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Rare Condition Causes Inflammation, Scarring in Tissues Surrounding Small Intestine
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Rare Condition Causes Inflammation, Scarring in Tissues Surrounding Small Intestine
November 11, 2011
Dear Mayo Clinic:
Our 50-year-old son recently went to the emergency room with severe abdominal pain. The CT scan showed a mass in his abdomen and he was diagnosed with sclerosing mesenteritis. The inflammation has diminished, but he still has some discomfort. How will this affect him long-term, and what is the chance of this illness returning?
Answer:
Sclerosing mesenteritis is a rare condition that causes inflammation and scarring in the tissues surrounding the small intestine, called the mesentery. Most often, it occurs in middle-aged and older adults, and more frequently in men.
Symptoms may include abdominal pain or an obvious abdominal mass (palpable lump), weight loss, a swollen abdomen, nausea, vomiting, diarrhea, constipation and/or fever. What causes sclerosing mesenteritis is unknown. However, a Mayo Clinic study found that about 40 percent of people with the condition previously had abdominal surgery. The study also showed that people who have sclerosing mesenteritis have a hardening or rigidity, often with calcification, in the mesentery, which is not usually present with other disorders.
In some cases, sclerosing mesenteritis causes no symptoms. In others, symptoms may be minimal. In both situations, treatment may not be necessary. In fact, about half of all people with sclerosing mesenteritis may not need therapy.
In more severe cases, a medication such as tamoxifen can be helpful. Additional treatment with prednisone may provide quicker relief. One study found that 60 percent of patients who received the combination of tamoxifen and prednisone therapy had improvement in their symptoms within four months. Mayo Clinic doctors usually recommend tapering the dose of prednisone over three months, and continuing tamoxifen treatment indefinitely if symptoms are improved.
Other less common medications, such as thalidomide, colchicine, azathioprine, or cyclophosphamide, may be combined with prednisone if tamoxifen is not an option or is not successful.
Because other conditions can look like sclerosing mesenteritis, a biopsy of the involved tissue is necessary to make a definitive diagnosis. To ensure an accurate interpretation, a pathologist with expertise in diagnosing sclerosing mesenteritis should review the tissue.
The long-term outcome for patients with sclerosing mesenteritis can vary greatly from patient to patient. Many have stable disease and stable symptoms over years of follow-up; but some patients experience complications, such as blockage of the bowel or narrowing of the blood vessels that supply circulation to the bowel. Also, patients with sclerosing mesenteritis appear to be at a slightly increased risk of developing a malignancy, such as lymphoma. If your son's symptoms have improved, I recommend an annual CT scan to monitor his condition.
Unfortunately, there are no real measures that can be taken to prevent the worsening of sclerosing mesenteritis, unless the bowel is partially blocked. In that case, a low-fiber diet may help. This involves limiting intake of high fiber foods and other foods that are difficult to digest. Your son should talk to his physician about this and other treatment options.
— Darrell Pardi, M.D., Gastroenterology, Mayo Clinic, Rochester, Minn.
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