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Clin Exp Rheumatol. 2012 Jan-Feb;30(1 Suppl 70):S77-82. Epub 2012 May 11.

Alveolar haemorrhage in ANCA-associated vasculitides: 80 patients' features and prognostic factors.

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1
Department of Internal Medicine, Hôpital Cochin, Assistance Publique, Hôpitaux de Paris, Université Paris-Descartes, Paris, France. akostianovsky@gmail.com

Abstract

OBJECTIVES:

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.

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.

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 (pulmo-renal syndrome): 37/49 GPA (Wegener) (75.5% of all GPA (Wegener)), 19/21 MPA (90.4% of all MPA), 3/8 had CSS and 2/2 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.

CONCLUSIONS:

As previously demonstrated by the Five-Factor Score, AH alone is not predictive of poor prognosis, unlike kidney involvement, which dictates a poor outcome.

PMID:
22640651
[Indexed for MEDLINE]

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