[Antineutrophil cytoplasmic antibodies (ANCA): clinico-pathologic correlations iin a series of 82 cases].

OBJECTIVE

Antineutrophil cytoplasmic antibodies (ANCA) have been described in a great variety of diseases in addition to the ANCA-associated systemic vasculitis (Wegener's granulomatosis (WG), microscopic polyangiitis (MPA) and Churg-Strauss syndrome (CSS)) and the pauci-immune rapidly progressive glomerulonephritis (RPGN). The objective of this work is to describe the clinical-pathological diagnosis of a series of patients with ANCA.

MATERIAL AND

METHODS

Retrospective review of patients with positive ANCA by indirect immunofluorescence in our hospital between January 1997 and December 1998.

RESULTS

We identify 82 patients with ANCA. Six cases (7.3%) showed a C-ANCA pattern, 19 (23.2%) a P-ANCA pattern and 57 (69.5%) an atypical pattern. Eight patients (9.7%) had an ANCA-associated systemic vasculitis (5 WG, 2 MPA and 1 SCS). Four patients (4.9%) had a pauci-immune RPGN without systemic vasculitis. One only case (1.2%) in our series presented a C-ANCA pattern without evidence of an ANCA-associated systemic vasculitis or a pauci-immune RPGN.

CONCLUSIONS

The atypical pattern is the most frequent in patients with ANCA. A minority of patients with ANCA have an ANCA-associated systemic vasculitis (WG, MPA or SCS) or a pauci-immune RPGN. The occurrence of a C-ANCA pattern is exceptional in patients without an ANCA-associated systemic vasculitis or a pauci-immune RPGN.