Juvenil idiopatik inflamatuar miyopati: tek merkez verileri

Year 2018, Volume: 43 Issue: 3, 685 - 691, 30.09.2018

Sibel Balcı , Rabia Miray Kışla Ekinci , Derya Ufuk Altıntaş , Mustafa Yılmaz

https://doi.org/10.17826/cumj.337661

Cited By: 1

Abstract

Amaç: Juvenil idiyopatik inflamatuvar miyopatiler (JIIM) nadir görülen, kronik kas inflamasyonu ile seyreden, ciddi morbidite, mortalite ile sonuçlanan bir grup otoimmün hastalıktır.  Çalışmamızda Çukurova Üniversitesi Tıp Fakültesi Çocuk Romatoloji Bilim Dalı tarafından JIIM tanısı ile izlenen hastaların klinik ve laboratuvar verilerinin, tedavi yanıtlarının değerlendirilmesi amaçlanmıştır. 

Gereç ve Yöntem: Eylül 2003 ile Haziran 2017 tarihleri arasında JIIM tanısı ile izlemde olan 15 hasta çalışmaya alındı. Demografik veriler, tanı ve takip laboratuvar verileri, EMG ve kas biyopsi sonuçları, uygulanan tedaviler, tedavi yanıtları ve hastalık komplikasyonları geriye dönük arşiv dosyalarından elde edildi. 

Bulgular: JIIM hastalarının kız erkek oranı 2.75:1 idi. Hastaların 13 tanesi (%80) Juvenil Dermatomiyozit (JDM), 2 tanesi (%13.3) Juvenil Polimiyozit (JPM) ve bir tanesi (%6.7) Overlap Miyozit (OM) tanısı ile izlemdeydi. Başlangıç bulguları heliotrop raş ve malar raş (%86.7), Gottron papülü (%73.3) ve proksimal kas güçsüzlüğü (%100) idi. Erken dönem kortikosteroid tedavisi tüm hastalara ve metotreksat tedavisi 13 hastaya (%86.7) başlandı. Ayrıca 3 hastaya (%20) siklosporin, bir hastaya (%6.7) siklofosfamid, bir hastaya (%6.7) mikofenolat mofetil ve 4 hastaya (%26.7) intravenöz immunglobulin (IVIg) kullanıldı. Sonuçta tüm hastalar remisyona ulaştı fakat hastalık komplikasyonu olarak 2 hastada kalsinozis, bir hastada lipodistrofi gelişti.

Sonuç: JIIM’de erken tanı ve yoğun immünsupresif tedavi hastalık komplikasyonu gelişimini önlemede önemlidir. Bu nedenle uluslararası standart tedavi ve izlem programları hastalık süreci boyunca dikkate alınarak izlem yapılmalıdır.  

Keywords

Juvenil idiopatik inflamatuar miyozit, Juvenil dermatomiyozit

Juvenile idiopathic inflammatory myopathy: single center data

Abstract

Purpose: Juvenile idiopathic inflammatory myopathies (JIIMs) are rare autoimmune diseases. Delay in treatment may cause serious morbidity and mortality. Aim of this study is to evaluate demographic and clinical features, laboratory data, treatment modalities and outcome of patients with JIIMs.

Materials and Methods: 15 patients with JIIMs, followed between 2003 and 2017 at Pediatric Rheumatology Department were included this study. We retrospectively collected data from patients’ medical records and analyzed them. 

Results: Out of the 15 patients, 13 (80 %) had Juvenile Dermatomyositis (JDM), 2 (13.3%) Juvenile Polymyositis (JPM) and 1 (6.7 %) Overlap Myositis (OM, with scleroderma). Clinical symptoms at diagnosis were proximal muscle weakness (100%), heliotrope rash and malar rash (86.7%), Gottron papule (73.3%). Early aggressive treatment with corticosteroid (100%) and methotrexate (86.7%) were started. Intravenous immunoglobulin in 4 patients (26.7 %), cyclosporine in 3 patients (20 %), cyclophosphamide in one patient (6.7 %), and mycophenolate mofetil in one patient (6.7%) were among additional immunosuppressive treatments. Eventually all patients achieved remission but two had calcinosis and one had lipodystrophy.

Conclusion: Early diagnosis and early aggressive treatment are the key points for JIIMs. For this reason, international standard treatment and follow-up programs should be taken into consideration during the disease process.

Keywords

Juvenile idiopathic inflammatory myopathies, Juvenile dermatomyositis, Childhood, muscle weakness.

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