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Semin Respir Crit Care Med 2011; 32(3): 254-263
DOI: 10.1055/s-0031-1279823
© Thieme Medical Publishers

Lung Vasculitis and Alveolar Hemorrhage: Pathology

Gregory A. Fishbein1 , Michael C. Fishbein2
  • 1David Geffen School of Medicine at UCLA, Los Angeles, California
  • 2Department of Pathology and Laboratory Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California
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Publication History

Publication Date:
14 June 2011 (online)

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ABSTRACT

Pulmonary vasculitides are a diverse group of limited and systemic disorders associated with inflammation of pulmonary vessels and parenchyma. These diseases often have distinctive clinical, serological, and histopathological features—extrapulmonary sites of involvement, circulating autoantibodies, predispositions for small or large vessels, and others. Some have characteristic inflammatory lesions; others are characterized by the absence of such lesions. Frequently pathological findings overlap, rendering classification, and diagnosis a challenge.

The anti-neutrophil cytoplasmic antibody (ANCA)-associated small-vessel diseases constitute the major pulmonary vasculitides. These include Wegener granulomatosis (WG), Churg Strauss syndrome (CSS), and microscopic polyangiitis (MPA). Less frequently, diseases such as polyarteritis nodosa, Takayasu arteritis, Behçet syndrome, and connective tissue diseases may involve pulmonary vessels, but these entities are better associated with extrapulmonary disease.

Diffuse alveolar hemorrhage (DAH) is a severe manifestation of pulmonary vasculitis. DAH is most commonly seen in small-vessel vasculitides, specifically MPA and WG. Other syndromes associated with DAH include Goodpasture syndrome, Henoch-Schönlein purpura, and systemic lupus erythematosus. Less commonly, DAH may be secondary to infection or drugs/toxins. Furthermore, in the absence of discernable systemic disease, DAH may be idiopathic—referred to as isolated pulmonary capillaritis (IPC) or idiopathic pulmonary hemosiderosis (IPH), depending on the presence of capillaritis.

KEYWORDS

Vasculitis - Wegener granulomatosis - microscopic polyangiitis - Churg-Strauss syndrome - diffuse alveolar hemorrhage

