Management of Sclerosing Mesenteritis
For symptomatic sclerosing mesenteritis, initiate combination therapy with tamoxifen plus prednisone, as this regimen demonstrates a 60% response rate compared to only 8% with non-tamoxifen-based treatments. 1
Initial Assessment and Diagnosis
- Confirm diagnosis with CT imaging showing characteristic mesenteric fat stranding, soft tissue mass, and "fat ring sign" (preserved fat around mesenteric vessels), though histopathologic confirmation via biopsy may be needed in unclear cases 1, 2
- Evaluate for triggering factors including recent abdominal surgery, trauma, autoimmune disease, or underlying malignancy, as these may influence treatment approach 2, 3
- Assess symptom severity focusing on abdominal pain (present in 70% of cases), diarrhea (25%), weight loss (23%), and any obstructive symptoms 1
Treatment Algorithm
Asymptomatic or Minimally Symptomatic Disease
- Watchful waiting is appropriate for patients discovered incidentally without significant symptoms, as sclerosing mesenteritis can have a benign course in approximately 52% of untreated cases 1, 2
- Serial imaging every 3-6 months to monitor for progression or development of complications 2
Symptomatic Disease Requiring Medical Therapy
- First-line: Tamoxifen 20-40 mg daily plus prednisone (typically starting at 40 mg daily with gradual taper), as this combination achieved 60% response rate in the largest case series 1
- Duration of therapy: Continue for at least 6-12 months with gradual steroid taper once clinical improvement is documented 1, 4
- Tamoxifen monotherapy may be considered in patients who cannot tolerate corticosteroids, with documented cases of complete resolution, though response rates are lower than combination therapy 4
Surgical Indications
- Emergency surgery is indicated for complications including bowel perforation, complete obstruction, or bowel ischemia 3, 5
- Elective surgery alone (without medical therapy) shows only 10% response rate and should be reserved for localized disease amenable to complete resection 1
- Surgery followed by medical therapy improves outcomes to 20% response rate when complete resection is not achievable 1
Critical Management Considerations
Common pitfalls to avoid:
- Do not use non-tamoxifen immunosuppressive regimens as first-line therapy, as they demonstrate only 8% response rates compared to 60% with tamoxifen-based treatment 1
- Do not delay treatment in symptomatic patients, as 17% of deaths in the largest series were attributed to complications of sclerosing mesenteritis or its treatment 1
- Search for and eradicate any triggering focus (such as occult perforation), as this is paramount for successful outcomes even when immunosuppressive therapy is used 5
Monitoring and Follow-up
- Monitor for treatment-related complications including steroid side effects and tamoxifen-associated thromboembolic events 1
- Serial CT imaging to assess radiographic response, typically at 3-6 month intervals during active treatment 2, 4
- Long-term follow-up is essential as the disease can have a prolonged, debilitating course with potential for relapse after treatment discontinuation 1
Prognosis
- Overall mortality directly attributable to sclerosing mesenteritis is 17%, emphasizing that while often benign, this condition can be fatal 1
- Medical therapy alone achieved 38% response rate in the largest case series, supporting aggressive medical management in symptomatic patients 1
- Complete symptomatic and radiographic resolution is achievable with appropriate therapy, particularly with tamoxifen-based regimens 4