多臓器病変を呈した7歳女児のIgG4関連疾患。稀な症例

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

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

抄録

自己免疫性膵炎は、典型的な病理組織学的、放射線学的、および臨床的特徴によって特徴づけられる免疫グロブリンG4関連疾患の膵臓症状として記述されてきました。免疫グロブリンG4関連疾患は、通常、血清免疫グロブリンG4レベルの上昇を伴い、複数の臓器／システムに関与しうる。免疫グロブリンG4関連疾患は小児ではほとんど報告されていません。また，免疫グロブリンG4関連疾患に関連した炎症性腸疾患の報告はほとんどない．膵炎と強直性胆管炎を併発し，経過観察中に血性下痢を発症した7歳女児を対象とした．内視鏡検査で炎症性腸疾患が認められ，後に涙腺病変も認められた．免疫グロブリンG4関連疾患と診断され，ステロイド治療により臨床症状は劇的に改善した．したがって、臨床像の認識が重要であり、早期診断により線維化や臓器障害を防ぐことができる。

Autoimmune pancreatitis has been described as a pancreatic manifestation of immunoglobulin G4-related disease, which is characterized by typical histopathologic, 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 with immunoglobulin G4-related disease. We describe a 7-year-old girl who presented with pancreatitis and concurrent sclerosing cholangitis, and developed bloody diarrhea during follow-up. An endoscopic examination revealed inflammatory bowel disease, and later lacrimal gland involvement was also recognized. She was diagnosed as having immunoglobulin G4-related disease, and her clinical signs and symptoms improved dramatically after steroid treatment. Hence, awareness of the clinical picture is important and early diagnosis can prevent fibrosis and organ damage.

腸内フローラG4のシステムは、あなたの臓器の機能を向上させ、組織の機能を向上させ、組織の機能を向上させ、臨床の機能を向上させることができます。臓器の損傷は、臓器の損傷を防ぐために、臓器の損傷を防ぐために、臓器の損傷を防ぐために、臓器の損傷を防ぐために、臓器の損傷を防ぐために、臓器の損傷を防ぐために、臓器の損傷を防ぐために、臓器の損傷を防ぐためのシステムを構築する必要があります。血清免疫グロブリンG4の値は、その遺伝子を反映しています。このような場合には、当院のスタッフに相談してください。このような場合には、G4免疫グロブリンを使用したシステムを使用する必要があります。自己免疫性G4のシステムは、あなたの臓器の中で最も重要なものの一つです。

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.

Copyright: © 2020 Turkish Archives of Pediatrics.