Çiğdem A. Çelikel, Deniz Ertem, Engin Tutar, Rabia Ergelen, Bilge Şahin Akkelle

Yedi yaşındaki kız olguda çoklu organ tutulumlu IgG4 ilişkili hastalık: Ender bir prezentasyon

İmmünglobulin G4 ilişkili sistemik hastalık çocuk yaş grubunda ender görülür ve tipik histopatolojik, radyolojik ve klinik özellikler ile belirgindir. Birçok organ ya da sistem tutulumunun görülebildiği hastalıkta pankreas tutulumu otoimmun pankreatit olarak tanımlanmıştır. Hastalıkta serum immünglobulin G4 düzeyi genellikle yüksektir. Enflamatuvar bağırsak hastalığı birlikteliği ender bildirilmiştir. Pankreatit ve eş zamanlı sklerozan kolanjit tablosu ile başvuran yedi yaşındaki olgumuz izlemde gelişen kanlı dışkılama nedeniyle endoskopik olarak incelenmiş ve enflamatuvar bağırsak hastalığı saptanmıştır. Ayrıca lakrimal bez tutulumu saptanan hasta immünglobulin G4 ilişkili sistemik hastalık tanısı almış ve steroid tedavisi ardından hastanın semptom ve bulgularında dramatik iyileşme sağlanmıştır. İmmünglobulin G4 ilişkili sistemik hastalıkta farkındalık, erken tanıda ve hastalık ilişkili organ hasarının önlenmesinde önemlidir.

IgG4 related disease in a seven year old girl with multiple organ involvement: A rare presentation

Autoimmune pancreatitis has been described as a pancreatic manifestationof immunoglobulin G4-related disease, which is characterized by typicalhistopathologic, radiologic, and clinical features. Immunoglobulin G4-related disease is usually accompanied by elevated serum immunoglobulin G4 level, and can involve multiple organ/systems. Immunoglobulin G4-related disease has rarely been reported in pediatric population.There are few reports of inflammatory bowel disease in association withimmunoglobulin G4-related disease. We describe a 7-year-old girl whopresented with pancreatitis and concurrent sclerosing cholangitis, anddeveloped bloody diarrhea during follow-up. An endoscopic examinationrevealed inflammatory bowel disease, and later lacrimal gland involvement was also recognized. She was diagnosed as having immunoglobulinG4-related disease, and her clinical signs and symptoms improved dramatically after steroid treatment. Hence, awareness of the clinical pictureis important and early diagnosis can prevent fibrosis and organ damage.

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