Clinical associations, outcomes, and therapeutic management of patients with symptomatic sclerosing mesenteritis
Abstract
Background Sclerosing mesenteritis (SM) is a rare fibroinflammatory disease of unknown etiology that primarily affects the root of the small bowel mesentery. Associated diseases that contribute to or predispose to the development of SM have not been well-documented. Objectives We aimed to describe the clinical associations of patients diagnosed with symptomatic SM, their clinical features, outcomes, and therapeutic management. Design We performed a prospective cohort study from 2014 to 2025. Methods Eligible patients were those aged ≥ 18 years who presented at two tertiary Medical Centers in Greece with SM-specific symptoms after ruling out other causes of abdominal pain and radiographic signs indicative of the Coulier criteria on computed tomography scans (N=58). SM cases lost to follow-up were excluded (n=3). Descriptive statistics and comparative statistical analysis were used. Results In total, 55 patients with symptomatic SM were included, of whom 25.5% developed severe intra-abdominal complications, with even fatal outcomes. Colchicine in combination with corticosteroid tapering was the most frequently administered therapy (47.3%). SM improved in most patients after treatment (72.9%). During the disease course, 23.6% of patients were diagnosed with immunoglobulin G4-related disease (IgG4-RD), 9.1% had co-existing systemic autoimmune rheumatic diseases, and 9.1% were diagnosed with an abdominal/pelvic malignancy within 6 months of initial SM presentation. In the remaining cases (58.2%), an association with metabolic syndrome conditions and excessive accumulation of visceral adipose tissue was observed. Conclusion In this prospective cohort study, we identified a correlation of symptomatic SM with chronic inflammatory diseases. Certain SM cases are probably part of the IgG4 disease spectrum or systemic autoimmune rheumatic diseases. These results should be taken into account when consulting patients with symptomatic SM, as the clinical management, prognosis, and therapeutic approach may differ according to the underlying systemic disorder.