CINCA syndrome with surgical intervention for valgus deformity and flexion contracture of the knee joint: A case report. Issue 6 (2nd November 2017)

• Record Type: Journal Article
• Authors: Harada, Yuki; Fukiage, Kenichi; Nishikomori, Ryuta; Suzuki, Shigeo; Futami, Tohru
• Is Part Of: Modern rheumatology. Volume 27:Issue 6(2017)
• Page Start: 1098
• Page End: 1100
• Publication Date: 2017-11-02
• Subjects: CINCA syndrome -- Flexion contracture -- Hemiepiphysiodesis -- IL-1 beta inhibitor; Rheumatology -- Periodicals; 616.723005
• DOI: 10.3109/14397595.2015.1040609
• Languages: English
• ISSNs: 1439-7595

Abstract

Abstract : Chronic infantile neurological, cutaneous, and articular (CINCA) syndrome is a systemic autoinflammatory disease caused by increased production of interleukin (IL)-1β. We present a case of CINCA syndrome followed up to skeletal maturity. Joint contracture and valgus deformity of the knee had developed before diagnosis. Surgical interventions by soft tissue release and hemiepiphysiodesis improved the contracture and the deformity, and IL-1 receptor antagonist dramatically controlled systemic inflammation, and the patient lives without any disabilities.