Pulmonary Capillaritis

Augustine S. Lee; Ulrich Specks

doi:10.1055/s-2004-836146

Seminars in Respiratory and Critical Care Medicine, Table of Contents

Semin Respir Crit Care Med 2004; 25(5): 547-555
DOI: 10.1055/s-2004-836146

Pulmonary Capillaritis

Augustine S. Lee¹ , Ulrich Specks¹

¹Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, Minnesota

Abstract

Pulmonary capillaritis is defined as a histopathologic pattern of alveolar wall inflammation that leads to the disruption of the integrity of alveolar-capillary basement membranes and flooding of the alveoli with blood. The clinical presentation is that of diffuse alveolar hemorrhage (DAH). Pulmonary capillaritis is usually the consequence of an underlying immune-mediated process that is systemic in nature. Rarely, pulmonary capillaritis occurs in isolation. This article outlines a systematic approach to the management of patients presenting with DAH, and provides an overview of specific causes of pulmonary capillaritis.

KEYWORDS

Pulmonary capillaritis - pulmonary vasculitis - small vessel vasculitis - diffuse alveolar hemorrhage

Full Text

References

REFERENCES

1 Colby T V, Fukuoka J, Ewaskow S P, Helmers R, Leslie K O. Pathologic approach to pulmonary hemorrhage. Ann Diagn Pathol. 2001; 5 309-319
2 Mark E J, Ramirez J F. Pulmonary capillaritis and hemorrhage in patients with systemic vasculitis. Arch Pathol Lab Med. 1985; 109 413-418
3 Kadokura M, Colby T V, Myers J L et al.. Pathologic comparison of video-assisted thoracic surgical lung biopsy with traditional open lung biopsy. J Thorac Cardiovasc Surg. 1995; 109 494-498
4 Jennette J C, Falk R J, Andrassy K et al.. Nomenclature of systemic vasculitides: The proposal of an international consensus conference. Arthritis Rheum. 1994; 37 187-192
5 Lai R S, Lin S L, Lai N S, Lee P C. Churg-Strauss syndrome presenting with pulmonary capillaritis and diffuse alveolar hemorrhage. Scand J Rheumatol. 1998; 27 230-232
6 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. 1999; 78 26-37
7 Keogh K A, Specks U. Churg-Strauss syndrome. clinical presentation, antineutrophil cytoplasmic antibodies, and leukotriene receptor antagonists. Am J Med. 2003; 115 284-290
8 Nadrous H F, Yu A C, Specks U, Ryu J H. Pulmonary involvement in Henoch-Schoenlein purpura. Mayo Clin Proc. 2004; 79 1151-1157
9 Zamora M R, Warner M L, Tuder R, Schwarz M I. Diffuse alveolar hemorrhage and systemic lupus erythematosus: clinical presentation, histology, survival, and outcome. Medicine (Baltimore). 1997; 76 192-202
10 Crausman R S, Achenbach G A, Pluss W T, O'Brien R F, Jennings C A. Pulmonary capillaritis and alveolar hemorrhage associated with the antiphospholipid antibody syndrome. J Rheumatol. 1995; 22 554-556
11 Gertner E. Diffuse alveolar hemorrhage in the antiphospolipid syndrome: spectrum of disease and treatment. J Rheumatol. 1999; 26 805-807
12 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
13 Schwarz M I, Sutarik J M, Nick J A, Leff J A, Woodruff Emlen J, Tuder R M. Pulmonary capillaritis and diffuse alveolar hemorrhage: a primary manifestation of polymyositis. Am J Respir Crit Care Med. 1995; 151 2037-2040
14 Nicolls M R, Terada L S, Ruder R M, Prindiville S A, Schwarz M I. Diffuse alveolar hemorrhage with underlying pulmonary capillaritis in the retinoic acid syndrome. Am J Respir Crit Care Med. 1998; 158 1302-1305
15 Choi H K, Merkel P A, Walker A M, Niles J L. Drug-associated antineutrophil cytoplasmic antibody-positive vasculitis: prevalence among patients with high titers of antimyeloperoxidase antibodies. Arthritis Rheum. 2000; 43 405-413
16 Gamble C N, Wiesner K B, Shapiro R F, Boyer W J. The immune complex pathogenesis of glomerulonephritis and pulmonary vasculitis in Behçet's disease. Am J Med. 1979; 66 1031-1039
17 Lombard C M, Colby T V, Elliott C G. Surgical pathology of the lung in anti-basement membrane antibody-associated Goodpasture's syndrome. Hum Pathol. 1989; 20 445-451
