, Vital Durand D, Bernard J P. Unusual manifestations of giant cell arteritis: pulmonary nodules, cough, conjunctivitis and otitis with deafness.  Eur Respir J . 1994;  7 2252-2254
    CrossrefPubMedSearch in Google Scholar
  • 15 Kramer M R, Melzer E, Nesher G, Sonnenblick M. Pulmonary manifestations of temporal arteritis.  Eur J Respir Dis . 1987;  71 430-433
    PubMedSearch in Google Scholar
  • 16 Bradley J D, Pinals R S, Blumenfeld H B, Poston W M. Giant cell arteritis with pulmonary nodules.  Am J Med . 1984;  77 135-140
    CrossrefPubMedSearch in Google Scholar
  • 17 Amato M B, Barbas C S, Delmonte V C, Carvalho C R. Concurrent Churg-Strauss syndrome and temporal arteritis in a young patient with pulmonary nodules.  Am Rev Respir Dis . 1989;  139 1539-1542
    PubMedSearch in Google Scholar
  • 18 Frey E E, Matherne G P, Mahoney L T, Sato Y, Stanford W, Smith W L. Coronary artery aneurysms due to Kawasaki disease: diagnosis with ultrafast CT.  Radiology . 1988;  167 725-726
    PubMedSearch in Google Scholar
  • 19 Bonsib S M. Polyarteritis nodosa.  Semin Diagn Pathol . 2001;  18 14-23
    PubMedSearch in Google Scholar
  • 20 Matsumoto T, Homma S, Okada M. et al . The lung in polyarteritis nodosa: a pathologic study of 10 cases.  Hum Pathol . 1993;  24 717-724
    CrossrefPubMedSearch in Google Scholar
  • 21 Watts R A, Scott D G, Lane S E. Epidemiology of Wegener's granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome.  Cleve Clin J Med . 2002;  69 (suppl 2) SII84-SII86
    PubMedSearch in Google Scholar
  • 22 Cheah F K, Sheppard M N, Hansell D M. Computed tomography of diffuse pulmonary haemorrhage with pathological correlation.  Clin Radiol . 1993;  48 89-93
    PubMedSearch in Google Scholar
  • 23 Primack S L, Miller R R, Müller N L. Diffuse pulmonary hemorrhage: clinical, pathologic, and imaging features.  AJR Am J Roentgenol . 1995;  164 295-300
    PubMedSearch in Google Scholar
  • 24 Murayama S, Murakami J, Yabuuchi H, Soeda H, Masuda K. "Crazy paving appearance" on high-resolution CT in various diseases.  J Comput Assist Tomogr . 1999;  23 749-752
    PubMedSearch in Google Scholar
  • 25 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
    CrossrefPubMedSearch in Google Scholar
  • 26 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
    CrossrefPubMedSearch in Google Scholar
  • 27 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
    CrossrefPubMedSearch in Google Scholar
  • 28 Wolff S M, Fauci A S, Horn R G, Dale D C. Wegener's granulomatosis.  Ann Intern Med . 1974;  81 513-525
    PubMedSearch in Google Scholar
  • 29 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
    PubMedSearch in Google Scholar
  • 30 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
    CrossrefPubMedSearch in Google Scholar
  • 31 Devaney K O, Travis W D, Hoffman G, Leavitt R, Lebovics R, Fauci A S. Interpretation of head and neck biopsies in Wegener's granulomatosis: a pathologic study of 126 biopsies in 70 patients.  Am J Surg Pathol . 1990;  14 555-564
    CrossrefPubMedSearch in Google Scholar
  • 32 Yi E S, Colby T V. Wegener's granulomatosis.  Semin Diagn Pathol . 2001;  18 34-46
    PubMedSearch in Google Scholar
  • 33 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
    PubMedSearch in Google Scholar
