Emine ÖZÇELİK, Yasemin UĞUR ES, Didem ÖZTÜRK, Mehveş IŞIKLAR EKİCİ, Sultan Nilay YOĞUN, Şeyma Erdem TORUN, Elif ÇELİKEL, Şeyma ERTEM, Esra Esen TEKER, Cüneyt KARAGÖL, Banu Çelikel ACAR, Zahide Ekici TEKİN
Trends in Pediatrics - 2026;7(3):176-183
Objective: Juvenile idiopathic inflammatory myopathies (JIIM) exhibit heterogeneous presentation, and intravenous immunoglobulin (IVIG) is often required in refractory cases. This study aimed to identify the clinical characteristics associated with IVIG use. Methods: This retrospective study included patients aged 0-18 years who fulfilled the Bohan and Peter and/or 2017 EULAR/ACR criteria for JIIM and were followed between March 2009 and August 2025. Demographic, clinical, laboratory, and treatment data were recorded. Results: Thirty-eight patients were included; 29 (76.3%) had juvenile dermatomyositis. Sixteen patients received IVIG. Severe or unresponsive muscle weakness was the main indication (56.3%). IVIG-treated patients had significantly lower Childhood Myositis Assessment Scale (CMAS) scores at diagnosis and at the third month, higher AST levels, more frequent nailfold capillaroscopy abnormalities, and lower remission rates. Each one-point decrease in CMAS increased the odds of IVIG use. Additionally, the third-month CMAS score was the only independent predictor for IVIG therapy. Conclusion: In conclusion, IVIG is an effective treatment option for patients with more severe and refractory JIIM. These patients are characterized by lower CMAS scores and marked microvascular involvement. Earlier use of IVIG as a steroid-sparing strategy may help restrict adverse effects.
