Juvenil Dermatomiyozitte Farklı Klinik Fenotipler ve Prognoz: Referans Hastane Deneyimi

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

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

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