What is the appropriate management for Sclerosing Mesenteritis?

Management of Sclerosing Mesenteritis

For symptomatic sclerosing mesenteritis, initiate combination therapy with tamoxifen 10 mg twice daily plus prednisone 40 mg daily as first-line treatment, while asymptomatic patients require no treatment and only observation. 1, 2

Clinical Recognition and Diagnosis

Sclerosing mesenteritis is a rare idiopathic inflammatory condition affecting the small bowel mesentery, occurring in 0.18% to 3.14% of the population, typically presenting in the fifth or sixth decade of life. 1 The condition predominantly affects males (69.3%) with a male-to-female ratio of 2.3:1. 3

Key Presenting Features

• Abdominal pain is the most common symptom, occurring in 70% of patients 2
• Weight loss occurs in 23% of cases 2
• Diarrhea affects 25% of patients 2
• Palpable abdominal mass may be detected on physical examination 1

Risk Factors to Identify

• Prior abdominal surgery or trauma (28.6% of cases) 3
• History of autoimmune disease (5.7% of cases) 3
• Previous malignancy (8.9% of cases) 3

Diagnostic Approach

CT imaging of the abdomen is now the primary diagnostic modality, replacing the historical reliance on biopsy due to its invasive nature. 1 CT findings vary depending on the predominant tissue component (fat, inflammation, or fibrosis). 4

Biopsy should be reserved for cases where imaging is equivocal and conditions mimicking sclerosing mesenteritis cannot be excluded, including: 1

• Mesenteric lymphoma
• Metastatic carcinoid tumor
• Desmoid tumor
• Mesenteric carcinomatosis

Treatment Algorithm

Asymptomatic Patients

No treatment is required for asymptomatic patients—observation alone is appropriate. 1 Many patients remain asymptomatic throughout their disease course.

Symptomatic Patients: Medical Management

First-line therapy: Tamoxifen 10 mg twice daily PLUS prednisone 40 mg daily 1, 2

This combination demonstrates superior efficacy:

• 60% response rate with tamoxifen-prednisone combination 2
• Only 8% response rate with non-tamoxifen-based regimens 2
• 38% response rate with medical therapy alone (various regimens) 2

The dramatic difference in response rates makes this combination the clear first choice despite the absence of randomized controlled trials. 1

Alternative Medical Therapies

When tamoxifen-prednisone fails or is contraindicated, consider: 4

• Colchicine
• Immunosuppressive agents (such as azathioprine) 5
• Orally administered progesterone 4

Surgical Management

Surgery is reserved for specific complications only, not as primary treatment: 1, 3

Indications for surgical intervention:

• Persistent bowel obstruction refractory to medical management 1
• Biopsy for definitive diagnosis when imaging is inconclusive 5, 4
• Management of complications (bowel ischemia, obstruction) 3

Surgical outcomes are limited:

• Only 10% respond to surgery alone 2
• 20% respond when medical therapy is added after surgery 2
• Surgical resection is often technically limited by vascular involvement 4

Prognostic Indicators

Poor Response Predictors

Patients are more likely to have poor treatment response with: 3

• Symptom duration exceeding one month (66.7% vs 40.4%, p < 0.05)
• Underlying autoimmune disorder (14.3% vs 4.0%, p < 0.05)
• Low protein at presentation (14.3% vs 4.0%, p < 0.05)

Good Response Predictors

Patients are more likely to respond favorably with: 3

• Tender abdomen at presentation (45.0% vs 19.0%, p < 0.05)
• Leukocytosis at presentation (20.5% vs 0.0%, p < 0.05)

Complications Requiring Monitoring

Common complications include: 3

• Bowel obstruction/ileus/ischemia (23.8% of complications)
• Obstructive uropathy/renal failure (23.8% of complications)
• Chylous ascites 1
• Mesenteric vessel thrombosis 1

Mortality considerations: While sclerosing mesenteritis has an overall benign course in most cases, 17% of deaths during follow-up were attributed to complications of the disease or its treatment. 2 Disease progression and fatal outcomes have been reported, making long-term follow-up essential. 1, 2

Follow-Up Strategy

CT with three-dimensional volume rendering is optimal for accurate, noninvasive follow-up to monitor disease progression and detect potential complications. 4 Long-term follow-up is necessary given the chronic nature of the disease and potential for prolonged debilitating course. 2

Critical Pitfalls to Avoid

• Do not perform surgery as first-line treatment—medical therapy is superior, with surgery reserved only for complications 1, 2
• Do not use non-tamoxifen regimens as first-line therapy—the response rate is only 8% compared to 60% with tamoxifen-prednisone 2
• Do not assume all mesenteric masses are sclerosing mesenteritis—biopsy when malignancy cannot be excluded radiologically 1, 4
• Do not treat asymptomatic patients—observation is appropriate as many remain stable without intervention 1