J Korean Med Sci.  2006 Apr;21(2):265-271. 10.3346/jkms.2006.21.2.265.

Churg-Strauss Syndrome: The Clinical Features and Long-term Follow-up of 17 Patients

• Oh MJ
• Lee JY
• Kwon NH
• Choi DC

• KMID: 1781833
• DOI: http://doi.org/10.3346/jkms.2006.21.2.265

Abstract

Churg-Strauss syndrome (CSS) is a rare multi-system vasculitis; some cases have been reported in Korea. The aim of this study is to describe the clinical features, treatment outcome, and long-term follow-up of CSS from a single Korean medical center. Between 1995 and 2004, seventeen patients were diagnosed with CSS at the Department of Medicine of the Samsung Medical Center, Sungkyunkwan University School of Medicine. The diagnosis of CSS is based on the classification criteria of the American Collage of Rheumatology. All patients had asthma. As in other case series, the lung, peripheral nervous system, and skin were the most commonly involved organs. During the active stage of the disease, most of the patients exhibited peripheral blood eosinophilia and an elevated serum eosinophil cationic protein level. Ten patients were treated with pulses of methylprednisolone followed by tapering and cyclophosphamide, and the others were treated with corticosteroids alone. The outcomes after long-term follow-up were generally good. One patient who was refractory to initial treatment died of heart failure during the follow-up period. CSS was highly variable in its presentation and course. The manifestations may range from mild symptoms to life-threatening conditions. The outcome after long-term follow-up was as good as that of previous studies.

Keyword

Churg-Strauss Syndrome; Treatment Outcome; Follow-up Studies

MeSH Terms

Treatment Outcome
Time Factors
Middle Aged
Methylprednisolone/therapeutic use
Male
Humans
Follow-Up Studies
Female
Cyclophosphamide/therapeutic use
Churg-Strauss Syndrome/*diagnosis/drug therapy
Adult

Figure

• 

  Fig. 1 Therapeutic modalities and disease course of 17 patients with Churg-Strauss syndrome. MPD, methylprednisolone; CPM, cyclophosphamide; CS, corticosteroids.

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