Idiopathic sclerosing mesenteritis

Did You Know?

• It can occur in children.
• The disorder is probably underdiagnosed.

Signs and symptoms

For some people, idiopathic sclerosing mesenteritis causes no symptoms at all. However, many patients report ongoing, severe abdominal pain as their main issue. Others deal with chronic bowel troubles like diarrhea, bloating, gas, and cramping, which can be mild or severe. Doctors diagnose the condition through tissue analysis, which reveals fat necrosis, fibrosis, and ongoing inflammation in the small intestine. The mesentery may show a single lump, but more often it appears diffusely thickened.

Diagnosis

A CT scan can help assess the disease; "misty mesentery" describes increased mesenteric fat density but is not specific and can occur in other conditions such as mesenteric oedema, lymphedema, haemorrhage, or neoplastic and inflammatory cells. Mesenteric lymph nodes are rarely larger than 10 mm; larger nodes should prompt further investigation with PET scan or biopsy. MRI may show intermediate T1 intensity and variable T2 intensity depending on oedema and fibrosis. CT scanning is important for initial diagnosis.

Research

A large study of 92 patients from the Mayo Clinic reported widely ranging severity of symptoms. The majority were male with an average age of 65 years; common symptoms included abdominal pain (70%), diarrhea (25%), and weight loss (23%). In that series, 56% received only pharmacological therapy, most often tamoxifen with a reducing dose of prednisone, or also colchicine, azathioprine, or thalidomide. Symptomatic patients benefited from medical therapy, usually tamoxifen and prednisone.