Alveolar haemorrhage in ANCA-associated vasculitides: 80 patients%26apos; features and prognostic factors

作者：Kostianovsky A; Hauser T; Pagnoux C; Cohen P; Daugas E; Mouthon L; Miossec P; Cordier J F; Guillevin L^*

来源：Clinical and Experimental Rheumatology, 2012, 30(1): S77-S82.

• alveolar haemorrhage
• ANCA
• Churg-Strauss syndrome
• microscopic polyangiitis
• Wegener%26apos
• s granulomatosis

Objective. Alveolar haemorrhage (AH) can be a mild or life-threatening manifestation of antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV), but its prognostic impact and specific characteristics remain controversial. Our objective was to determine the prognostic value of AH in this context. %26lt;br%26gt;Methods. AH episodes that occurred, between 1991 and 2010, in AAV patients entered in the FVSG database were retrospectively analysed. Data on AH characteristics and outcome measures were collected on a specific form. %26lt;br%26gt;Results. Among the 80 cases analysed, AAV were 61.25% granulomatosis with polyangiitis (GPA) (Wegener), 26.25% microscopic polyangiitis (MPA), 10% Churg-Strauss syndrome and 2 (2.5%) unclassified. Mild or severe haemoptysis alone, or together with other clinical symptoms was present in 77(96.2%) patients before AAV diagnosis. Among 10 (12.5%) patients requiring mechanical ventilation, 4 had prior minor haemoptysis before abundant AH. Sixty-one (76.3%) patients had concomitant active rapid crescentic glomerulonephritis causing renal insufficiency (pulmorenal syndrome): 37149 GPA (Wegener) (75.5% of all GPA (Wegener)), 19121 MPA (90.4% of all MPA), 318 had CSS and 212 had unclassified vasculitis. The mean AH-to-treatment-onset interval was 5.9 days. Mean follow-up was 7.3 years. Forty-seven (58.8%) patients relapsed: 23 with AH and with (13) or without (10) other organ involvement, 24 with non-AH manifestation(s). Three patients underwent kidney transplantation. Sixteen (20%, 8 GPA (Wegener) and 8 MPA) patients died. No death resulted directly from the initial AH; 14 (87.5%) patients with pulmo-renal syndrome died. %26lt;br%26gt;Conclusion. As previously demonstrated by the Five-Factor Score, AH alone is not predictive of poor prognosis, unlike kidney involvement, which dicates a poor outcome.

• 出版日期2012-2