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.
CITATION STYLE
van Bommel, E. F. H., & van Putte-Katier, N. (2017). Sclerosing Mesenteritis. In Rare Diseases of the Immune System (pp. 137–153). Springer Nature. https://doi.org/10.1007/978-3-319-41349-5_11
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