Sibel BALCI, Rabia Miray KIŞLA EKİNCİ, Derya Ufuk ALTINTAŞ, Mustafa YILMAZ

Juvenil idiopatik inflamatuar miyopati: tek merkez verileri

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.

Anahtar Kelimeler:

Juvenil idiopatik inflamatuar miyozit, Juvenil dermatomiyozit

Juvenile idiopathic inflammatory myopathy: single center data

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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1. Rider LG, Katz JD, Jones OY. Developments in the classification and treatment of the juvenile idiopathic inflammatory myopathies. Rheum Dis Clin North Am. 2013;39:877-904.
2. Symmons DP, Sills JA, Davis SM The incidence of juvenile dermatomyositis: results from a nation-wide study. Br J Rheumatol. 1995;34:732-6.
3. Mendez EP, Lipton R, Ramsey-Goldman R, Roettcher P, Bowyer S, Dyer A et al. US incidence of juvenile dermatomyositis, 1995–1998: results from the National Institute of Arthritis and Musculoskeletal and Skin Diseases registry. Arthritis Rheum. 2003;49:300-5.
4. McCann LJ, Juggins AD, Maillard SM, Wedderburn LR, Davidson JE, Murray KJ et al. The Juvenile Dermatomyositis National Registry and Repository (UK and Ireland) clinical characteristics of children recruited within the first 5 yr. Rheumatology (Oxford). 2006;45:1255-60.
5. Pachman LM, Lipton R, Ramsey-Goldman R, Shamiyeh E, Abbott K, Mendez EP et al. History of infection before the onset of juvenile dermatomyositis: results from the National Institute of Arthritis and Musculoskeletal and Skin Diseases Research Registry. Arthritis Rheum. 2005;53:166-172.
6. Barut K, Aydin PO, Adrovic A, Sahin S, Kasapcopur O. Juvenile dermatomyositis: a tertiary center experience. Clin Rheumatol. 2017;36:361-6.
7. Rider LG, Lindsley CB, Miller FW. Juvenile dermatomyositis. In Textbook of Pediatric Rheumatology. 7th ed (Eds RE Petty, RM Laxer, CB Lindsley, L Wedderburn):351-83. Philadelphia, Saunders, 2016.
8. Brown VE, Pilkington CA, Feldman BM, Davidson JE; Network for Juvenile Dermatomyositis, Paediatric Rheumatology European Society (PReS). An international consensus survey of the diagnostic criteria for juvenile dermatomyositis (JDM). Rheumatology (Oxford). 2006;45:990-3.
9. Shah M, Mamyrova G, Targoff IN, Huber AM, Malley JD, Rice MM et al. Childhood Myositis Heterogeneity Collaborative Study Group. The clinical phenotypes of the juvenile idiopathic inflammatory myopathies. Medicine (Baltimore). 2013;92:25-41.
10. Mamyrova G, Katz JD, Jones RV, Targoff IN, Lachenbruch PA, Jones OY et al. Clinical and laboratory features distinguishing juvenile polymyositis and muscular dystrophy. Arthritis Care Res (Hoboken). 2013;65:1969-75.
11. Lazarevic D, Pistorio A, Palmisani E, Miettunen P, Ravelli A, Pilkington C et al. The PRINTO criteria for clinically inactive disease in juvenile dermatomyositis. Ann Rheum Dis. 2013;72:686-93.
12. Lorenzoni PJ, Scola RH, Kay CS, Prevedello PG, Espindola G, Werneck LC. Idiopathic inflammatory myopathies in childhood: a brief of 27 cases. Pediatr Neurol. 2011;45:17-22.
13. Sarkar S, Mondal T, Saha A, Mondal R, Datta S. Profile of pediatric idiopathic inflammatory myopathies from a tertiary care center of eastern India. Indian J Pediatr. 2017;84:299-306.
14. Malek A, Raeeskarami SR, Ziaee V, Aghighi Y, Moradinejad MH. Clinical course and outcomes of Iranian children with juvenile dermatomyositis and polymyositis. Clin Rheumatol. 2014;33:1113-8.
15. Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med. 1975;292:344–7.
16. Bohan A, Peter JB. Polymoyositis and dermatomyositis (second of two parts). N Engl J Med. 1975;292:403-7.
17. Maillard SM, Jones R, Owens C, Pilkington C, Woo P, Wedderburn LR et al. Quantitative assessment of MRI T2 relaxation time of thigh muscles in juvenile dermatomyositis. Rheumatology 2004;43:603-8.
18. Hernandez RJ, Sullivan DB, Chenevert TL, Keim DR. MR imaging in children with dermatomyositis: musculoskeletal findings and correlation with clinical and laboratory findings. AJR Am J Roentgenol. 1993;161:359-66.
19. Enders FB, Bader-Meunier B, Baildam E, Constantin T, Dolezalova P, Feldman BM et al. Consensus-based recommendations for the management of juvenile dermatomyositis. Ann Rheum Dis. 2017;76:329-40.
20. Singh S, Kumar L, Shankar KR. Indian Pediatr. Juvenile dermatomyositis in north India. 1997;34:193-8.