Korean J Intern Med.  2014 Jan;29(1):85-95. 10.3904/kjim.2014.29.1.85.

Clinical features and prognostic factors of Churg-Strauss syndrome

• Kim MY
• Sohn KH
• Song WJ
• Park HW
• Cho SH
• Min KU
• Kang HR

• KMID: 1792016
• DOI: http://doi.org/10.3904/kjim.2014.29.1.85

Abstract

BACKGROUND/AIMS
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.

Keyword

Churg-Strauss syndrome; Antibodies, antineutrophil cytoplasmic; Prognosis

MeSH Terms

Adolescent
Adult
Aged
Antibodies, Antineutrophil Cytoplasmic/blood
Churg-Strauss Syndrome/drug therapy/*epidemiology/immunology
Female
Glucocorticoids/therapeutic use
Humans
Immunosuppressive Agents/therapeutic use
Male
Middle Aged
Prednisolone/therapeutic use
Republic of Korea/epidemiology
Retrospective Studies
Young Adult
Antibodies, Antineutrophil Cytoplasmic
Glucocorticoids
Immunosuppressive Agents
Prednisolone