Juvenil Dermatomiyozitte Farklı Klinik Fenotipler ve Prognoz: Referans Hastane Deneyimi

Yıl 2021, , 331 - 336, 16.07.2021

Elif Çelikel , Fatma Aydın , Zahide Ekici Tekin , Tuba Kurt , Cüneyt Karagöl , Nilüfer Tekgöz , Müge Sezer , Melike Kaplan , Serkan Coşkun , Banu Acar

https://doi.org/10.12956/tchd.934302

Cited By: 1

Öz

Amaç: Bu çalışmada, juvenil dermatomiyozit (JDM) tanısı ile takip edilen çocukların klinik özelliklerinin, laboratuvar bulgularının ve prognozunun değerlendirmesi amaçlanmıştır.

Gereç ve Yöntemler: Ocak 2005-Nisan 2021 tarihleri arasında merkezimiz Çocuk Romatoloji Kliniği’nde JDM tanısı alan, en az 6 ay izlemine devam edilen, 17 çocuğun tıbbi kayıtları geriye dönük olarak değerlendirildi.

Bulgular: JDM tanılı 17 hastanın 10’u (%59) kızdı. Hastaların ortanca yaşı 8 (1.5-14)’dü. Tanıdan önceki semptom süresi ortanca 2.5 (1-36) ay; ortalama takip süresi ortanca 24 (6-156) aydı. İlk başvuruda hastaların 15’inde (%88.2) cilt bulgusu, 14’ünde (%82.4) proksimal kas güçsüzlüğü tespit edildi. Başvuruda hastaların 8’inde (%47) eritrosit sedimantasyon hızı ve C-reaktif proteinde yükseklik saptanırken 12’sinde (%70.6) laktat dehidrogenaz, 12’sinde (%70.6) aspartat aminotransferaz, 13’ünde (%76.5) alanin aminotransferaz, 9’unda (%53) kreatinin kinaz yüksekliği saptandı. Miyozit spesifik antikorlar; 5/17 hastada çalışıldı; 4 hastada pozitif bulundu. Kas biyopsisi 6 hastaya yapıldı ve inflamatuar miyozit ile uyumlu bulundu. On üç hastaya ekstremite manyetik rezonans görüntüleme yapıldı ve 10 hastada aktif miyozit gösterildi. Tüm hastalara başlangıç tedavisi olarak steroid ve eş zamanlı olarak metotreksat başlandı. Üç hastada metotreksat yan etkisi nedeni ile mikofenolat mofetil/ siklosporin A verildi. İki hastada kalsinozis gelişti. Son takipte 13 hastanın steroid tedavisi kesilebildi. Bir hasta immünsüpresif ajan olmaksızın, 16 hasta immünsüpresif ajanlarla remisyondaydı. İki hasta 18 yaşın üzerinde olduğu için erişkin romatoloji kliniğine devredildi.

Sonuç: JDM çocukluk çağında nadir görülen bir hastalık olmasına rağmen kalsinozis ve vaskülopatiye ikincil ciddi komplikasyonlara neden olabilir. JDM’li tüm çocuklar yakından takip edilmelidir.

Anahtar Kelimeler

Çocukluk çağı, Juvenil dermatomiyozit, Prognoz

Destekleyen Kurum

yok

Different Clinical Phenotypes and Prognosis in Juvenile Dermatomyositis: Reference Hospital Experience

Öz

Objective: The aim of this study is to evaluate the clinical features, laboratory findings and prognosis of children followed up with a diagnosis of juvenile dermatomyositis (JDM).

Material and Methods: The medical records of 17 children who were diagnosed with JDM in the Pediatric Rheumatology Clinic of our center between January 2005 and April 2021 and were followed up for at least 6 months were evaluated retrospectively.

Results: Ten of the 17 patients (59%) with a diagnosis of JDM were girls. Median age was 8 (1.5-14). Median duration of symptoms before diagnosis was 2.5 (1-36) months; the median follow-up time was 24 (6-156) months. Skin findings were detected in 15 (88.2%) patients and proximal muscle weakness in 14 (82.4%) patients at the first admission. Erythrocyte sedimentation rate and C-reactive protein elevation were detected in 8 (47%) patients at admission, while 12 (70.6%) had lactate dehydrogenase, 12 (70.6%) aspartate aminotransferase, and 13 (76.5%), alanine aminotransferase and 9 (53%) increased creatine kinase. Myositis-specific antibodies; assessed in 5/17 patients; found positive in 4 patients. Muscle biopsy was performed on 6 patients and was found to be compatible with inflammatory myositis. Magnetic resonance imaging of the limbs was performed in 13 patients and active myositis was demonstrated in 10 patients. Steroid and simultaneous methotrexate were started as initial therapy in all patients. Mycophenolate mofetil/cyclosporine A was given to three patients because of the side effect of methotrexate. Calcinosis developed in two patients. Steroid therapy could be discontinued in 13 patients at the last follow-up. One patient was in remission without an immunosuppressive agent, and 16 patients were in remission with immunosuppressive agents. Two patients were transferred to the adult rheumatology clinic because they were over 18 years old.

Conclusion: Although JDM is a rare disease in childhood, it can cause serious complications secondary to calcinosis and vasculopathy. All children with JDM should be closely monitored.

