Article Text

Download PDFPDF

THU0338 Clinical presentation and outcomes of eosinophilic granulomatosis with polyangiitis: anca-negative versus anca-positive
  1. E Zagvozdkina,
  2. P Novikov,
  3. S Moiseev
  1. Sechenov First Moscow State Medical University, Moscow, Russia, Moscow, Russian Federation


Objectives The aim of the prospective study was to compare clinical presentation at diagnosis and long-term outcomes of eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) patients according to antineutrophil cytoplasmic antibody (ANCA) status.

Methods EGPA was classified according to the Revised CHCC Nomenclature and ACR1990 criteria. Activity of vasculitis was evaluated using BVAS. A minor relapse was defined as an increase in at least one new or worse minor item and no major BVAS items. A major relapse was an increase in at least one major BVAS item.

Results We followed 93 patients with EGPA for a mean±SD of 6.3±6.5 years. Their mean±SD age was 46.6±13.8 years, and 96.8% patients had a history of asthma. The most common EGPA manifestations at diagnosis included ENT manifestations (88.2%), fever (78.5%), peripheral neuropathy (73.1%), lung involvement (59.1%) and skin lesions (49.5%). Thirty seven of 93 patients (39.8%) were ANCA-positive. These patients had significantly more frequent myalgia and mononeuritis multiplex, than the ANCA-negative patients. The difference in occurrence of kidney disease between the two groups did not reach statistical significance. However, all three patients with rapidly progressive kidney failure were ANCA-positive. BVAS at diagnosis and VDI at the end of the follow-up were significantly higher for ANCA-positive patients. The follow up duration was 587.7 patient-years. The incidence of all vasculitis relapses was 14 per 100 patient-years, including major and minor vasculitis relapses (5.3 and 8.7 per 100 patient-years, respectively). The 3- and 5-year relapse-free survival rate was 65.4% (95% CI 54.6–76.2) and 43.1% (95% CI 30.4–55.7), respectively. 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).

Table 1.

Main clinical characteristics at diagnosis of the 71 patients with EGPA, according to ANCA status

Conclusions The characteristics of EGPA patients differ according to their ANCA status, although long-term outcomes were similar in both groups. EGPA is characterized by relapsing course of disease. The minor vasculitis relapses predominated in the structure of relapses.

Disclosure of Interest None declared

Statistics from

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.