Prognosis of Sclerosing Mesenteritis
Sclerosing mesenteritis generally has a benign prognosis with most patients experiencing a favorable long-term outcome, though approximately 17% of deaths may be attributed to complications of the disease or its treatment. 1
Overall Disease Course and Mortality
The natural history of sclerosing mesenteritis is typically benign, but the disease can follow a prolonged and debilitating course in some patients. 1 In the largest case series of 92 patients followed over 23 years, 18 deaths occurred during the study period, with 17% of these deaths directly attributed to complications of sclerosing mesenteritis or its treatment. 1 Despite potential for severe complications, good prognosis can be expected even in cases with complex courses and life-threatening complications. 2
Clinical Presentation and Symptom Burden
The disease typically presents in the fifth or sixth decade of life, affecting approximately 0.18% to 3.14% of the population. 3 Many patients remain asymptomatic and require no treatment. 3 When symptomatic, patients commonly experience:
- Abdominal pain (70% of cases) 1
- Diarrhea (25% of cases) 1
- Weight loss (23% of cases) 1
- Palpable abdominal mass on examination 3
Disease Progression and Complications
While the overall course is benign in most cases, disease progression and fatal outcomes have been reported. 3 Serious complications can occur from both the disease itself and its surgical treatment. 2 Potential complications include:
- Bowel obstruction (may require surgical intervention in refractory cases) 3
- Chylous ascites 3
- Mesenteric vessel thrombosis 3
- Occult bowel perforation (rare but potentially life-threatening) 2
Treatment Response and Long-term Outcomes
Symptomatic patients demonstrate variable but generally favorable treatment responses:
- 38% respond to medical therapy alone 1
- 60% improve with tamoxifen plus prednisone combination (the most effective regimen) 1
- 20% respond to medical treatment after surgery 1
- 10% respond to surgery alone 1
- Only 8% improve with non-tamoxifen-based regimens 1
Clinical remission can be achieved with appropriate therapy, with follow-up imaging at 6 and 12 months typically documenting stability of lesions. 4
Prognostic Factors
Patients at potentially higher risk include those with:
- History of abdominal surgery 3
- Autoimmune disease 3
- Extensive disease mimicking advanced intra-abdominal malignancy 2
Key Clinical Pitfalls
The main prognostic challenge is distinguishing sclerosing mesenteritis from conditions that mimic it on imaging, including mesenteric lymphoma, metastatic carcinoid tumor, desmoid tumor, and mesenteric carcinomatosis. 3 If clinical suspicion is equivocal, biopsy may be warranted for definitive diagnosis to avoid misclassifying a malignant process. 3
Asymptomatic patients require no treatment and can be monitored conservatively. 3 For symptomatic patients, early identification and full eradication of any possible triggering focus (such as occult perforation) is of paramount importance for optimal outcomes. 2