REFERENCES

  • 1 Lanham J, Elkon K, Pusey C, Hughes G. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome.  Medicine. 1984;  63 (2) 65-81
    CrossrefPubMedGoogle Scholar
  • 2 Mark E, Flieder D, 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 (4) 450-458
    CrossrefPubMedGoogle Scholar
  • 3 Jennette J, Falk R, Andrassy K et al.. Nomenclature of systemic vasculitides.  Arthritis Rheum. 1994;  37 (2) 187-192
    CrossrefPubMedGoogle Scholar
  • 4 Leavitt R, Fauci A, Bloch D et al.. The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis.  Arthritis Rheum. 1990;  33 (8) 1101-1107
    CrossrefPubMedGoogle Scholar
  • 5 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 (4) 315-333
    CrossrefPubMedGoogle Scholar
  • 6 Yousem S, Lombard C. The eosinophilic variant of Wegener's granulomatosis.  Hum Pathol. 1988;  19 (6) 682-688
    CrossrefPubMedGoogle Scholar
  • 7 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 (7) 794-801
    CrossrefPubMedGoogle Scholar
  • 8 Masi A, Hunder G, Lie J et al.. The American college of rheumatology 1990 criteria for the classification of chrug-strauss syndrome (allergic granulomatosis and angitis).  Arthritis Rheum. 1990;  33 (8) 1094-1100
    CrossrefPubMedGoogle Scholar
  • 9 Pel G, Tirabassi G, Pattoneri P, Pavone L, Garini G, Bruschi G. Cardiac involvement in the Churg-Strauss syndrome.  Am J Cardiol. 2006;  97 (10) 1519-1524
    CrossrefPubMedGoogle Scholar
  • 10 Kane G, Keogh K. Involvement of the heart by small and medium vessel vasculitis.  Curr Opin Rheumatol. 2009;  21 (1) 29-34
    CrossrefPubMedGoogle Scholar
  • 11 Hellemans S, Dens J, Knockaert D. Coronary involvement in the Churg-Strauss syndrome.  Heart. 1997;  77 (6) 576-578
    PubMedGoogle Scholar
  • 12 Guillevin L, Lhote F, Gallais V et al.. Gastrointestinal tract involvement in polyarteritis nodosa and Churg-Strauss syndrome.  Ann Med Interne (Paris). 1995;  146 (4) 260-267
    PubMedGoogle Scholar
  • 13 Sinico R, Di Toma L, Maggiore U et al.. Renal involvement in Churg-Strauss syndrome.  Am J Kidney Dis. 2006;  47 (5) 770-779
    CrossrefPubMedGoogle Scholar
  • 14 Jennette J, Falk R. Small-vessel vasculitis.  N Engl J Med. 1997;  337 (21) 1512-1523
    CrossrefPubMedGoogle Scholar
  • 15 Niles J, Bottinger E, Saurina G et al.. The syndrome of lung hemorrhage and nephritis is usually an ANCA-associated condition.  Arch Intern Med. 1996;  156 (4) 440-445
    CrossrefPubMedGoogle Scholar
  • 16 Matsumoto T, Homma S, Okada M et al.. The lung in polyarteritis nodosa: a pathologic study of 10 cases.  Hum Pathol. 1993;  24 (7) 717-724
    CrossrefPubMedGoogle Scholar
  • 17 Numano F, Ohta N, Sasazuki T. HLA and clinical manifestations in Takayasu disease.  Jpn Circ J. 1982;  46 (2) 184-189
    CrossrefPubMedGoogle Scholar
  • 18 Kerr G, Hallahan C, Giordano J et al.. Takayasu arteritis.  Ann Intern Med. 1994;  120 (11) 919-929
    CrossrefPubMedGoogle Scholar
  • 19 Endo M, Tomizawa Y, Nishida H et al.. Angiographic findings and surgical treatments of coronary artery involvement in Takayasu arteritis.  J Thorac Cardiovasc Surg. 2003;  125 (3) 570-577
    CrossrefPubMedGoogle Scholar
  • 20 Uzun O, Akpolat T, Erkan L. Pulmonary vasculitis in behcet disease: a cumulative analysis.  Chest. 2005;  127 (6) 2243-2253
    CrossrefPubMedGoogle Scholar
  • 21 Churg A, Carrington C, Gupta R. Necrotizing sarcoid granulomatosis.  Chest. 1979;  76 (4) 406-413
    CrossrefPubMedGoogle Scholar
  • 22 Lynch III J, Kazerooni E, Gay S. Pulmonary sarcoidosis.  Clin Chest Med. 1997;  18 (4) 755-785
    CrossrefPubMedGoogle Scholar
  • 23 Isenberg J, Goldstein H, Korn A, Ozeran R, Rosen V. Pulmonary vasculitis–an uncommon complication of ulcerative colitis. Report of a case.  N Engl J Med. 1968;  279 (25) 1376-1377
    CrossrefPubMedGoogle Scholar
  • 24 Pagnoux C. Vasculitides secondary to infections.  Clin Exper Rheum. 2006;  24 (2) 71-81
    PubMedGoogle Scholar
  • 25 Milburn D S, Myers C W. Tryptophan toxicity: a pharmacoepidemiologic review of eosinophilia-myalgia syndrome.  DICP. 1991;  25 (11) 1259-1262
    PubMedGoogle Scholar
  • 26 Colby T, Fukuoka J, Ewaskow S, Helmers R, Leslie K. Pathologic approach to pulmonary hemorrhage.  Ann Diagn Pathol. 2001;  5 (5) 309-319
    CrossrefPubMedGoogle Scholar
  • 27 Green R, Ruoss S, Kraft S, Duncan S, Berry G, Raffin T. Pulmonary capillaritis and alveolar hemorrhage. Update on diagnosis and management.  Chest. 1996;  110 (5) 1305-1316
    CrossrefPubMedGoogle Scholar
  • 28 Kadokura M, Colby T, Myers J et al.. Pathologic comparison of video-assisted thoracic surgical lung biopsy with traditional open lung biopsy.  J Thorac Cardiovasc Surg. 1995;  109 (3) 494-498
    CrossrefPubMedGoogle Scholar
  • 29 Sherman J, Winnie G, Thomassen M, Abdul-Karim F, Boat T. Time course of hemosiderin production and clearance by human pulmonary macrophages.  Chest. 1984;  86 (3) 409-411
    CrossrefPubMedGoogle Scholar
  • 30 Lendrum A. Pulmonary haemosiderosis: pathological aspects.  Proc R Soc Med. 1960;  53 338-340
    PubMedGoogle Scholar
  • 31 Lara A, Schwarz M. Diffuse alveolar hemorrhage.  Chest. 2010;  137 (5) 1164-1171
    CrossrefPubMedGoogle Scholar
  • 32 Gmurczyk A, Ahya S, Goldschmidt R, Kim G, Ho L, Nash K. A case of simultaneous, biopsy-proven, classic, ANCA-positive Wegener's granulomatosis and anti-GBM disease, but without detectable circulating anti-GBM antibodies.  ScientificWorldJournal. 2010;  10 1078-1083
    CrossrefPubMedGoogle Scholar
  • 33 Milman N, Pedersen F M. Idiopathic pulmonary haemosiderosis. Epidemiology, pathogenic aspects and diagnosis.  Respir Med. 1998;  92 (7) 902-907
    CrossrefPubMedGoogle Scholar

Michael C FishbeinM.D. 

Department of Pathology and Laboratory Medicine, David Geffen School of Medicine at UCLA

Rm. 13-145H CHS, 10833 Le Conte Ave., Los Angeles, CA 90095

Email: mfishbein@mednet.ucla.edu