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Sclerosing Mesenteritis

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Systemic Fibroinflammatory Disorders

Part of the book series: Rare Diseases of the Immune System ((RDIS))

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Abstract

Sclerosing mesenteritis (SM) is a rare, idiopathic disorder that involves the adipose tissue of the mesentery of the small intestine and sometimes colon. It is characterized by chronic, nonspecific fibrous inflammation in the mesenteric fat with varying degrees of fat necrosis, chronic inflammation, and fibrosis. The pathogenic mechanisms may involve a nonspecific response to various stimuli, including malignancy, abdominal trauma, and surgery. SM has also been associated with other chronic fibroinflammatory disorders and may arise in the context of IgG4-related disease. The disease typically affects middle-aged to older adults and is more common in men. After careful age-appropriate cancer screening, a diagnosis of SM can be made with near-certainty with abdominal CT scanning, thereby obviating the need for routine biopsy. Many patients are only mildly symptomatic and SM may even be an incidental finding in otherwise asymptomatic patients. However, the disease may also have a chronic, debilitating course. Treatment should be guided by the severity of signs and symptoms and may include different drugs, surgical procedures or both. Herein, we provide an overview of this intriguing disease, including its potential pathogenesis, pathological findings, the possible association with other fibroinflammatory disorders, and outline the diagnostic and therapeutic approach to this disease.

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Notes

  1. 1.

    PubMed search strategy:

    (“panniculitis, peritoneal”[MeSH Terms] OR (“panniculitis”[All Fields] AND “peritoneal”[All Fields]) OR “peritoneal panniculitis”[All Fields] OR (“mesenteric”[All Fields] AND “panniculitis”[All Fields]) OR “mesenteric panniculitis”[All Fields]) OR (“panniculitis, peritoneal”[MeSH Terms] OR (“panniculitis”[All Fields] AND “peritoneal”[All Fields]) OR “peritoneal panniculitis”[All Fields] OR (“sclerosing”[All Fields] AND “mesenteritis”[All Fields]) OR “sclerosing mesenteritis”[All Fields]) OR (“panniculitis, peritoneal”[MeSH Terms] OR (“panniculitis”[All Fields] AND “peritoneal”[All Fields]) OR “peritoneal panniculitis”[All Fields] OR (“mesenteric”[All Fields] AND “lipodystrophy”[All Fields]) OR “mesenteric lipodystrophy”[All Fields])

  2. 2.

    From their study [1], we analyzed data of individual patients receiving medical treatment without surgical intervention with follow-up ≥ one month and categorized patients according to four different treatment regimens. Per treatment category we calculated the number of patients, mean age (y), male sex (n), response rate (n, %) and mean follow-up (range). We also calculated dose range per drug and mean (initial) dose (mg/day) and duration (month) of prednisone and tamoxifen in those patients who received medical treatment including both prednisone and tamoxifen.

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Authors and Affiliations

  1. Department of Internal Medicine, Albert Schweitzer Hospital, Dordrecht, 444, NL-3300 AK, The Netherlands

    Eric F. H. van Bommel MD, PhD

  2. Department of Radiology, Albert Schweitzer hospital, Dordrecht, The Netherlands

    Nienke van Putte-Katier

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  1. Eric F. H. van Bommel MD, PhD
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Correspondence to Eric F. H. van Bommel MD, PhD .

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  1. Department of Nephrology, University Hospital, Parma, Italy

    Augusto Vaglio

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van Bommel, E.F.H., van Putte-Katier, N. (2017). Sclerosing Mesenteritis. In: Vaglio, A. (eds) Systemic Fibroinflammatory Disorders. Rare Diseases of the Immune System. Springer, Cham. https://doi.org/10.1007/978-3-319-41349-5_11

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  • DOI: https://doi.org/10.1007/978-3-319-41349-5_11

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