Mesenteric Panniculitis

National Organization for Rare Disorders, Inc.

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Important
It is possible that the main title of the report Mesenteric Panniculitis is not the name you expected.

Disorder Subdivisions

  • None

General Discussion

Summary
Mesenteric panniculits, also known as sclerosing mesenteritis, belongs to a spectrum of rare diseases of the fatty (adipose) tissue of the mesentery. Mesenteric panniculitis is characterized by fat degeneration and necrosis, chronic inflammation, and at times, scarring and fibrosis of fatty tissue within the mesentery. This inflammatory and at times progressive condition is most likely on the spectrum of autoimmune disorders.

The mesentery is a fold of tissue within the peritoneum that supports and attaches the small and large intestines to the walls of the abdomen. The mesentery contains fat, blood vessels, lymphatic tissue, lymphatic vessels and other forms of connective tissue. The peritoneum is a membrane that lines the abdominal cavity and covers the abdominal organs. The mesentery of the small intestine is most often affected in mesenteric panniculitis. Although the exact cause of mesenteric panniculitis remains unknown, the disease has been associated with a variety of other conditions, including neoplasms, other autoimmune diseases and abdominal trauma.

Clinical symptoms of mesenteric panniculitis are highly variable. Some individuals have few or no noticeable symptoms; others may be greatly affected by a variety of complaints including abdominal pain, nausea/vomiting, bloating, early satiety, loss of appetite and diarrhea or constipation. Systemic symptoms, especially fatigue, commonly occur in patients with mesenteric panniculitis. A computerized tomogram (CT) or other imaging of the abdomen shows thickening of the mesentery, sometimes with lymph node enlargement. Due to its variable clinical presentation and rarity, the diagnosis of mesenteric panniculitis is often delayed. Tissue biopsy is required to secure the diagnosis of mesenteric panniculitis and rule out neoplastic infiltration of the mesentery. However, not every patient suspected of having the disease will require a biopsy. There is limited information on the natural history of mesenteric panniculitis, but a stable clinical course is generally anticipated. Due to the rarity of mesenteric panniculitis, there is little prospective data available on its treatment. Nonetheless, corticosteroids and other medications directed at lowering the degree of inflammation and other medications that improve symptoms are felt to be the mainstay of treatment for mesenteric panniculitis.

Introduction

Mesenteric panniculitis is a rare disorder that is part of a spectrum of diseases affecting the mesentery, a portion of the peritoneum that supports and attaches the intestines to the abdominal wall. Individuals with mesenteric panniculitis develop inflammation and necrosis of the fatty tissue of the mesentery, especially in the area of the small bowel. The condition progresses to cause chronic inflammation of the mesentery. In some patients, ongoing inflammation can result in scarring (fibrosis) of the mesentery.

Mesenteric panniculitis was first described in the medical literature in 1924 as "retractile mesenteritis". Since that time, alternative names have been used to describe the condition including mesenteric panniculitis, retractile mesenteritis and mesenteric lipodystrophy. These names denote the predominant features of the disease process in the mesentery. Mesenteric panniculitis refers to a prevalence of inflammation; sclerosing mesenteritis (or retractile mesenteritis) refers to a predominance of fibrosis; and mesenteric lipodystrophy refers to a predominance of fat necrosis. At present, mesenteric panniculitis is the most commonly used and recommended name for the condition. Additional names that have been used for this disorder include mesenteric fibrosis, mesenteric sclerosis, liposclerotic mesenteritis, mesenteric Pfeifer-Weber-Christian disease, mesenteric lipogranuloma, xanthogranulomatous mesenteritis, inflammatory pseudotumor, retroperitoneal xanthogranuloma and isolated lipodystrophy.

Supporting Organizations

Digestive Disease National Coalition

507 Capitol Court, NE
Suite 200
Washington, DC 20002
Tel: (202)544-7497
Fax: (202)546-7105
Email: ddnc@hmcw.org
Website: http://www.ddnc.org

Genetic and Rare Diseases (GARD) Information Center

PO Box 8126
Gaithersburg, MD 20898-8126
Tel: (301)251-4925
Fax: (301)251-4911
Tel: (888)205-2311
Website: http://rarediseases.info.nih.gov/GARD/

International Mesenteric Panniculitis Society

Email: milliemoos531@yahoo.com
Website: https://groups.yahoo.com/neo/groups/InternationalMesentericPanniculitisSociety/info

NIH/National Institute of Diabetes, Digestive & Kidney Diseases

Office of Communications & Public Liaison
Bldg 31, Rm 9A06
Bethesda, MD 20892-2560
Tel: (301)496-3583
Email: NDDIC@info.niddk.nih.gov
Website: http://www2.niddk.nih.gov/

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders (NORD). For a full-text version of this report, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only.

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Last Updated:  7/25/2016
Copyright  2016 National Organization for Rare Disorders, Inc.