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NORD is very grateful to Eli D. Ehrenpreis, MD, Chief, Department of Gastroenterology and Endoscopy, Highland Park Hospital, NorthShore University Health System and Parakkal Deepak, MD, LifeTract Clinical Research Fellow, Department of Gastroenterology, NorthShore University Health System, for assistance in the preparation of this report.
Sclerosing mesenteritis is a rare spectrum of disease characterized by degeneration (necrosis), inflammation and scarring (fibrosis) of fatty (adipose) tissue of the mesentery. The mesentery is a fold of tissue of the peritoneum that supports and attaches the intestines to the wall of the abdomen. The peritoneum is the membrane that lines the abdominal cavity and covers the abdominal organs. The small bowel mesentery is most often affected. The symptoms of sclerosing mesenteritis can vary greatly from one person to another and can include a variety of gastrointestinal symptoms such as abdominal pain, nausea/vomiting, bloating, loss of appetite and diarrhea or constipation. Non-gastrointestinal symptoms include fatigue, weight loss, night sweats and fever. Some individuals do not develop any noticeable symptoms (asymptomatic). The exact cause of sclerosing mesenteritis is unknown.
Sclerosing mesenteritis was first described in the medical literature in 1924 as retractile mesenteritis. Since that time numerous, varied names have been used to describe the condition. Three prominent names that have been used in the literature are retractile mesenteritis, mesenteric panniculitis, and mesenteric lipodystrohpy. These three names denote the predominant feature in a particular case. Sclerosing mesenteritis or retractile mesenteritis refers to a predominance of fibrosis; mesenteric panniculitis refers to a predominance of inflammation; and mesenteric lipodystrophy refers to a predominance fat necrosis. Because of the presence of some degree of fibrosis, many physicians advocate sclerosing mesenteritis as the best name for this spectrum of disease.
Additional names that have been used for this disorder include mesenteric fibrosis, mesenteric sclerosis, liposclerotic mesenteritis, mesenteric Pfeifer-Weber-Christian disease, mesenteric lipogranuloma, and xanthogranulomatous mesenteritis.
Sclerosing mesenteritis represents a spectrum of disease affecting the mesentery, a fold of tissue of the peritoneum that supports and attaches the intestines to the wall of the abdomen. Individuals with sclerosing mesenteritis develop degeneration (necrosis) of the fatty tissue of the mesentery, especially the small bowel mesentery. The condition progresses to cause chronic inflammation of the mesentery. Eventually, thickening and scarring (fibrosis) of the mesentery develops as well.
Some of the affected individuals do not develop any noticeable symptoms (asymptomatic) and a diagnosis may be made incidentally during a routine procedure undertaken for other reasons. Generally, sclerosing mesenteritis is reported as a benign, self-limited disorder with a favorable prognosis that may even go away on its own (spontaneous regression). However, in some cases, the disorder can potentially be severe and debilitating. The symptoms of sclerosing mesenteritis that can develop are highly variable from one person to another and, therefore, it is important to note that affected individuals may not have all of the symptoms discussed below. Affected individuals should talk to their physician and medical team about their specific case, associated symptoms and overall prognosis.
The most common symptom associated with sclerosing mesenteritis is abdominal pain, which can be severe. Additional symptoms can include nausea/vomiting, constipation, diarrhea, fatigue, fever, night sweats, swelling (distention) of the abdomen, loss of appetite, and unintended weight loss. Some individuals may have a palpable abdominal mass. Additional symptoms may include raised body temperature (pyrexia), a general feeling of poor health (malaise), and swelling of the abdomen due to the accumulation of a milky fluid containing lymph and fat (chylous ascites).
In some cases, affected individuals may develop small bowel obstruction or acute abdomen. Small bowel obstruction prevents the passage of food through the intestines and can cause a variety of nonspecific gastrointestinal symptoms as well as a reduced ability to absorb nutrients (malabsorption). Acute abdomen is a serious condition characterized by the sudden onset of acute abdominal pain and additional gastrointestinal symptoms. Acute abdomen requires immediate treatment.
The exact cause of sclerosing mesenteritis is unknown (idiopathic). Numerous theories have been proposed to explain the development of the disorder including a lack of blood flow (ischemia) to the mesentery region, the use of certain drugs, infection and trauma to the affected region. Some individuals with sclerosing mesenteritis have had prior abdominal surgery. It is possible that sclerosing mesenteritis may have different causes in different people.
Some researchers have proposed that sclerosing mesenteritis is an autoimmune disorder. Autoimmune disorders are caused when the body’s natural defenses (antibodies, lymphocytes, etc.) against invading organisms suddenly begin to attack healthy tissue. Researchers believe that multiple factors including genetic and environmental ones play a role in the development of autoimmune disorders. The favorable response of some individuals with sclerosing mesenteritis to drugs that suppress the immune system supports this theory.
