Idiopathic sclerosing mesenteritis

Idiopathic Sclerosing Mesenteritis (ISM) is a rare condition characterized by chronic, non-specific inflammation of the mesentery, the fold of tissue that attaches the intestines to the abdominal wall and supplies it with blood and nerves. This disease is part of a broader category of diseases known as mesenteric panniculitis or sclerosing mesenteritis, and it involves a spectrum of inflammatory reactions in the mesentery. The cause of ISM is unknown, which is what the term "idiopathic" suggests. It is a condition that can mimic many other abdominal diseases, making its diagnosis challenging.

Symptoms

The symptoms of Idiopathic Sclerosing Mesenteritis can vary widely among individuals, but they often include abdominal pain, nausea, vomiting, fever, unintentional weight loss, and a palpable abdominal mass. Some patients may experience more severe complications such as intestinal obstruction or ischemia, due to the inflammation and fibrosis affecting the blood supply to the intestines.

Diagnosis

Diagnosis of ISM typically involves a combination of clinical evaluation, imaging studies, and histopathological analysis. Imaging techniques such as Computed Tomography (CT) scans or Magnetic Resonance Imaging (MRI) are commonly used to identify the characteristic features of the disease, such as a "misty mesentery" appearance. However, a definitive diagnosis usually requires a biopsy of the affected mesentery to observe the specific histological patterns of inflammation and fibrosis.

Treatment

There is no standardized treatment protocol for Idiopathic Sclerosing Mesenteritis, and the management of the condition is largely symptomatic. Treatment options may include anti-inflammatory medications, corticosteroids, and immunosuppressive agents to reduce inflammation. In cases where the disease leads to severe complications, surgical intervention may be necessary to remove obstructions or ischemic sections of the intestine.

Epidemiology

The exact prevalence of Idiopathic Sclerosing Mesenteritis is unknown due to its rarity and the lack of comprehensive studies. It can occur at any age but is most commonly diagnosed in middle-aged and older adults. There is no clear gender predilection.

Prognosis

The prognosis for individuals with Idiopathic Sclerosing Mesenteritis varies. Some patients may experience a benign course with minimal symptoms, while others may have a progressive disease that leads to significant morbidity. The response to treatment is also variable, and some cases may resolve spontaneously.

See Also

• Mesentery
• Inflammation
• Fibrosis
• Mesenteric Panniculitis

NIH genetic and rare disease info

• NIH info on Idiopathic sclerosing mesenteritis

Idiopathic sclerosing mesenteritis is a rare disease.