Dipartimento di Fisiopatologia Medico-chirurgica e dei Trapianti, Università degli Studi di Milano, Milan, Italy
Medicina Nucleare, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
BMJ Case Rep. 2023 Feb 9;16(2):e252345. doi: 10.1136/bcr-2022-252345.
Adult-onset Still's disease (AOSD) is a rare systemic autoinflammatory disease with a broad spectrum of clinical manifestations including fever, skin rash, arthralgia and neutrophilic leucocytosis. Small bowel inflammation in AOSD has been reported in association with Crohn's disease, coeliac disease and chronic intestinal pseudo-obstruction. We have here reported the first-time case of AOSD with small bowel involvement, presenting with chronic vomiting. Fluorodeoxyglucose total-body positron emission tomography/CT showed high uptake of the duodenum and first jejunal loop. Faecal calprotectin increased. Duodenal histology revealed neutrophilic infiltrate among duodenal enterocytes and severe chronic lymphoplasmacellular infiltrate of the lamina propria. Vomiting disappeared when duodenojejunal inflammation was controlled by immunomodulatory treatments for AOSD, suggesting a possible causal relation between duodenojejunal inflammation and the symptom.
成人Still 病（AOSD）是一种罕见的全身炎症性疾病，具有广泛的临床表现，包括发热、皮疹、关节炎和中性粒细胞增多。AOSD 中的小肠炎症与克罗恩病、乳糜泻和慢性假性肠梗阻有关。我们在此报告首例 AOSD 合并小肠受累，表现为慢性呕吐。氟脱氧葡萄糖全身正电子发射断层扫描/CT 显示十二指肠和第一段空肠环摄取增加。粪便钙卫蛋白增加。十二指肠组织学显示十二指肠上皮细胞间中性粒细胞浸润和固有层严重慢性淋巴浆细胞浸润。当 AOSD 的免疫调节治疗控制了十二指肠空肠炎症时，呕吐消失，提示十二指肠空肠炎症与症状之间可能存在因果关系。
