Clinicalfeaturesofeosinophilicgranulomatosis with polyangiitis in ANCA-positive and ANCA-negative patients

Download in PDF


To compare clinical presentation at diagnosis and longterm outcomes of eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) patients according to antineutrophil cytoplasmic antibody (ANCA) status.

Material and methods

EGPA was classified according to ACR 1990 criteria and the Revised CHCC Nomenclature. Serum ANCA was studied by ELISA.


We followed 93 patients with EGPA (64 females and 29 males at a mean age of 46.6±13.8 years). Thirty seven of 93 patients (39.8%) were ANCA-positive. Demographic characteristics were similar in the ANCA-negative and ANCApositive patients. ANCA-positive patients had significantly more frequent myalgia (59.5% and 29.4%, respectively; р=0.02) and mononeuritis multiplex (45.9% and 17.6%, respectively; р=0.01), than ANCA-negative patients, while the frequency of cardiac and other organs involvement was not significantly different. The difference in occurrence of kidney disease between the two groups did not reach statistical significance. The frequency of vasculitis relapses was 21.0 per 100 patient-years in the ANCA-positive group versus 19.6 per 100 patient-years in the ANCA-negative group (P=0.4). ANCA-positive patients had additional immunosuppressive therapy at diagnosis more frequently, than ANCA-negative patients (р=0.02) due to a higher vasculitis activity and a more frequent peripheral nervous system involvement.


The presence of ANCA in patients with EGPA was associated with a significantly more frequent mononeuritis multiplex and a significantly higher vasculitis activity, while the frequency of vasculitis relapses was similar in both groups.

Key words

.Systemic vasculitis, eosinophilic granulomatosis with polyangiitis, antineutrophil cytoplasmic antibody.