Duodenojejunal inflammation causing chronic vomiting in adult-onset Still’s disease

Abstract

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.

Adult-onset Still's Disease (AOSD) with Gastrointestinal Lesions

“…Other known symptoms of AOSD include weight loss, myalgia, drug allergies, nephropathy, pleurisy, pericarditis, and interstitial pneumonia (3,4). The only report of gastrointestinal lesions due to AOSD was that of a case demonstrating duodenitis (5). We herein report that the gastrointestinal lesions observed in a Japanese woman might have been caused by AOSD.…”

“…In addition to these diagnostic criteria, symptoms such as weight loss, myalgia, drug allergies, nephropathy, pleurisy, pericarditis, and interstitial pneumonia are known symptoms of AOSD (3,4). The only report of gastrointestinal lesions due to AOSD was duodenitis (5). Although a few cases of Crohn's disease, ulcerative colitis, and celiac disease have been reported to be complicated by AOSD, the gastrointestinal lesions in these cases were not caused by AOSD, but rather had been caused by a different associated disease (6-10) (Table 2).…”

Intestinal pseudo-obstruction with life-threatening hypokalaemia in a patient with adult-onset Still’s disease

“…Only four other cases with pseudoobstruction have been previously reported [5][6][7][8] . Additionally, a recent case report described duodenojejunal inflammation causing chronic vomiting [9] , suggesting that further investigation into potential gastrointestinal involvement in AOSD may be warranted. Previous reports have implicated an affectation of the sympathetic tone of the bowel and an imbalance in the autonomic enteric nervous system, leading to colonic distension [8] .…”