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Disease Profile
Sclerosing mesenteritis
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
Unknown
Age of onset
Adult
ICD-10
K65.8
Inheritance
Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.
Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.
X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.
Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.
Not applicable
Other names (AKA)
Retractile mesenteritis; Liposclerotic mesenteritis; Mesenteric lipogranuloma;
Categories
Digestive Diseases
Summary
Sclerosing mesenteritis is one of many terms that describes a spectrum of inflammatory disorders that affect the mesentery. The mesentery is the membrane that anchors the small intestine to the back of the abdominal wall.[1][2][3] The condition mostly affects men between their 40s and 60s, but women and children can also be affected. It may result in a variety of symptoms including abdominal pain, nausea and vomiting, constipation or diarrhea, weight loss, and fever.[4] Some people have an abdominal mass. The cause of the condition is poorly understood. Suggested possible causes have included previous abdominal surgery or trauma, autoimmunity, paraneoplastic syndromes, ischemic injury, and infection.[4]
Treatment depends on the symptoms in each person and may include the use of glucocorticoids, hormonal therapy (tamoxifen or
Symptoms
- Abdominal pain
- Nausea and vomiting
- Diarrhea or constipation
- Rectal bleeding
- Weight loss
- Abdominal distention (bloating)
- Fever
- A palpable abdominal mass
Reports in the literature have indicated that symptoms can last anywhere from days to years.[4] In severe cases, the symptoms can have a significant effect on quality of life.[3] Some individuals develop complications such as a bowel blockage.[1][3]
Treatment
- Glucocorticoids (
prednisone ) - Hormonal therapy (tamoxifen and
progesterone ) - Low dose naltrexone
- Colchicine
- Cyclophosphamide
- Thalidomide
Surgery is typically reserved for obstructive complications (bowel obstruction). In some cases, the condition goes away on its own.[4]
Organizations
Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.
Organizations Supporting this Disease
-
The Digestive Disease National Coalition
507 Capitol Court, NE
Suite 200
Washington, DC 20002
Telephone: 202-544-7497
Fax: (202) 546-7105
E-mail: [email protected]
Website: https://www.ddnc.org/
Learn more
These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.
Where to Start
- The Mayo Clinic Web site provides information on sclerosing mesenteritis. Click on the link to view the information pages.
- The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.
- The The Cleveland Clinic Web site has an information page on Sclerosing mesenteritis. Click on the Cleveland Clinic link to view this page.
In-Depth Information
- Medscape Reference provides information on mesenteric tumors, including sclerosing mesenteritis (also known as mesenteric lipodystrophy). You may need to register to view the article, but registration is free.
- The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
- PubMed is a searchable database of medical literature and lists journal articles that discuss Sclerosing mesenteritis. Click on the link to view a sample search on this topic.
Selected Full-Text Journal Articles
- Akram s, Pardi D, Schaffner J, Smyrk T. Sclerosing mesenteritis: Clinical features, treatment, and outcome in ninety-two patients. Clinical Gastroenterology and Hepatology. May 2007;5(5):589-596.
References
- Sclerosing mesenteritis. MayoClinic.org. March 22, 2014; https://www.mayoclinic.org/diseases-conditions/sclerosing-mesenteritis/basics/definition/con-20037148.
- S Akram, DS Pardi, JA Schaffner, TC Smyrk. Sclerosing mesenteritis: clinical features, treatment, and outcome in ninety-two patients. Clin Gastroenterol Hepatol. 2007 May; 5(5):589-96. https://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=DetailsSearch&Term=17478346%5Buid%5D.
- Ehrenpreis ED, Tun NN. Mesenteric Panniculitis. National Organization for Rare Disorders (NORD). 2016; https://rarediseases.org/rare-diseases/sclerosing-mesenteritis/.
- Friedman LS. Sclerosing mesenteritis. UpToDate. Waltham, MA: UpToDate; June, 2017; https://www.uptodate.com/contents/sclerosing-mesenteritis.
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