title: Long-term outcome of severe alveolar haemorrhage in ANCA-associated vasculitis: a retrospective cohort study.
abstract: OBJECTIVE
  Alveolar haemorrhage (AH) is a major cause of early death in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). There is a paucity of information regarding the outcomes of AAV patients presenting with severe AH.
  
  
  METHODS
  A retrospective cohort study. Patients with severe AH were identified from a case review of 824 AAV patients. Demography, presenting features, treatment, and outcomes are described.
  
  
  RESULTS
  Fifty-three patients (33 males, 20 females; median age 59 years) with severe AH were identified: 37 (69.8%) with granulomatosis with polyangiitis (Wegener's) and 16 with microscopic polyangiitis [36 proteinase 3 (PR3)-ANCA positive and 17 myeloperoxidase (MPO)-ANCA positive]. AH was the first disease manifestation in 46 (86.8%) patients. Assisted ventilation was required in 36 (67.9%), renal involvement was present in 52 (98.1%), and 28 (52.8%) required dialysis. Forty (75.5%) received plasma exchange. At 3 months, 44/53 (83.0%) were alive. The mean follow-up was 49 months when 31 (58.5%) were alive and 24 (45.3%) dialysis independent. Mortality was higher in those requiring dialysis at entry (57.1% vs. 24%, p = 0.02) and in patients aged > 65 years (71.4% vs. 30.8%, p = 0.01), and tended to be higher in those requiring intubation (54.5% vs. 32.2%, p = 0.1).
  
  
  CONCLUSIONS
  Severe AH was more commonly associated with PR3-ANCA (vs. MPO-ANCA) and strongly correlated with renal vasculitis. Current treatment of severe AH leads to remission but long-term mortality remains high. Concurrent renal failure and older age were associated with higher mortality.
authors: Department of Nephrology, General University Hospital and First Faculty of Medicine, Charles University, Prague, Czech Republic. zd.hruskova@seznam.cz
authors: Z
authors: Z
authors: Hruskova
authors: A L
authors: AL
authors: Casian
authors: P
authors: P
authors: Konopasek
authors: B
authors: B
authors: Svobodova
authors: D
authors: D
authors: Frausova
authors: V
authors: V
authors: Lanska
authors: V
authors: V
authors: Tesar
authors: D R W
authors: DR
authors: Jayne
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000150:complications* / Q000401:mortality*; D005260:Female; D006470:Hemorrhage / Q000209:etiology / Q000401:mortality* / Q000628:therapy; D006801:Humans; D008171:Lung Diseases / Q000209:etiology / Q000401:mortality* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D011650:Pulmonary Alveoli; D012189:Retrospective Studies; D055815:Young Adult