Diffuse Alveolar Haemorrhage

Potentially life-threatening hypoxaemic respiratory failure characterised by:

Epidemiology and Risk Factors

Pathophysiology

Aetiology

Precipitants include:

  • Pulmonary capillaritis secondary to autoimmune disease, including:
    • Systemic vasculitis
      • Granulomatosis with Polyangiitis
      • Churg-Strauss syndrome
      • Microscopic polyangiitis
    • Primary immune complex-mediated vasculitis
      • Goodpasture’s syndrome
      • Henoch-Schonlein purpura
    • Secondary vasculitis
      • SLE
      • RA
      • APS
      • Mixed connective tissue disease
      • POlymyositis
      • Dermatomyositis
    • Essential cryoglobulinaemia
    • Behcet’s disease
    • Anti-GBM disease
  • Coagulation disorders
  • Pulmonary vascular disease
    • MS
    • Pulmonary veno-occulisive disease
  • Drugs
    • Amiodarone
    • Nitrofurantoin
    • Chemotherapeutics
    • Propylthiouracil
  • Toxins
  • Transplantation
    • BMT
  • ARDS

Assessment

Presentation typically with:

  • Fever
  • Dyspnoea
  • Cough
    Haemoptysis is rare.
  • Blood alveolar lavage samples

History

Exam

Investigations

Bedside:

Laboratory:

A rheumatological screen consists of:

  • Initial investigations:
    • FBE
    • UEC
    • ESR
    • CRP
    • LFT
    • ANA
    • Rheumatoid factor
    • Anti-CCP antibodies
    • Vitamin D
    • Urinealysis
  • Second-line investigations
    For patients with strong clinical suspicion:
    • Anti-synthetase antibodies
    • Creatine kinase
    • Aldolase
    • Sjögren’s antibodies
      • SS-A
      • SS-B
    • Scleroderma antibodies
      • Anti-topoisomerase (Scl-70)
      • Anti-PM-1 antibody
      • Anti-centromere
    • Anti-dsDNA antibodies
    • Myositis-associated antibodies
      • Jo-1
      • PL-7
      • PL-12
    • ANCA
    • Anti-melanoma differentiation-associated gene 5
    • Overlap antibodies
      PM-1.
  • Blood
    • FBE
    • Coagulation screen
    • Rheumatoid screen

Imaging:

Diffuse Alveolar Haemorrhage

  • CXR
  • High-resolution CT Chest

Other:

  • BAL
    Essential for diagnosis, and to exclude infection.

Diagnostic Approach and DDx

Management

  • Treat underlying cause
  • Immunosuppression
    Corticosteroids as first line therapy.

Resuscitation:

  • A
    • Intubation and mechanical ventilation

Specific therapy:

  • Pharmacological
    • Corticosteroids
      • Methylprednisolone 500mg Q6H for 4-5 days, followed by taper
    • Other immunosuppressants may be indicated
      • Methotrexate
      • MMF
      • Etanercept
  • Procedural
    • Plasmapheresis
      If indicated for the primary immunological precipitant, e.g. Goodpasture’s or vasculitis.
  • Physical

Supportive care:

Disposition:

Preventative:

Marginal and Ineffective Therapies

Anaesthetic Considerations

Complications

  • Death

Prognosis

Key Studies

References

  1. Park MS. Diffuse Alveolar Hemorrhage. Tuberc Respir Dis (Seoul). 2013 Apr;74(4):151–62.
  2. Case courtesy of Jan Frank Gerstenmaier. Radiopaedia.org. From the case: rID: 28582.