Anahtar Kelimeler

Childhood, Juvenile dermatomyositis, Prognosis

Kaynakça

• 1. Woo P, Laxer RM, Sherry DD. Juvenile dermatomyositis. In: Woo P, Laxer RM, Sherry DD, editors. Pediatric Rheumatology in clinical practice. 1st ed. London: Springer; 2007. p. 66-76.
• 2. Compeyrot-Lacassagne S, Feldman BM. Inflammatory myopathies in children. Pediatr Clin North Am 2005;52:493-520.
• 3. Feldman BM, Rider LG, Reed AM, Pachman LM. Juvenile dermatomyositis and other idiopathic inflammatory myopathies of childhood. Lancet 2008;371:2201-12.
• 4. Symmons DP, Sills JA, Davis SM. The incidence of juvenile dermatomyositis: results from a nation-wide study. Br J Rheumatol. 1995;34:732–6.
• 5. Mendez EP, Lipton R, Ramsey-Goldman R, Roettcher P, Bowyer S, Dyer A, et al. NIAMS Juvenile DM Registry Physician Referral Group 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.
• 6. Fisler RE, Liang MG, Fuhlbrigge RC, Yalcindag A, Sundel RP. Aggressive management of juvenile dermatomyositis results in improved outcome and decreased incidence of calcinosis. J Am Acad Dermatol 2002;47:505-11.
• 7. Kim S, El-Hallak M, Dedeoglu F, Zurakowski D, Fuhlbrigge RC, Sundel RP. Complete and sustained remission of juvenile dermatomyositis resulting from aggressive treatment. Arthritis Rheum 2009;60:1825-30.
• 8. Traineau H, Aggarwal R, Monfort JB, Senet P, Oddis CV, Chizzolini C, et al. Treatment of calcinosis cutis in systemic sclerosis and dermatomyositis: A review of the literature. J Am Acad Dermatol. 2020;82:317-25.
• 9. Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med 1975;292:344-7.
• 10. Campanilho-Marques R, Deakin CT, Simou S, Papadopoulou C, Wedderburn LR, Pilkington CA; Juvenile Dermatomyositis Research Group (JDRG). Retrospective analysis of infliximab and adalimumab treatment in a large cohort of juvenile dermatomyositis patients. Arthritis Res Ther. 2020 15;22:79.
• 11. Barut K, Aydin PO, Adrovic A, Sahin S, Kasapcopur O. Juvenile dermatomyositis: a tertiary center experience. Clin Rheumatol. 2017;36:361-6.
• 12. Sun C, Lee JH, Yang YH, Yu HH, Wang LC, Lin YT, Chiang BL. Juvenile dermatomyositis: a 20-year retrospective analysis of treatment and clinical outcomes. Pediatr Neonatol. 2015;56:31-9.
• 13. Sasaki H, Kohsaka H. Current diagnosis and treatment of polymyositis and dermatomyositis. Mod Rheumatol. 2018;28:913-21.
• 14. Tansley Sarah L., Simou Stefania, Shaddick Gavin, Betteridge Zoe E., Almeida Beverley, Gunawardena Harsha. Autoantibodies in juvenile-onset myositis: Their diagnostic value and associated clinical phenotype in a large UK cohort. Journal of Autoimmunity. 2017;84:55–64.
• 15. Aouizerate J, De Antonio M, Bader-Meunier B, Barnerias C, Bodemer C, Isapof A, et al. Muscle ischaemia associated with NXP2 autoantibodies: a severe subtype of juvenile dermatomyositis. Rheumatology. 2018;57:873–9.
• 16. Rider LG, Shah M, Mamyrova G, Huber AM, Rice MM, Targoff IN, Miller FW, Childhood Myositis Heterogeneity Collaborative Study Group The myositis autoantibody phenotypes of the juvenile idiopathic inflammatory myopathies. Medicine. 2013;92:223–43.
• 17. Tansley SL, Betteridge ZE, Shaddick G, Gunawardena H, Arnold K, Wedderburn LR, McHugh N, Juvenile Dermatomyositis Research Group Calcinosis in juvenile dermatomyositis is influenced by both anti-NXP2 autoantibody status and age at disease onset. Rheumatology. 2014;53:2204–08.
• 18. Gunawardena H, Wedderburn LR, Chinoy H, Betteridge ZE, North J, Ollier WE, et al. Autoantibodies to a 140-kd protein in juvenile dermatomyositis are associated with calcinosis. Arthritis Rheum. 2009;60:1807–14.
• 19. Gunawardena H, Wedderburn LR, North J, Betteridge Z, Dunphy J, Chinoy H, et al. Juvenile Dermatomyositis Research Group UK Clinical associations of autoantibodies to a p155/140 kDa doublet protein in juvenile dermatomyositis. Rheumatology. 2008;47:324–8.
• 20. Almeida B, Tansley S, Simou S, Gunawardena H, McHugh N, Wedderburn L. Anti-synthetase autoantibody is seen in patients with overlap myositis in the UK cohort of patients with Jveunile Dermatomyositis. Rheumatology. 2017;84:55-64.
• 21. Kobayashi N, Takezaki S, Kobayashi I, Iwata N, Mori M, Nagai K, et al. Clinical and laboratory features of fatal rapidly progressive interstitial lung disease associated with juvenile dermatomyositis. Rheumatology. 2015;54:784–91.
• 22. Tansley SL, Betteridge ZE, Gunawardena H, Jacques TS, Owens CM, Pilkington C, et al. Anti-MDA5 autoantibodies in juvenile dermatomyositis identify a distinct clinical phenotype: a prospective cohort study. Arthritis Research & Therapy. 2014;16:R138.
• 23. 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.
• 24. Traineau H, Aggarwal R, Monfort JB, Senet P, Oddis CV, Chizzolini C, et al. Treatment of calcinosis cutis in systemic sclerosis and dermatomyositis: A review of the literature. J Am Acad Dermatol. 2020;82:317-25.