Churg-Strauss syndrome (allergic granulomatous angitis) presenting with ileus caused by ischemic ileal ulcer

Abstract

We report a rare case of Churg-Strauss syndrome (CSS) in a 41-year-old Japanese man with a history of middle-age onset of bronchial asthma who had severe abdominal pain. He presented with ileus caused by an annular ulcer of the ileum, attributable to mucosal ischemia resulting from necrotizing vasculitis of the mesenteric artery. He also had marked hypereosinophilia (51.5%), elevated serum IgE levels (34040 IU/ml), and generalized enlargement of the superficial cervical lymph nodes, containing eosinophilic granulomas. A stenotic lesion caused by an annular ulcer in the ileum was found and resected by laparotomy. Microscopic examination of the resected specimen revealed luminal narrowing or occlusion of small arteries in the ulcer base, subserosa, and mesenterium resulting from marked fibrotic intimal thickening with fragmentation or lack of the internal elastic lamina. These findings were diagnosed as vasculitis, scar stage. The postoperative course was uneventful, with the patient receiving a maintenance dose of prednisolone (10-15 mg/day) for 7 years subsequently. We must carefully diagnose and treat patients with middle-age onset asthma, because the symptom may be a lung manifestation of CSS, in which various organs including gastrointestinal tract are involved as a result of systemic necrotizing vasculitis.

An aggressive and lethal course of Churg–Strauss syndrome with alveolar hemorrhage, intestinal perforation, cardiac failure and peripheral neuropathy

“…Ulceration and perforation of the gastrointestinal tract are assumed to be the result of ischemia caused by vasculitis [18]. The small intestine is the most predisposing site like in our case.…”

Severe Cryptogenic Multifocal Ulcerous Stenosing Enteritis. A Report of Three Cases and Review of the Literature

“…Any type of vasculitis of any cause, if found, should be assigned simply as "vasculitis" not CMUSE. Small intestinal involvement can be found in Churg-Strauss syndrome (20,27,36), systemic lupus erythematosus (43), Buerger's disease (30), Wegener's granulomatosis (11), Weber-Christian disease (28) and systemic sclerosis (13). Vasculitis could be associated with multiple small intestinal ulcers and their complications (including perforation) but quite rarely with multifocal stenoses of the small bowel.…”

A limited form of Churg-Strauss syndrome presenting as acute abdominal catastrophe

“…Most cases of intestinal involvement in CSS have classical features of CSS such as asthma and eosinophilia. Gastrointestinal symptoms can rarely be the first manifestation of typical or limited forms of CSS such as in our case [3,4]. The differential diagnosis of CSS includes Wegener's granulomatosis, polyarteritis nodosa, and microscopic polyarteritis [3,4,8].…”

“…Gastrointestinal manifestations of CSS include diarrhea, melena, and abdominal cramps [3,4,7]. Rare cases of intestinal perforation and ulceration due to vasculitis have been reported [1,5,8].…”

“…Gastrointestinal symptoms can rarely be the first manifestation of typical or limited forms of CSS such as in our case [3,4]. The differential diagnosis of CSS includes Wegener's granulomatosis, polyarteritis nodosa, and microscopic polyarteritis [3,4,8]. Criteria for the diagnosis of CSS, which have been developed by the American College of Rheumatology are; asthma, eosinophilia, neuropathy, paranasal sinus abnormalities, extravascular eosinophils, and pulmonary infiltrates [6].…”