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Diffuse alveolar hemorrhage

  • Lesion

Bleeding into many alveoli at once, from autoimmune attack on the basement membrane or from capillaritis. It shows as hemoptysis, anaemia and hemosiderin.

Updated

What it is

Diffuse pulmonary hemorrhage syndromes flood the alveoli with blood. Three groups matter: anti-glomerular basement membrane disease with lung involvement (Goodpasture syndrome), vasculitis-associated hemorrhage (granulomatosis with polyangiitis, microscopic polyangiitis, lupus) and idiopathic pulmonary hemosiderosis. Pulmonary-renal syndromes (lung hemorrhage plus rapidly progressive glomerulonephritis) are emergencies.

Structure

  • Gross: heavy lungs with red-brown consolidation.
  • H&E: blood in alveoli, focal necrosis of alveolar walls, many hemosiderin-laden macrophages; later septal fibrosis and type II hyperplasia.
  • Immunofluorescence (Goodpasture): linear IgG and complement along basement membranes.
  • GPA: neutrophilic capillaritis, necrotising vasculitis, geographic necrosis and poorly formed granulomas with giant cells.

Step by step

  1. Autoantibodies (anti-GBM) or ANCA-activated neutrophils attack the alveolar capillary wall.
  2. In Goodpasture, smoke or infection may expose the hidden collagen epitope in the lung.
  3. Complement and neutrophils destroy the capillaries (capillaritis) and the basement membrane.
  4. Red cells pour into the alveoli; macrophages ingest them and convert haem iron to hemosiderin.
  5. The same attack in glomeruli causes crescentic glomerulonephritis.
  6. Repeated episodes leave fibrosis and iron deficiency.

Causes of diffuse alveolar hemorrhage

DiseaseAntibodyImmunofluorescenceOther clues
Anti-GBM (Goodpasture)Anti-α3 type IV collagenLinear IgGYoung male smokers, renal failure
Granulomatosis with polyangiitisPR3-ANCAPauci-immuneSinusitis, nasal destruction, granulomas
Microscopic polyangiitisMPO-ANCAPauci-immuneNo granulomas
Systemic lupus erythematosusANA, anti-dsDNAGranular immune complexesOther lupus features
Idiopathic pulmonary hemosiderosisNoneNegativeYoung children, anaemia

Markers

  • Prussian blue
  • Linear IgG
  • PR3-ANCA
  • MPO-ANCA

Clinical notes

  • Hemoptysis (sometimes absent despite major bleeding), falling haemoglobin, new bilateral infiltrates, rising DLCO.
  • Check urine sediment and creatinine; test anti-GBM and ANCA.
  • Treatment: plasma exchange (anti-GBM) and immunosuppression; outcomes improve with early diagnosis.

Test yourself

Think of the answer, then tap the question to see it.

What is the target antigen in Goodpasture syndrome?

The noncollagenous domain of the α3 chain of type IV collagen in glomerular and alveolar basement membranes.

What immunofluorescence pattern does anti-GBM disease show?

Linear deposits of IgG (and complement) along the basement membranes.

Which cells prove repeated alveolar bleeding, and how are they shown?

Hemosiderin-laden macrophages, highlighted blue by the Prussian blue iron stain.

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Educational content on the mechanisms of disease; not a diagnostic or treatment guide and no substitute for a health professional's advice. No doses are given.

Sources: Lung Diseases (MedlinePlus, US National Library of Medicine, 2025).

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