Five factor score of more than 1 is associated with relapse during the first 2 year-follow up in patients with eosinophilic granulomatosis with polyangiitis.

Abstract

We investigated what variables at diagnosis might be associated with the relapse of eosinophilic granulomatosis with polyangiitis (EGPA) and whether five factor score (FFS) at diagnosis might predict it. We reviewed the medical records of 30 patients with EGPA having results of myeloperoxidase (MPO)-antineutrophil cytoplasmic antibodies (ANCA) and proteinase 3 (PR3)-ANCA and having achieved remission during the first therapeutic regimen administration within follow-up duration for at least more than 2 years. Clinical manifestations, the presence of relapse and items for FFS at diagnosis were collected and compared among groups. The mean age of patients (18 women) was 50.3 years and the mean follow-up duration was 2175 days. EGPA patients having MPO-ANCA had shown higher frequency of skin involvement and proteinuria over 1 g/day than those having PR3-ANCA and no ANCA. EGPA patients with relapse had more frequent baseline MPO-ANCA and kidney involvement,including proteinuria over 1 g/day and renal insufficiency and higher FFS at diagnosis than those without. However, only FFS showed the predictive significance for relapse (Exp(B) 2.624, P = 0.014) on Cox Hazard model analysis. When the cutoff of FFS for the relapse was set as 1, relapse was identified more frequently in patients with FFS ≥ 1 than those with FFS <1 (68.8% vs. 7.1%, P = 0.001; relative risk 28.6). In this study, FFS at diagnosis was associated with relapse and FFS ≥ 1 was the independent predictor of relapse during follow-up for more than 2 years.