  • 34 Aberle D R, Gamsu G, Lynch D. Thoracic manifestations of Wegener granulomatosis: diagnosis and course.  Radiology . 1990;  174 703-709
    PubMedSearch in Google Scholar
  • 35 Maskell G F, Lockwood C M, Flower C D. Computed tomography of the lung in Wegener's granulomatosis.  Clin Radiol . 1993;  48 377-380
    PubMedSearch in Google Scholar
  • 36 Pretorius E S, Stone J H, Hellman D B, Fishman E K. Wegener's granulomatosis: spectrum of CT findings in diagnosis, disease progression, and response to therapy.  Crit Rev Diagn Imaging . 2000;  41 279-313
    PubMedSearch in Google Scholar
  • 37 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
    CrossrefPubMedSearch in Google Scholar
  • 38 McDonald T J, Neel H B, DeRemee R A. Wegener's granulomatosis of the subglottis and the upper portion of the trachea.  Ann Otol Rhinol Laryngol . 1982;  91 588-592
    PubMedSearch in Google Scholar
  • 39 Daum T E, Specks U, Colby T V. et al . Tracheobronchial involvement in Wegener's granulomatosis.  Am J Respir Crit Care Med . 1995;  151 522-526
    PubMedSearch in Google Scholar
  • 40 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
    CrossrefPubMedSearch in Google Scholar
  • 41 Jennette J C, Falk R J. Small-vessel vasculitis.  N Engl J Med . 1997;  337 1512-1523
    CrossrefPubMedSearch in Google Scholar
  • 42 Guillevin L, Durand-Gasselin B, Cavallos R. et al . Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients.  Arthritis Rheum . 1999;  42 421-430
    CrossrefPubMedSearch in Google Scholar
  • 43 Nachman P H, Hogan S L, Jennette J C, Falk R J. Treatment response and relapse in antineutrophil cytoplasmic autoantibody-associated microscopic polyangiitis and glomerulonephritis.  J Am Soc Nephrol . 1996;  7 33-39
    PubMedSearch in Google Scholar
  • 44 Hogan S L, Nachman P H, Wilkman A S, Jennette J C, Falk R J. Prognostic markers in patients with antineutrophil cytoplasmic autoantibody-associated microscopic polyangiitis and glomerulonephritis.  J Am Soc Nephrol.. 1996;  7 23-32
    PubMedSearch in Google Scholar
  • 45 Jennette J C, Thomas D B, Falk R J. Microscopic polyangiitis (microscopic polyarteritis).  Semin Diagn Pathol . 2001;  18 3-13
    PubMedSearch in Google Scholar
  • 46 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
    PubMedSearch in Google Scholar
  • 47 Lauque D, Cadranel J, Lazor R. et al . Microscopic polyangiitis with alveolar hemorrhage: a study of 29 cases and review of the literature. Groupe d'Etudes et de Recherche sur les Maladies "Orphelines" Pulmonaires (GERM"O"P).  Medicine (Baltimore) . 2000;  79 222-233
    CrossrefPubMedSearch in Google Scholar
  • 48 Brugiere O, Raffy O, Sleiman C. et al . Progressive obstructive lung disease associated with microscopic polyangiitis.  Am J Respir Crit Care Med . 1997;  155 739-742
    PubMedSearch in Google Scholar
  • 49 Conron M, Beynon H L. Churg-Strauss syndrome.  Thorax . 2000;  55 870-877
    CrossrefPubMedSearch in Google Scholar
  • 50 Worthy S A, Müller N L, Hansell D M, Flower C D. Churg-Strauss syndrome: the spectrum of pulmonary CT findings in 17 patients.  AJR Am J Roentgenol . 1998;  170 297-300
    CrossrefPubMedSearch in Google Scholar
  • 51 Choi Y H, Im J G, Han B K, Kim J H, Lee K Y, Myoung N H. Thoracic manifestation of Churg-Strauss syndrome: radiologic and clinical findings.  Chest . 2000;  117 117-124
    CrossrefPubMedSearch in Google Scholar
