[Long-term development of children with Schönlein-Henoch purpura associated with anti-neutrophil cytoplasmic antibodies].

OBJECTIVE

The purpose of this study was to assess the frequency of antineutrophil cytoplamic antibodies (ANCA) in Schönlein-Henoch purpura (SHP) and its long-term significance.

PATIENTS AND METHODS

IgG and IgA classes of ANCA were studied by indirect immunofluorescence (IIF) and IgG-ANCA against myeloperoxidase (MPO) and against proteinase-3 (PR-3) were determined by ELISA in 50 children with SHP. Eight (16%) of the patients had renal involvement during the acute phase, but none had a permanent nephropathy after a 7-17 year follow-up.

RESULTS

Positive IgG ANCA were found in 5 (10%) of the cases and only one of these children also had IgA ANCA. The ELISA against MPO and P-3 was negative in all patients. None of the 5 patients with ANCA showed nephropathy during the acute phase nor relapses or permanent nephropathy.

CONCLUSIONS

A minority of children with SHP are positive for ANCA and, in the absence of nephropathy, this is not associated with a bad long-term prognosis.