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Semin Respir Crit Care Med 2004; 25(5): 547-555
DOI: 10.1055/s-2004-836146
Copyright © 2004 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA.

Pulmonary Capillaritis

Augustine S. Lee1 , Ulrich Specks1
  • 1Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, Minnesota
Further Information

Publication History

Publication Date:
09 November 2004 (online)

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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

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
    CrossrefPubMedSearch in Google Scholar
  • 2 Mark E J, Ramirez J F. Pulmonary capillaritis and hemorrhage in patients with systemic vasculitis.  Arch Pathol Lab Med. 1985;  109 413-418
    PubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    PubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    PubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    PubMedSearch in Google Scholar
  • 11 Gertner E. Diffuse alveolar hemorrhage in the antiphospolipid syndrome: spectrum of disease and treatment.  J Rheumatol. 1999;  26 805-807
    PubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    PubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    PubMedSearch in Google Scholar
  • 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
    PubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 21 Gaskin G, Pusey C D. Plasmapheresis in antineutrophil cytoplasmic antibody-associated systemic vasculitis.  Ther Apher. 2001;  5 176-181
    PubMedSearch in Google Scholar
  • 22 Jayne D. Current attitudes to the therapy of vasculitis.  Kidney Blood Press Res. 2003;  26 231-239
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    PubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    PubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    PubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    PubMedSearch in Google Scholar
  • 43 Clutterbuck E J, Pusey C D. Severe alveolar haemorrhage in Churg-Strauss syndrome.  Eur J Respir Dis. 1987;  71 158-163
    PubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    PubMedSearch in Google Scholar
  • 46 Koh W H, Thumboo J, Boey M L. Pulmonary haemorrhage in Oriental patients with systemic lupus erythematosus.  Lupus. 1997;  6 713-716
    PubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 50 Erickson R W, Franklin W A, Emlen W. Treatment of hemorrhagic lupus pneumonitis with plasmapheresis.  Semin Arthritis Rheum. 1994;  24 114-123
    PubMedSearch in Google Scholar
  • 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
    PubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 53 Espinosa G, Cervera R, Font J, Asherson R A. The lung in the antiphospholipid syndrome.  Ann Rheum Dis. 2002;  61 195-198
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 55 Gertner E, Lie J T. Pulmonary capillaritis, alveolar hemorrhage, and recurrent microvascular thrombosis in primary antiphospholipid syndrome.  J Rheumatol. 1993;  20 1224-1228
    PubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 58 Salama A D, Pusey C D. Immunology of anti-glomerular basement membrane disease.  Curr Opin Nephrol Hypertens. 2002;  11 279-286
    CrossrefPubMedSearch in Google Scholar
  • 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
    PubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 61 Merkel P A. Drug-induced vasculitis.  Rheum Dis Clin North Am. 2001;  27 849-862
    CrossrefPubMedSearch in Google Scholar
  • 62 Yermakov V M, Hitti I F, Sutton A L. Necrotizing vasculitis associated with diphenylhydantoin: two fatal cases.  Hum Pathol. 1983;  14 182-184
    PubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 64 Elkayam O, Yaron M, Caspi D. Minocycline-induced autoimmune syndromes: an overview.  Semin Arthritis Rheum. 1999;  28 392-397
    CrossrefPubMedSearch in Google Scholar
  • 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
    PubMedSearch in Google Scholar
  • 66 Afessa B, Cowart R G, Koenig S M. Alveolar hemorrhage in IgA nephropathy treated with plasmapheresis.  South Med J. 1997;  90 237-239
    PubMedSearch in Google Scholar

Ulrich SpecksM.D. 

Division of Pulmonary and Critical Care Medicine, Mayo Clinic

200 First St. SW

Rochester, MN 55905

Email: specks.ulrich@mayo.edu