18 Badesch D B, Zamora M, Fullerton D et al.. Pulmonary capillaritis: a possible histologic form of acute pulmonary allograft rejection. J Heart Lung Transplant. 1998; 17 415-422
19 Jennings C A, King Jr T E, Tuder R, Cherniack R M, Schwarz M I. Diffuse alveolar hemorrhage with underlying isolated, pauciimmune pulmonary capillaritis. Am J Respir Crit Care Med. 1997; 155 1101-1109
20 Levy J B, Turner A N, Rees A J, Pusey C D. Long-term outcome of anti-glomerular basement membrane antibody disease treated with plasma exchange and immunosuppression. Ann Intern Med. 2001; 134 1033-1042
21 Gaskin G, Pusey C D. Plasmapheresis in antineutrophil cytoplasmic antibody-associated systemic vasculitis. Ther Apher. 2001; 5 176-181
22 Jayne D. Current attitudes to the therapy of vasculitis. Kidney Blood Press Res. 2003; 26 231-239
23 Merkel P A, Choi H K, Niles J L. Evaluation and treatment of vasculitis in the critically ill patient. Crit Care Clin. 2002; 18 321-344
24 Haubitz M, Bohnenstengel F, Brunkhorst R, Schwab M, Hofmann U, Busse D. Cyclophosphamide pharmacokinetics and dose requirements in patients with renal insufficiency. Kidney Int. 2002; 61 1495-1501
25 Glasmacher A, Prentice A, Gorschluter M et al.. Itraconazole prevents invasive fungal infections in neutropenic patients treated for hematologic malignancies: evidence from a meta-analysis of 3,597 patients. J Clin Oncol. 2003; 21 4615-4626
26 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
27 Niles J L, Böttinger E P, Saurina G R et al.. The syndrome of lung hemorrhage and nephritis is usually an ANCA-associated condition. Arch Intern Med. 1996; 156 440-445
28 Hoffman G S, Kerr G S, Leavitt R Y et al.. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med. 1992; 116 488-498
29 Guillevin L, Durand-Gasselin B, Cevallos R et al.. Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients. Arthritis Rheum. 1999; 42 421-430
30 Group T WR. Limited versus severe Wegener's granulomatosis: baseline data on patients in the Wegener's granulomatosis etanercept trial. Arthritis Rheum. 2003; 48 2299-2309
31 Hagen E C, Daha M R, Hermans J et al.. Diagnostic value of standardized assays for anti-neutrophil cytoplasmic antibodies in idiopathic systemic vasculitis. EC/BCR Project for ANCA Assay Standardization[see comments]. Kidney Int. 1998; 53 743-753
32 Savige J, Gillis D, Benson E et al.. International consensus statement on testing and reporting of antineutrophil cytoplasmic antibodies (ANCA). Am J Clin Pathol. 1999; 111 507-513
33 Russell K A, Wiegert E, Schroeder D R, Homburger H A, Specks U. Detection of anti-neutrophil cytoplasmic antibodies under actual clinical testing conditions. Clin Immunol. 2002; 103 196-203
34 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
35 Lauque D, Cadranel J, Lazor R et al.. Microscopic polyangiitis with alveolar hemorrhage: a study of 29 cases and review of the literature. Medicine (Baltimore). 2000; 79 222-233
36 Gallagher H, Kwan J T, Jayne D R. Pulmonary renal syndrome: a 4-year, single-center experience. Am J Kidney Dis. 2002; 39 42-47
37 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
38 Franssen C F, Gans R O, Arends B et al.. Differences between anti-myeloperoxidase- and anti-proteinase 3-associated renal disease. Kidney Int. 1995; 47 193-199
39 Booth A D, Almond M K, Burns A et al.. Outcome of ANCA-associated renal vasculitis: a 5-year retrospective study. Am J Kidney Dis. 2003; 41 776-784
40 Bosch X, Mirapeix E, Font J et al.. Prognostic implication of anti-neutrophil cytoplasmic autoantibodies with myeloperoxidase specificity in anti-glomerular basement membrane disease. Clin Nephrol. 1991; 36 107-113
41 Short A K, Esnault V LM, Lockwood C M. Antineutrophil cytoplasm antibodies and anti-glomerular basement membrane antibodies: two coexisting distinct autoreactivities detectable in patients with rapidly progressive glomerulonephritis. Am J Kidney Dis. 1995; 26 439-445
