Figen ÇAKMAK, Fatma Gül DEMİRKAN, Nuray AKTAY AYAZ

Juvenil Dermatomiyozit Tanısı ile Takipli Çocuk Hastaların Klinik, Laboratuvar ve Görüntüleme Bulgularının Değerlendirilmesi: Tek Merkez Deneyimi

Giriş ve Amaç: Juvenil dermatomiyozit (JDM) idiyopatik inflamatuar miyozitlerin büyük çoğunluğunu oluşturan, kas ve cilt tutulumu ile seyreden nadir görülen bir otoimmün miyopatidir. Çalışmamızın amacı merkezimizde JDM tanısı ile takip edilen çocuk hastaların demografik, klinik, laboratuvar, görüntüleme özelliklerinin ve tedavilerinin değerlendirilmesidir. Gereç ve Yöntemler: Kliniğimizce 2015-2021 tarihleri arasında JDM tanısı ile en az 6 ay takip edilen hastaların verileri geriye yönelik olarak hasta tıbbi kayıtları incelenerek değerlendirildi. Bulgular: Çalışmaya 6 kız 6 erkek hasta dahil edildi. Başvuru sırasında 5 hasta hafif, 3 hasta orta, 4 hasta ağır klinik bulgular ile tanı aldı. Takip süresince klinik seyir 2 hastada monosiklik, 7 hastada kronik persistan ve 3 hastada polisiklikti. Tüm hastaların tanı sırasında cilt ve kas bulguları mevcuttu, laboratuvar olarak 9 hastada en az bir kas enzimi yüksekliği, 7 hastada CK yüksekliği, 7 hastada LDH yüksekliği ve 5 hastada AST yüksekliği vardı. Takip süresince 5 hastada kalsinozis, 2 hastada eklem kontraktürü ve 1 hastada lipodistrofi gelişimi gözlendi. Başlangıç tedavisi olarak 9 hastaya yoğun yüksek doz metilprednizolon 3 gün süre ile verildi. Takipte yedi (58,3) hastaya IVIG tedavisi verildi. Sonuç: JDM tedavisi ve takibi zor olan, multidisipliner deneyimli çocuk romatoloji merkezlerince yönetilen bir hastalıktır. Mevcut hasta verilerinin dökümente edilmesi ve multidisipliner verilerin sunulması bu hastaları takip eden hekimler için yol gösterici olacaktır.

Anahtar Kelimeler:

Juvenil dermatomiyozit, juvenil idiopatik inflamatuar miyopati, elektromiyografi

The Clinical, Laboratory, and Imaging Features of Children with Juvenile Dermatomyositis: A Single Center Experience

Objective: Juvenile dermatomyositis (JDM) is a rare disease that accounts for the majority of idiopathic inflammatory myositis, typically affecting muscle and skin. Our study aims to evaluate pediatric patients' demographic, clinical, laboratory, and imaging characteristics and treatments followed up with the diagnosis of JDM in a tertiary pediatric rheumatology center. Materials and Methods: The patients' medical records followed up with the diagnosis of JDM for at least 6 months between 2015- 2021 and were evaluated retrospectively. Results: Six female and six male patients were included in the study. At the time of diagnosis, 5 patients had mild, 3 patients had moderate and 4 patients had severe clinical findings. During the follow-up, the clinical course was monocyclic in 2 patients, chronic persistent in 7 patients, and polycyclic in 3 patients. All patients had skin and muscle involvement at the time of diagnosis. At least one muscle enzyme elevation was found in 9 patients, CK elevation in 7 patients, LDH elevation in 7 patients, and AST elevation in 5 patients. Calcinosis was observed in 5 patients, joint contracture in 2 patients, and lipodystrophy in 1 patient during the follow-up period. High-dose methylprednisolone was given to 9 patients for 3 days as initial treatment. Seven (58.3) patients received IVIG. Conclusion: Managing JDM is difficult because of patients are followed by experienced multidisciplinary pediatric rheumatology centers. Documenting existing patient data and presenting multidisciplinary data will be a guide for physicians who have undertaken the management of these patients.

Keywords:

Juvenile dermatomyositis, Juvenile idiopathic inflammatory myopathies, electromyography,

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