  • 52 Johkoh T, Müller N L, Akira M. et al . Eosinophilic lung diseases: diagnostic accuracy of thin-section CT in 111 patients.  Radiology . 2000;  216 773-780
    PubMedSearch in Google Scholar
  • 53 Salerno S M, Ormseth E J, Roth B J, Meyer C A, Christensen E D, Dillard T A. Sulfasalazine pulmonary toxicity in ulcerative colitis mimicking clinical features of Wegener's granulomatosis.  Chest . 1996;  110 556-559
    PubMedSearch in Google Scholar
  • 54 Dhillon S S, Singh D, Doe N, Qadri A M, Ricciardi S, Schwarz M I. Diffuse alveolar hemorrhage and pulmonary capillaritis due to propylthiouracil.  Chest . 1999;  116 1485-1488
    CrossrefPubMedSearch in Google Scholar
  • 55 Yokose T, Aida J, Ito Y, Ogura M, Nakagawa S, Nagai T. A case of pulmonary hemorrhage in Henoch-Schonlein purpura accompanied by polyarteritis nodosa in an elderly man.  Respiration . 1993;  60 307-310
    CrossrefPubMedSearch in Google Scholar
  • 56 Olson J C, Kelly K J, Pan C G, Wortmann D W. Pulmonary disease with hemorrhage in Henoch-Schoenlein purpura.  Pediatrics . 1992;  89 1177-1181
    PubMedSearch in Google Scholar
  • 57 Ball J A, Young Jr R K. Pulmonary manifestations of Goodpasture's syndrome: antiglomerular basement membrane disease and related disorders.  Clin Chest Med . 1998;  19 777-791
    CrossrefPubMedSearch in Google Scholar
  • 58 Vazquez-Del Mercado M, Mendoza-Topete A, Best-Aguilera C R, Garcia-De La Torre I. Diffuse alveolar hemorrhage in limited cutaneous systemic sclerosis with positive perinuclear antineutrophil cytoplasmic antibodies.  J Rheumatol . 1996;  23 1821-1823
    PubMedSearch in Google Scholar
  • 59 Schwarz M I, Zamora M R, Hodges T N, Chan E D, Bowler R P, Tuder R M. Isolated pulmonary capillaritis and diffuse alveolar hemorrhage in rheumatoid arthritis and mixed connective tissue disease.  Chest . 1998;  113 1609-1615
    CrossrefPubMedSearch in Google Scholar
  • 60 Schwarz M I, Sutarik J M, Nick J A, Leff J A, Emlen J W, Tuder R M. Pulmonary capillaritis and diffuse alveolar hemorrhage: a primary manifestation of polymyositis.  Am J Respir Crit Care Med . 1995;  151 2037-2040
    CrossrefPubMedSearch in Google Scholar
  • 61 Schnabel A. Reuter M, Csernok E, Richter C, Gross WL.  Subclinical alveolar bleeding in pulmonary vasculitides: correlation with indices of disease activity. Eur Respir J . 1999;  14 118-124
    CrossrefPubMedSearch in Google Scholar
  • 62 Murin S, Wiedemann H P, Matthay R A. Pulmonary manifestations of systemic lupus erythematosus.  Clin Chest Med . 1998;  19 641-665
    PubMedSearch in Google Scholar
  • 63 Erkan F, Gul A, Tasali E. Pulmonary manifestations of Behçet's disease.  Thorax . 2001;  56 572-578
    CrossrefPubMedSearch in Google Scholar
  • 64 Tunaci M, Ozkorkmaz B, Tunaci A, Gul A, Engin G, Acunas B. CT findings of pulmonary artery aneurysms during treatment for Behçet's disease.  AJR Am J Roentgenol . 1999;  172 729-733
    PubMedSearch in Google Scholar
  • 65 Tunaci A, Berkmen Y M, Gokmen E. Thoracic involvement in Behçet's disease: pathologic, clinical, and imaging features.  AJR Am J Roentgenol . 1995;  164 51-56
    PubMedSearch in Google Scholar
  • 66 Ahn J M, Im J G, Ryoo J W. et al . Thoracic manifestations of Behçet syndrome: radiographic and CT findings in nine patients.  Radiology . 1995;  194 199-203
    PubMedSearch in Google Scholar