Gastrointestinal tract involvement in polyarteritis nodosa and Churg-Strauss syndrome

Abstract

Objective: To study the nature and incidence of gastrointestinal (GI) manifestations in polyarteritis nodosa (PAN) and Churg-Strauss syndrome (CSS) and define their therapeutic and prognostic implications.

Methods: Fifty-three patients (29 males, 24 females) affected with PAN or CSS and followed in our institution were included in a retrospective study. Patients were divided into 2 groups: patients without GI manifestations (group A) and patients with GI manifestations (group B). Among patients with GI manifestations we have studied a subgroup with a possibly poorer prognosis in whom the following symptoms were present: GI tract hemorrhage, intestinal perforation, digestive tract surgery due to PAN manifestations, intractable abdominal pain and weight loss greater than 20% of normal weight due to GI tract ischemia.

Results: The clinical manifestations were those that are classically encountered in PAN and CSS. Every patient fulfilled the American College of Rheumatology (ACR) criteria for PAN and CSS. Thirty-five patients without GI manifestations were included in group A and 18 patients (34%) with GI manifestations in group B. The mean age of the group at the time of diagnosis was 56.9 +/- 19.1 years (range: 21-71 years) in group A and 47.5 +/- 16.8 years (range: 12-82) in group B. GI manifestations were considered as one of the symptoms revealing PAN in 7 (13.2%) cases. Six of the 18 patients with GI manifestations had definite organ involvement related to vasculitis. Abdominal pain without characteristic organ involvement or surgical emergency was present in 12/18 patients. HBV infection was more frequently observed in group B than in group A. Survival curves showed that at 10 years, 80% of the patients in group A were alive versus 67% in group B (P not significant). For the 9 patients with severe GI manifestations, the survival curves showed that, at 10 years, 44% of them were alive versus 80% in the other group A (p < 0.001).

Conclusions: GI manifestations are frequent in PAN and CSS and were present in 34% of our patients. Prognosis of PAN with GI manifestations is not statistically different than in PAN without GI involvement, except for patients with severe digestive complications.