What is the treatment for diffuse alveolar hemorrhage?

Management of Diffuse Alveolar Hemorrhage

The treatment of diffuse alveolar hemorrhage (DAH) requires prompt intervention with high-dose glucocorticoids plus either cyclophosphamide or rituximab as standard of care, with plasma exchange considered for cases with hypoxemia due to the high mortality risk. 1

Initial Management

• Immediate respiratory support to maintain adequate oxygenation, which may require mechanical ventilation in severe cases 1
• Control of bleeding through high-dose intravenous glucocorticoids (methylprednisolone 500-1000 mg/day for 3 days) as first-line therapy 1
• Assess for hypoxemia, as this indicates a more severe presentation with higher mortality risk 1

Specific Immunosuppressive Therapy

• For DAH associated with ANCA-associated vasculitis (AAV) or other autoimmune causes:
  • Combine high-dose glucocorticoids with either cyclophosphamide or rituximab 1
  • Consider avacopan as an adjunctive therapy to reduce glucocorticoid exposure, particularly in patients at high risk for steroid toxicity 2

Plasma Exchange Considerations

• Plasma exchange should be considered for patients with DAH who have hypoxemia 1
• Specific indications for plasma exchange include:
  • Patients with serum creatinine >3.4 mg/dl (>300 mmol/l) 2
  • Patients requiring dialysis or with rapidly increasing serum creatinine 2
  • Patients with diffuse alveolar hemorrhage who have hypoxemia 2
  • Patients with concomitant anti-glomerular basement membrane (GBM) disease 2

Adjunctive Therapies

• Platelet transfusions may be necessary to maintain adequate platelet counts 3
• Consider antifibrinolytic agents (e.g., aminocaproic acid) in refractory cases 4
• Intrapulmonary recombinant factor VIIa (rFVIIa) may be effective for refractory DAH when standard therapies have failed 4
• In extreme cases of life-threatening DAH unresponsive to medical management, extracorporeal membrane oxygenation (ECMO) may be considered as a rescue therapy 5

Etiology-Specific Management

• For infectious causes of DAH, appropriate antimicrobial therapy must be initiated promptly 6
• For DAH associated with hematopoietic cell transplantation, treatment includes supportive care, systemic corticosteroids, and platelet transfusions 3
• For refractory cases in systemic lupus erythematosus, consider intravenous immunoglobulin and plasmapheresis 7

Monitoring and Follow-up

• Monitor treatment response through:
  • Clinical symptoms improvement
  • Oxygenation parameters
  • Radiographic improvement 1
• For patients with AAV, continue maintenance immunosuppression after remission for 18 months to 4 years to prevent relapse 2, 1

Prognosis and Complications

• Mortality risk is higher in patients with hypoxemia and is more closely associated with the rate of hemoptysis rather than the quantity 1
• Risk factors for mortality in AAV-associated DAH include older age, severe kidney failure, degree of hypoxemia, and involvement of >50% of lung area 1
• Standard venous thromboprophylaxis should be commenced as soon as possible after bleeding has been controlled, as patients rapidly develop a prothrombotic state 2

Special Considerations

• DAH without hypoxemia generally has a more benign prognosis and responds as the underlying disease is controlled 1
• Patients with refractory disease should be referred to centers with expertise in vasculitis 1
• For kidney transplant candidates with history of AAV and DAH, delay transplantation until complete clinical remission for ≥6 months 1