A retrospective review of 34 cases of DAH revealed nearly one-third of the cases were caused by granulomatosis with polyangiitis (previously termed Wegener granulomatosis).
About
- Blood acutely fills alveolar spaces at multiple sites.
Aetiology
- Disruption of the alveolar-capillary basement membrane.
- Bleeding from pulmonary rather than bronchial vasculature
- Blood escapes through the capillary walls
- Caused by injury, inflammation, hypertension
- Bleeding from arterioles, venules or alveolar septal capillaries
Associations
- Pulmonary vasculitis including ANCA associated pulmonary vasculitides
- Microscopic polyangiitis
- Wegener's granulomatosis
- Churg-Strauss vasculitis
- Goodpasture syndrome
- Henoch Schonlein purpura
- SLE
- Mixed connective tissue disease
- Coagulopathy
- Cryoglobulinaemia (HCV/HIV)
- HIV : associated with Kaposi sarcoma
- Inhaled toxins.
- Mitral valve disease and heart failure
- Pulmonary veno-occlusive disease
- Haemorrhage with infection/alveolar damage/malignancy
- Antiphospholipid syndrome
- Allogeneic haematopoietic stem cell transplantation (allo-HSCT)
Triad
- Lung opacities - lung opacities, dyspnoea, haemoptysis, anaemia
- Chest CT - diffuse ground glass, alveolar infiltrates, no effusion, normal heart
- Bronchoscopy + BAL
Clinical
- Acute onset hypoxaemia, dyspnoea and/or haemoptysis
- Shock, renal failure
Investigations
- FBC: falling Hb and haematocrit
- LDH increased
- ANA, dsDNA, ANCA, anti-GBM, APL antibodies
- Clotting screen: coagulopathy
- CXR: diffuse alveolar infiltrates
- ABG: Respiratory failure
- Echocardiography to exclude cardiac disease
- Bronchoscopy: progressive bloody bronchoalveolar lavage (BAL)
- CT: ground glass changes and progressive fibrosis
Complications
- Respiratory failure
- DIC
- Organizing pneumonia
Management
- ABC, Oxygen, Supportive care, Ventilation, Antibiotics for infection
- Withdrawal of offending drugs, Treat any coagulopathy
- High-dose steroids, immunosuppressant drugs and plasmapheresis
- Systemic vasculitis: Cyclophosphamide/Rituximab
- Anti GBM: Cyclophosphamide/PLEX
- Antiphospholipid: Rituximab
References