Rare Cardiology News

Disease Profile

Sclerosing mesenteritis

Prevalence
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

ageofonset-adult.svg

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

no.svg

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

no.svg

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.

no.svg

X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

no.svg

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.

no.svg

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.

no.svg

Not applicable

notapplicable.svg

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 progesterone), or other medications, either alone or in combination (i.e. glucocorticoids in combination with tamoxifen). Surgery may be needed for an obstruction, if present. Although there are case reports of patients with aggressive disease and fatalities, it appears that the condition usually remains stable or is slowly progessive. In some cases, the condition goes away on its own.[4]

Symptoms

Sclerosing mesenteritis may cause a variety of gastrointestinal and systemic symptoms, such as:[4]

  • 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

There is no standard therapy for sclerosing mesenteritis, and treatment is based on the severity and specific symptoms in each person. Several medications have been used to try to stabilize the condition either alone or in combination.[4] However, use of most of these medications are based on case reports or small case series.[3] These have included:[4][3]

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

    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

          References

          1. Sclerosing mesenteritis. MayoClinic.org. March 22, 2014; https://www.mayoclinic.org/diseases-conditions/sclerosing-mesenteritis/basics/definition/con-20037148.
          2. 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.
          3. Ehrenpreis ED, Tun NN. Mesenteric Panniculitis. National Organization for Rare Disorders (NORD). 2016; https://rarediseases.org/rare-diseases/sclerosing-mesenteritis/.
          4. Friedman LS. Sclerosing mesenteritis. UpToDate. Waltham, MA: UpToDate; June, 2017; https://www.uptodate.com/contents/sclerosing-mesenteritis.

          Rare Cardiology News