A Case of Churg-Strauss Syndrome Affecting Lung and Neuromuscular System.

Song, Hyun Ju; Cha, Ju Hyun; Lee, Jin Hwa; Lee, Ji A; Sung, Sun Hui; Koo, Hea Soo; Kim, You Kyoung; Chang, Jung Hyun

doi:2002.53.2.183

Tuberc Respir Dis > Volume 53(2); 2002 > Article

Case Report

Tuberculosis and Respiratory Diseases 2002;53(2):183-189.
DOI: https://doi.org/10.4046/trd.2002.53.2.183 Published online August 1, 2002.

A Case of Churg-Strauss Syndrome Affecting Lung and Neuromuscular System.

Hyun Ju Song, Ju Hyun Cha, Jin Hwa Lee, Ji A Lee, Sun Hui Sung, Hea Soo Koo, You Kyoung Kim, Jung Hyun Chang

¹Department of Internal Medicine, College of Medicine, Ewha Womans University, Seoul, Korea. hs1017@ewha.ac.kr
²Department of Pathology, College of Medicine, Ewha Womans University, Seoul, Korea.
³Department of Radiology, College of Medicine, Ewha Womans University, Seoul, Korea.

Abstract

Churg-Strauss syndrome(CSS) is a systemic vascular disorder that has an unknown cause with multiorgan involvement and diverse presentations. The three main histologically distinct phases were necrotizing vasculitis, tissue eosinophilia and extravascular granulomas. A diagnosis of CSS can be made on four or more of the following six criteria : 1) asthma, 2) peripheral eosinophilia >10% on the differential leukocyte count, 3) mononeuropathy (including multiple) or polyneuropathy, 4) paranasal sinus abnormalities, 5) nonfixed pulmonary infiltrates, and 6) biopsy evidence of extravascular eosinophils in the skin, the nerves, or the lungs. CSS has a good prognosis with systemic steroid therapy. The 5 year survival is approximately 70 %. We experienced a 66-year-old man who presented with cough, sputum, edema and numbness in both legs. He presented with all of the 6 CSS criteria. A nerve and muscle biopsy confirmed the diagnosis. Here, we report this case with a review of the relevant literatures.

Key Words: Churg-Strauss syndrome