The symptoms of sclerosing mesenteritis in most cases are believed to occur due to direct physical effect of a mass in the mesentery encasing the bowel, blood vessels and other nearby structures.
According to the medical literature, some individuals with sclerosing mesenteritis have had an underlying form of cancer (malignancy) such as lymphoma, Hodgkin disease, leukemia, gastric carcinoma or colon cancer. However, in other reports, no increase in the occurrence of cancer is seen. The relationship, if any, between sclerosing mesenteritis and cancer is unknown. Some researchers have speculated that, in some cases, sclerosing mesenteritis may be a paraneoplastic syndrome, which is a disease or symptom that is a consequence of the presence of cancer in the body.
Other reports in the medical literature have noted a possible association between sclerosing mesenteritis and other fibro-sclerotic disorders. Some researchers believe that sclerosing mesenteritis may be part of a larger spectrum of disease in which inflammation and fibrosis affect multiple organ systems of the body. Fibro-sclerotic disorders that have been reported to occur with sclerosing mesenteritis include retroperitoneal fibrosis, Sjogren syndrome and sclerosing pancreatitis. More research is necessary to determine whether an association among these disorders exists.
In reported cases, sclerosing mesenteritis affects males more often than females, by a ratio of approximately 2:1. . The exact incidence and prevalence of the disorder is unknown. More than 250 cases have been reported in the medical literature. Sclerosing mesenteritis most often affects individuals in their 50s or 60s and the incidence increases with age. Although uncommon, sclerosing mesenteritis can affect children as well.
Symptoms of the following disorders can be similar to those of sclerosing mesenteritis. Comparisons may be useful for a differential diagnosis.
Numerous conditions can cause signs and symptoms similar to those associated with sclerosing mesenteritis. Such conditions include primary or metastatic mesentery cancer, pancreatic adenocarcinoma, gastrointestinal lymphoma, desmoids tumors, inflammation of the pancreas (pancreatitis), irritable bowel syndrome, idiopathic nodular panniculitis, and retroperitoneal fibrosis. (For more information on these disorders, choose the specific disorder name as your search term in the Rare Disease Database.)
A diagnosis of sclerosing mesenteritis is made based upon identification of characteristic symptoms, a detailed patient history, and a thorough clinical evaluation. Many cases are diagnosed incidentally when individuals are undergoing an unrelated procedure.
Clinical Testing and Work-Up
Individuals with sclerosing mesenteritis have a soft-tissue mass that is detectable by radiographic study such as computerized tomography (CT) scanning or magnetic resonance imaging (MRI). During CT scanning, a computer and x-rays are used to create a film showing cross-sectional images of certain tissue structures. An MRI uses a magnetic field and radio waves to produce cross-sectional images of particular organs and bodily tissues. Surgical sampling and microscopic study (biopsy) of affected tissue is required to rule out other conditions and help to confirm a diagnosis of sclerosing mesenteritis.
There is no standardized treatment regimen for individuals with sclerosing mesenteritis. Most treatment recommendations are based on case reports or small case series. Only thalidomide has been tested in an open-label trial setting. Treatment is directed toward the specific symptoms that are apparent in each individual. Physicians will tailor treatment based on the stage of the disease and what is considered best for an individual case.
During the first stage, when fat necrosis occurs, many physicians recommend no treatment. Generally, individuals with no symptoms are not treated, but are regularly monitored to see whether the disorder progresses (watch and wait). In some cases, the disease may remain at this stage for years or even regress or go away on its own.
The second stage, chronic inflammation, is usually treated with corticosteroids to help control or suppress inflammation. Corticosteroids may be administered alone or in combination with other drugs. Recently, individuals with sclerosing mesenteritis have also been treated with drugs that suppress the activity of the immune system (immunosuppressive therapy), which has been beneficial in some cases.
Additional drugs that have been used to treat individuals with sclerosing mesenteritis include azathioprine, colchicine, tamoxifen, cyclophosphamide, pentoxifylline, thalidomide, and various hormonal therapies. These drugs have had varying degrees of success and have proven effective in some individuals, but ineffective in others. Because of the rarity of sclerosing mesenteritis, controlled clinical studies on these medications have not been performed.
When individuals with sclerosing mesenteritis have symptoms similar to those associated with bowel obstruction and do not respond to other therapies (refractory), surgery may be recommended. Surgery may also be recommended to remove a mass. Surgery for individuals with sclerosing mesenteritis has had mixed results.
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Mayo Clinic for Medical Education and Research. Sclerosing Mesenteritis. . Available at: http://www.mayoclinic.org/sclerosing-mesenteritis/ Accessed On: October 1, 2011.
Tyer NM, Fang MA. Sclerosing mesenteritis: examining a poorly understood disease. Clinical Geriatrics. December 30, 2010. Available at: http://www.clinicalgeriatrics.com/articles/Sclerosing-Mesenteritis-Examining-Poorly-Understood-Disease Accessed On: October 1, 2011
Report last updated: 2011/12/18 00:00:00 GMT+0