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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. 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 AAV, with an overall survival of 63.7% and 47.3% at 1 year and 5 years, respectively. Multivariate analysis identified age > 65 years, severe renal disease (serum creatinine > 500 umol/L), ratio of partial pressure of arterial O2 to fraction of inspired O2 (PaO2/ FiO2) < 300 mmHg, and involved lung area of ≥ 50% at presentation as independent risk factors for death in this cohort. The most frequent cause of death was infection, and 22% of patients died of active vasculitis. This report raises two questions: (1) Why was the mortality so much higher than that reported from other centers; and (2) was the mortality in these patients with MPA attributable only to DAH, or did other concomitant factors play a significant role? Several factors should be considered as a possible explanation for the higher reported mortality in this cohort. In contrast to most previous reports from Western countries, the patients comprising this report are Chinese, and the different genetic background of these patients might have influenced the disease phenotype, the response to treatment, and ultimately the course of the disease12. All patients included in this study by Tang and colleagues11 had a diagnosis of MPA, whereas most other reported cohorts comprised a mix of MPA, GPA, and eosinophilic GPA (EGPA). Only one previous cohort included just patients with MPA, yet the 1-year and 5-year survival was 82% and 68%, respectively13 (Table 1). In addition, being diagnosed with MPA, rather than GPA or EGPA, was not identified as a risk factor for death or for progression to respiratory failure in other cohorts of DAH in AAV2,8,14. Similarly, 96% of the patients were positive for myeloper-

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