Elsevier

Clinics in Chest Medicine

Volume 25, Issue 3, September 2004, Pages 583-592
Clinics in Chest Medicine

Diffuse alveolar hemorrhage

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Definition

The term “diffuse alveolar hemorrhage” refers to a distinct form of pulmonary hemorrhage that originates from the pulmonary microcirculation (including alveolar capillaries, arterioles, and venules). Although alveolar hemorrhage can be focal, there are generally multiple areas of involvement, and thus the term DAH is preferred. DAH must be distinguished from other causes of pulmonary hemorrhage that are caused by localized abnormalities in the lung (eg, bronchiectasis, malignancy, infection)

Etiology

DAH can be caused by many conditions, with most etiologies sharing a common pathophysiologic theme: damage to the alveolar microcirculation. Any source of injury to the alveolar microcirculation theoretically can cause alveolar hemorrhage. The cause may be lung specific (eg, diffuse alveolar damage, infection) or more generalized (eg, vasculitis). Most cases of DAH are associated with a neutrophilic infiltration of the alveolar wall centering on capillaries and venules [1]. This “capillaritis”

Clinical presentation

The usual presentation of DAH includes hemoptysis, alveolar infiltrates on chest radiograph, and anemia. Many cases do not have a classic clinical picture, however, and the clinical suspicion for DAH should remain whenever any of the previously mentioned features is present.

Histopathology

Alveolar hemorrhage must be distinguished from other causes of red blood cell accumulation in the alveolar space, most notably surgical trauma at the time of biopsy. True alveolar hemorrhage often demonstrates intra-alveolar fibrin and hemosiderin in the alveolar walls and hemosiderin-laden alveolar macrophages (Fig. 2) [4]. Hemosiderin, a product of hemoglobin degradation, appears at least 48 hours after bleeding and is helpful in distinguishing DAH from surgical trauma. There also may be

Approach to diagnosis

DAH represents a medical emergency. A thoughtful and thorough approach to the diagnosis of DAH is of critical importance to appropriate management. There are two important goals of the clinical evaluation: (1) establishing the diagnosis of DAH and (2) identifying the underlying cause. Although individual cases of DAH may require modifications, the general approach outlined next is recommended.

Specific causes of diffuse alveolar hemorrhage

The management of DAH is discussed in the context of the underlying cause. The following section presents the most common causes of DAH individually and reviews the pathophysiology, diagnosis, and treatment in each case. The most common treatment recommendations for the most common causes of DAH are summarized in Table 1.

Summary

DAH represents a medical emergency, and clinicians must have an expedient approach to the identification of DAH and its underlying cause. There are many causes of DAH, including vasculitides, such as Wegener's granulomatosis and microscopic polyangiitis, Goodpasture's syndrome, collagen vascular disease, and idiopathic conditions. Careful attention to the medical history, physical examination, and targeted laboratory evaluation often suggests the underlying cause. Patients in whom the diagnosis

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References (74)

  • J. Bar et al.

    Pulmonary-renal syndrome in systemic sclerosis

    Semin Arthritis Rheum

    (2001)
  • N. Milman et al.

    Idiopathic pulmonary haemosiderosis: epidemiology, pathogenic aspects and diagnosis

    Respir Med

    (1998)
  • S. Kalra et al.

    Alveolar hemorrhage as a complication of treatment with abciximab

    Chest

    (2001)
  • S.S. Dhillon et al.

    Diffuse alveolar hemorrhage and pulmonary capillaritis due to propylthiouracil

    Chest

    (1999)
  • D.S. Matloff et al.

    D-Penicillamine-induced Goodpasture's-like syndrome in primary biliary cirrhosis: successful treatment with plasmapheresis and immunosuppressives

    Gastroenterology

    (1980)
  • I. Raz et al.

    Pulmonary manifestations in Behçet's syndrome

    Chest

    (1989)
  • S. Kathuria et al.

    Fatal pulmonary Henoch-Schönlein syndrome

    Chest

    (1982)
  • S.J. Robboy et al.

    Pulmonary hemorrhage syndrome as a manifestation of disseminated intravascular coagulation: analysis of ten cases

    Chest

    (1973)
  • W.D. Travis et al.

    A clinicopathologic study of 34 cases of diffuse pulmonary hemorrhage with lung biopsy confirmation

    Am J Surg Pathol

    (1990)
  • T.J. Franks et al.

    Pulmonary capillaritis

    Curr Opin Pulm Med

    (2000)
  • C.A. Jennings et al.

    Diffuse alveolar hemorrhage with underlying isolated, pauciimmune pulmonary capillaritis

    Am J Respir Crit Care Med

    (1997)
  • E.J. Mark et al.

    Pulmonary capillaritis and hemorrhage in patients with systemic vasculitis

    Arch Pathol Lab Med

    (1985)
  • P.W. Ewan et al.

    Detection of intrapulmonary hemorrhage with carbon monoxide uptake: application in Goodpasture's syndrome

    N Engl J Med

    (1976)
  • F.W. Kahn et al.

    Diagnosis of pulmonary hemorrhage in the immunocompromised host

    Am Rev Respir Dis

    (1987)
  • W.D. Travis et al.

    Surgical pathology of the lung in Wegener's granulomatosis: review of 87 open lung biopsies from 67 patients

    Am J Surg Pathol

    (1991)
  • W.D. Travis et al.

    Diffuse pulmonary hemorrhage: an uncommon manifestation of Wegener's granulomatosis

    Am J Surg Pathol

    (1987)
  • A. Schnabel et al.

    Subclinical alveolar bleeding in pulmonary vasculitides: correlation with indices of disease activity

    Eur Respir J

    (1999)
  • A.P. Greening et al.

    Serial estimations of carbon monoxide diffusing capacity in intrapulmonary haemorrhage

    Clin Sci (Lond)

    (1981)
  • D. Jayne et al.

    A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies

    N Engl J Med

    (2003)
  • D.R. Jayne et al.

    Intravenous immunoglobulin for ANCA-associated systemic vasculitis with persistent disease activity

    QJM

    (2000)
  • C. Thomas-Golbanov et al.

    Novel therapies in vasculitis

    Expert Opin Investig Drugs

    (2001)
  • J.B. Hall et al.

    Vasculitis and glomerulonephritis: a subgroup with an antineutrophil cytoplasmic antibody

    Aust N Z J Med

    (1984)
  • S. Zashin et al.

    Microscopic polyarteritis: a forgotten aetiology of haemoptysis and rapidly progressive glomerulonephritis

    Ann Rheum Dis

    (1990)
  • S.J. Haworth et al.

    Pulmonary haemorrhage complicating Wegener's granulomatosis and microscopic polyarteritis

    Br Med J (Clin Res Ed)

    (1985)
  • A.D. Betensley et al.

    Factor viia for alveolar hemorrhage in microscopic polyangiitis

    Am J Respir Crit Care Med

    (2002)
  • J.W. Leatherman et al.

    Alveolar hemorrhage syndromes: diffuse microvascular lung hemorrhage in immune and idiopathic disorders

    Medicine (Baltimore)

    (1984)
  • C.O. Savage et al.

    Antiglomerular basement membrane antibody mediated disease in the British Isles 1980–4

    Br Med J (Clin Res Ed)

    (1986)
  • Cited by (167)

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    Dr. Collard is supported by an institutional grant from the Pulmonary Fibrosis Foundation.

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