Gastroenterology, Southmead Hospital, Bristol, UK
North Bristol NHS Trust, Bristol, UK.
BMJ Case Rep. 2024 Aug 24;17(8):e259997. doi: 10.1136/bcr-2024-259997.
A woman in her 20s presented with 6 weeks of fever, persistent vomiting and 28% loss of body weight. Symptoms were refractory to treatment with antiemetics and broad spectrum antibiotics.Further investigation via oesophageogastroduedenoscopy revealed a large gastric ulcer and pyloric stricture, causing gastric outlet obstruction (GOO). Biopsies of the stomach and duodenum showed plasma cell infiltration with a large proportion being IgG4 positive.Treatment with methylprednisolone, and later prednisolone, quickly improved inflammatory markers and symptoms. Balloon dilatation of the pyloric stricture also improved vomiting, allowing eventual re-establishment of oral nutrition. The patient made a full recovery with maintenance treatment on mycophenolate mofetil.IgG4-related disease (IgG4-RD) is a multisystem disorder with unpredictable presentation. The case highlights diagnostic challenges in IgG4-RD and identifies it as a rare differential in upper gastrointestinal symptoms. To our knowledge this is the first published case of IgG4-RD in the duodenum causing GOO.
一位 20 多岁的女性患者出现 6 周发热、持续呕吐和体重下降 28%。抗呕吐药和广谱抗生素治疗无效。经食管胃十二指肠镜检查发现胃大溃疡和幽门狭窄，导致胃出口梗阻（GOO）。胃和十二指肠活检显示浆细胞浸润，其中很大一部分 IgG4 阳性。甲泼尼龙和后来的泼尼松龙治疗迅速改善了炎症标志物和症状。幽门狭窄的球囊扩张也改善了呕吐，最终恢复了口服营养。患者在吗替麦考酚酯维持治疗下完全康复。
IgG4 相关疾病（IgG4-RD）是一种多系统疾病，表现不可预测。该病例强调了 IgG4-RD 的诊断挑战，并将其确定为上消化道症状的罕见鉴别诊断。据我们所知，这是首例 IgG4-RD 累及十二指肠导致 GOO 的病例。