42 Hellmark T, Niles J L, Collins A B, McCluskey R T, Brunmark C. Comparison of anti-GBM antibodies in sera with or without ANCA. J Am Soc Nephrol. 1997; 8 376-385
43 Clutterbuck E J, Pusey C D. Severe alveolar haemorrhage in Churg-Strauss syndrome. Eur J Respir Dis. 1987; 71 158-163
44 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
45 Nowack R, Göbel 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
46 Koh W H, Thumboo J, Boey M L. Pulmonary haemorrhage in Oriental patients with systemic lupus erythematosus. Lupus. 1997; 6 713-716
47 Santos-Ocampo A S, Mandell B F, Fessler B J. Alveolar hemorrhage in systemic lupus erythematosus: presentation and management. Chest. 2000; 118 1083-1090
48 Chang M Y, Fang J T, Chen Y C, Huang C C. Diffuse alveolar hemorrhage in systemic lupus erythematosus: a single center retrospective study in Taiwan. Ren Fail. 2002; 24 791-802
49 Schwab E P, Schumacher H R, Freundlich B, Callegari P E. Pulmonary alveolar hemorrhage in systemic lupus erythematosus. Semin Arthritis Rheum. 1993; 23 8-15
50 Erickson R W, Franklin W A, Emlen W. Treatment of hemorrhagic lupus pneumonitis with plasmapheresis. Semin Arthritis Rheum. 1994; 24 114-123
51 Horiki T, Fuyuno G, Ishii M et al.. Fatal alveolar hemorrhage in a patient with mixed connective tissue disease presenting polymyositis features. Intern Med. 1998; 37 554-560
52 Wilson W A, Gharavi A E, Koike T et al.. International consensus statement on preliminary classification criteria for definite antiphospholipid syndrome: report of an international workshop. Arthritis Rheum. 1999; 42 1309-1311
53 Espinosa G, Cervera R, Font J, Asherson R A. The lung in the antiphospholipid syndrome. Ann Rheum Dis. 2002; 61 195-198
54 Asherson R A, Cervera R, Piette J C et al.. Catastrophic antiphospholipid syndrome: clinical and laboratory features of 50 patients. Medicine (Baltimore). 1998; 77 195-207
55 Gertner E, Lie J T. Pulmonary capillaritis, alveolar hemorrhage, and recurrent microvascular thrombosis in primary antiphospholipid syndrome. J Rheumatol. 1993; 20 1224-1228
56 Chung M H, Lee H G, Kwon S S, Kim Y S, Park S H. Pulmonary arterial aneurysms in primary antiphospholipid antibody syndrome. J Comput Assist Tomogr. 2002; 26 608-612
57 Waterer G W, Latham B, Waring J A, Gabbay E. Pulmonary capillaritis associated with the antiphospholipid antibody syndrome and rapid response to plasmapheresis. Respirology. 1999; 4 405-408
58 Salama A D, Pusey C D. Immunology of anti-glomerular basement membrane disease. Curr Opin Nephrol Hypertens. 2002; 11 279-286
59 Donaghy M, Rees A J. Cigarette smoking and lung haemorrhage in glomerulonephritis caused by autoantibodies to glomerular basement membrane. Lancet. 1983; 2 1390-1393
60 Salama A D, Dougan T, Levy J B et al.. Goodpasture's disease in the absence of circulating anti-glomerular basement membrane antibodies as detected by standard techniques. Am J Kidney Dis. 2002; 39 1162-1167
61 Merkel P A. Drug-induced vasculitis. Rheum Dis Clin North Am. 2001; 27 849-862
62 Yermakov V M, Hitti I F, Sutton A L. Necrotizing vasculitis associated with diphenylhydantoin: two fatal cases. Hum Pathol. 1983; 14 182-184
63 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
64 Elkayam O, Yaron M, Caspi D. Minocycline-induced autoimmune syndromes: an overview. Semin Arthritis Rheum. 1999; 28 392-397
65 Derk C T, Jimenez S A. Goodpasture-like syndrome induced by D-penicillamine in a patient with systemic sclerosis: report and review of the literature. J Rheumatol. 2003; 30 1616-1620
66 Afessa B, Cowart R G, Koenig S M. Alveolar hemorrhage in IgA nephropathy treated with plasmapheresis. South Med J. 1997; 90 237-239

Ulrich SpecksM.D.

Division of Pulmonary and Critical Care Medicine, Mayo Clinic

200 First St. SW

Rochester, MN 55905

Email: specks.ulrich@mayo.edu