Idiopathic sclerosing mesenteritis

Idiopathic sclerosing mesenteritis

Sclerosing mesenteritis , note the hemosidirin, chronic inflammation
Classification and external resources
ICD-10 K65.4

Idiopathic sclerosing mesenteritis (ISM) or mesenteric panniculitus is a rare disease of the small intestine, characterized by chronic inflammation and eventual fibrosis of the mesentery.[1] It has also been called mesenteric lipodystrophy, or retractile mesenteritis.[2]

Signs and symptoms

Sclerosing mesenteritis may present with no or nearly no signs or symptoms, but many people have chronic and severe pain in the abdomen as the most common chief complaint. Other people have chronic problems with bowel movements, resulting in diarrhea, bloating, gas, and cramping which can range from severe to mild.[2][3]

The disorder is identified by histopathology showing fat necrosis, fibrosis and chronic inflammation of the small intestine. Examination of the mesentery may indicate a solitary mass, but diffuse mesentery thickening is common.[2][4]

It often mimics other abdominal diseases such as pancreatic or disseminated cancer.[5] CT scanning is important for making the initial diagnosis.[6]

Mechanism

Several causes of sclerosing mesenteritis have been suggested, but the principal pathophysiological mechanism is probably autoimmune. Trauma and prior surgical procedures have also been indicated as possibilities.[7] It can be found in IgG4-related disease.[8]

Epidemiology

The epidemiology of Idiopathic sclerosing mesenteritis disease is extremely rare and has only been diagnosed in about an estimated 300 patients worldwide to date (as of 2014), it is probably under diagnosed.[9] It can occur in children.[10]

Research

The Mayo Clinic in Rochester, MN, reported a large study of 92 patients, with widely ranging severity of their symptoms. The majority were male, average age 65 years. They commonly had abdominal pain (70%), diarrhea (25%) and weight loss (23%). Depending on the stage of the scarring and fibrosis, several different treatments including surgery, for bowel obstruction, or drugs were used to halt the progression of the disease.[3]

In that case series, 56% of patients received only pharmacological therapy, most often receiving tamoxifen with a reducing dose of reducing prednisone, or also had colchicine, azathioprine or thalidomide.[3]

Their findings suggest that sclerosing mesenteritis can be debilitating although relatively benign. Symptomatic patients benefited from medical therapy, usually tamoxifen and prednisone, but further follow-up information would strengthen these results.[3]

References

  1. ↑ "Sclerosing mesenteritis | Disease | Overview | Office of Rare Diseases Research (ORDR-NCATS)". rarediseases.info.nih.gov. Retrieved 2015-08-12.
  2. 1 2 3 Emory TS, Monihan JM, Carr NJ, Sobin LH (1997). "Sclerosing mesenteritis, mesenteric panniculitis and mesenteric lipodystrophy: a single entity?". Am J Surg Pathol 21 (4): 392–8. PMID 9130985.
  3. 1 2 3 4 Akram S, Pardi DS, Schaffner JA, Smyrk TC (2007). "Sclerosing mesenteritis: clinical features, treatment, and outcome in ninety-two patients.". Clin Gastroenterol Hepatol 5 (5): 589–96. doi:10.1016/j.cgh.2007.02.032. PMID 17478346.
  4. ↑ Fletcher, Christopher D. M. (2007-03-29). Diagnostic Histopathology of Tumors: 2-Volume Set with CD-ROMs. Elsevier Health Sciences. ISBN 9780702032059.
  5. ↑ Scudiere JR, Shi C, Hruban RH, Herman JM, Fishman EK, Schulick RD, et al. (2010). "Sclerosing mesenteritis involving the pancreas: a mimicker of pancreatic cancer.". Am J Surg Pathol 34 (4): 447–53. doi:10.1097/PAS.0b013e3181d325c0. PMC 2861335. PMID 20351487.
  6. ↑ Horton KM, Lawler LP, Fishman EK (2003). "CT findings in sclerosing mesenteritis (panniculitis): spectrum of disease.". Radiographics 23 (6): 1561–7. doi:10.1148/rg.1103035010. PMID 14615565.
  7. ↑ Disorders, National Organization for Rare (2003-01-01). NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. ISBN 9780781730631.
  8. ↑ Kim JH, Byun JH, Lee SS, Kim HJ, Lee MG (2013). "Atypical manifestations of IgG4-related sclerosing disease in the abdomen: imaging findings and pathologic correlations.". AJR Am J Roentgenol 200 (1): 102–12. doi:10.2214/AJR.12.8783. PMID 23255748.
  9. ↑ Federle, Michael P.; Raman, Siva P. (2015-07-29). Diagnostic Imaging: Gastrointestinal. Elsevier Health Sciences. ISBN 9780323400404.
  10. ↑ Viswanathan, Vijay; Murray, Kevin J (2010). "Idiopathic sclerosing mesenteritis in paediatrics: Report of a successfully treated case and a review of literature". Pediatric Rheumatology (BioMed Central) 8 (5). doi:10.1186/1546-0096-8-5.

Further reading

External links

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