A Case of Churg-Strauss Syndrome with Interstinal Perforation.

Park, Jung Hoon; Jung, Yun Seok; Kim, Yang Ki; Lee, Young Mok; Hwang, Jung Hwa; Kim, Ki Up; Kim, Dong Won; Uh, Soo Taek; Kim, Jea Jun

doi:2009.66.5.374

Tuberc Respir Dis > Volume 66(5); 2009 > Article

Case Report

Tuberculosis and Respiratory Diseases 2009;66(5):374-379.
DOI: https://doi.org/10.4046/trd.2009.66.5.374 Published online May 1, 2009.

A Case of Churg-Strauss Syndrome with Interstinal Perforation.

Jung Hoon Park, Yun Seok Jung, Yang Ki Kim, Young Mok Lee, Jung Hwa Hwang, Ki Up Kim, Dong Won Kim, Soo Taek Uh, Jea Jun Kim

¹Department of Internal Medicine, School of Medicine, Soonchunhyang University, Seoul, Korea. kukim@hosp.sch.ac.kr
²Department of Radiology, School of Medicine, Soonchunhyang University, Seoul, Korea.
³Department of Pathology, School of Medicine, Soonchunhyang University, Seoul, Korea.
⁴Department of Surgery, School of Medicine, Soonchunhyang University, Seoul, Korea.

Abstract

Churg-Strauss syndrome (CSS) or allergic granulomatous angiitis is a rare syndrome that is characterized by hypereosinophilic systemic necrotizing vasculitis affecting small- to medium-sized arteries and veins. In general, it occurs in individuals with pre-existing allergic asthma. When CSS appears in patients, it has the following characteristics: eosinophilia of more than 10% in peripheral blood, paranasal sinusitis, pulmonary infiltrates, histological proof of vasculitis with extravascular eosinophils, and mononeuritis multiplex or polyneuropathy. Therapeutic trials dedicated to Churg-Strauss syndrome have been limited due to the rarity of this disorder and the difficulty in making a histological diagnosis. Proper treatment of patients with CSS is not widely known. In this case study, we report on our experience with an unusual patient case, characterized by purpura and a perforation of the small intestine after inadequate steroid therapy.

Key Words: Churg-Strauss syndrome, Asthma, Intestinal perforation, Eosinophilic infiltration