Fatal Acute Intestinal Obstruction with Hemophagocytic Lymphohistiocytosis and Multiple Organ Failure in Adult-Onset Still's Disease: A Rare Case Report

Abstract

Background: Adult-onset Still's disease (AOSD) is a systemic autoinflammatory disorder characterized by unpredictable multi-organ involvement. Although gastrointestinal complications are uncommon in AOSD, they can be life-threatening and present significant diagnostic and management challenges.

Case summary: We report the case of a 68-year-old man with AOSD who developed acute intestinal obstruction, a rare and critical complication. Imaging revealed significant colonic wall thickening, with a maximum thickness of 2.6 cm on contrast-enhanced computed tomography. The clinical status of the patient deteriorated, further complicated by the onset of hemophagocytic lymphohistiocytosis (HLH) and multi-organ failure, including acute renal dysfunction. Despite receiving intensive care and aggressive treatment, including supportive measures and immunosuppressive therapy, the patient succumbed to his illness.

Conclusion: This case underscores the importance of recognizing rare gastrointestinal and systemic complications in patients with AOSD. Early identification and prompt multidisciplinary management of conditions such as HLH and acute intestinal obstruction are essential for improving outcomes in such critical scenarios.

Keywords: acute intestinal obstruction; acute intestinal pseudo-obstruction; adult-onset Still’s disease; autoimmune disease; colonic; critical care; hemophagocytic lymphohistiocytosis; macrophage activation syndrome; multidisciplinary team (MDT); multiple organ failure.

Figure 1

Imaging findings from radiologic and bone marrow examinations of the patient during the hospital stay. (A) Fluorodeoxyglucose (18F-FDG) positron emission tomography (PET) imaging revealed mild FDG uptake in multiple small lymph nodes, a diffusely swollen spleen with mildly increased FDG metabolism compared to the liver, and diffuse FDG uptake in the bone marrow, including the spine, pelvis, and sternum. (B) Bone marrow cytology revealed no abnormalities in the proportion or morphology of mature lymphocytes. However, the proportion of plasma cells was elevated. (C) Bone marrow cytology revealed a reduced proportion of mature lymphocytes with no observable morphological abnormalities. Monocytoid reticular cells accounted for 5% of the cell population, while phagocytic reticular cells, identified by their engulfment of erythrocytes and platelets, constituted 2% of the cell population. (D) Abdominal computed tomography (CT) with contrast enhancement revealed marked edema and thickening of the colonic and rectal walls, with a maximum wall thickness of 1.7 cm. Abdominal and pelvic effusion, along with significant ascites, was evident. (E) Abdominal CT with contrast enhancement showed pronounced edema and thickening of the colonic and rectal walls, with a maximum thickness of 2.6 cm. Abdominal and pelvic effusion, accompanied by substantial ascites, was observed. The splenic artery and superior mesenteric artery were notably slender.