The Survival of Patients With Alveolar Hemorrhage Secondary to Antineutrophil Cytoplasmic Antibody–associated Vasculitis

Diffuse alveolar hemorrhage (DAH) is a clinical syndrome that can be caused by a variety of nonimmune-mediated and immune-mediated etiologies, including antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). DAH is one of the most severe and life-threatening manifestations of AAV, occurring in up to 25% of patients with AAV1. Some studies have suggested that DAH may be more common in microscopic polyangiitis (MPA) than in granulomatosis with polyangiitis (GPA), but reported frequencies may be affected by factors including variable epidemiology of MPA versus GPA in different regions of the world, applied disease definitions, and local referral patterns to tertiary care centers2,3,4,5. Preexisting airway disease has been reported as an independent risk factor for the development of DAH in AAV3. Irrespective of the frequency and severity of DAH in AAV, there is only a limited number of studies addressing the prognosis of DAH in patients with AAV (Table 1). The combination of glucocorticoids (GC) with cyclophosphamide (CYC) or rituximab (RTX), variably combined with plasma exchange (PLEX), is usually effective to induce remission of the disease in the majority of patients, and the main causes of death are infections or cardiovascular complications rather than vasculitis activity2,6,7,8,9,10. View this table: Table 1. The published cohorts of DAH occurring in patients with AAV (only studies with ≥ 20 patients are reported). In this issue of The Journal , Tang and colleagues11 report on their analysis of clinical features at presentation and the main prognostic factors of DAH in a single-center cohort of 92 patients with MPA. This is the largest cohort of DAH in patients with MPA published so far. Interestingly, in this cohort, the mortality was the highest reported for DAH in patients with … Address correspondence to Dr. A. Berti, Rheumatology Department, Santa Chiara Hospital, Largo Medaglie D'Oro 9, 38121, Trento, Trentino, Italy. Email: alvise.berti{at}apss.tn.it.