title: Clinical features and prognostic factors of Churg-Strauss syndrome.
abstract: OBJECTIVE
  Churg-Strauss syndrome (CSS) is a rare systemic necrotizing small-vessel vasculitis, with accompanying bronchial asthma, eosinophilia, and eosinophilic infiltration of various tissues. The purposes of our study were to characterize the clinical features of CSS and to identify factors associated with CSS prognosis in Koreans.
  
  
  METHODS
  Medical records were reviewed retrospectively for all physician-diagnosed CSS patients in the Seoul National University Hospital between January 1990 and March 2011.
  
  
  RESULTS
  Data from 52 CSS patients were analyzed. The respiratory tract was the most commonly involved organ (90.4%). Renal involvement was less frequent in antineutrophilic cytoplasmic antibody (ANCA)(-) patients than in ANCA(+) patients (p = 0.048). Clinical remission occurred in 95.3% of patients, but 16.3% of them relapsed. Patients who maintained remission for more than 6 months were relatively older (median, 51 years) at diagnosis (p = 0.004), had been diagnosed in earlier stages (p = 0.027), showed more frequent respiratory involvement (p = 0.024) and generalized symptoms (p = 0.039), and showed less frequent cutaneous involvement (p = 0.030) than those who did not achieve persistent (> 6 months) remission. Patients who achieved persistent remission also showed higher C-reactive protein (CRP) levels (p = 0.031) than those who did not.
  
  
  CONCLUSIONS
  ANCA(-) CSS patients showed less frequent renal involvement. Characteristics of good responders were older age, diagnosis at earlier stages, less cutaneous involvement, more respiratory involvement, high CRP values, and more generalized symptoms.
authors.affiliation: Department of Internal Medicine, Inje University Busan Paik Hospital, Busan, Korea.
authors.forename: Mi-Yeong
authors.initials: MY
authors.lastname: Kim
authors.affiliation: Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea. ; Institute of Allergy and Clinical Immunology, Seoul National University Medical Research Center, Seoul, Korea.
authors.forename: Kyoung-Hee
authors.initials: KH
authors.lastname: Sohn
authors.affiliation: Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea. ; Institute of Allergy and Clinical Immunology, Seoul National University Medical Research Center, Seoul, Korea.
authors.forename: Woo-Jung
authors.initials: WJ
authors.lastname: Song
authors.affiliation: Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea. ; Institute of Allergy and Clinical Immunology, Seoul National University Medical Research Center, Seoul, Korea.
authors.forename: Heung-Woo
authors.initials: HW
authors.lastname: Park
authors.affiliation: Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea. ; Institute of Allergy and Clinical Immunology, Seoul National University Medical Research Center, Seoul, Korea.
authors.forename: Sang-Heon
authors.initials: SH
authors.lastname: Cho
authors.affiliation: Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea. ; Institute of Allergy and Clinical Immunology, Seoul National University Medical Research Center, Seoul, Korea.
authors.forename: Kyung-Up
authors.initials: KU
authors.lastname: Min
authors.affiliation: Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea. ; Institute of Allergy and Clinical Immunology, Seoul National University Medical Research Center, Seoul, Korea.
authors.forename: Hye-Ryun
authors.initials: HR
authors.lastname: Kang
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D015267:Churg-Strauss Syndrome / Q000188:drug therapy / Q000453:epidemiology* / Q000276:immunology; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D011239:Prednisolone / Q000627:therapeutic use; D056910:Republic of Korea / Q000453:epidemiology; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D011239:Prednisolone
keywords: Antibodies, antineutrophil cytoplasmic; Churg-Strauss syndrome; Prognosis