mezenterik pannikülit

 

ETIOLOGY AND PATHOGENESIS

●Abdominal surgery or abdominal trauma – It is hypothesized that sclerosing mesenteritis results in genetically predisposed individuals who have abnormal responses to healing and repair of connective tissue in response to trauma 

Sclerosing mesenteritis has been reported in association with both acute abdominal injury and chronic repetitive trauma (eg, pneumatic jackhammer) and inflammation (eg, occult ileal perforation) . The use of powdered surgical gloves has also been implicated in the development of abdominal fibrosis as a precursor to sclerosing mesenteritis

Autoimmunity – Sclerosing mesenteritis has been reported in association with a number of immune-mediated conditions, including Riedel thyroiditis, primary sclerosing cholangitis, retroperitoneal fibrosis, and orbital pseudotumor . In addition, it has rarely been found as part of the initial presentation with autoimmune hemolytic anemia, minimal change nephropathy, systemic lupus erythematosus, relapsing polychondritis, multifocal fibrosclerosis, limited systemic sclerosis, and celiac-associated T-cell lymphoma

One series and case reports have described an association with elevated serum IgG4 and/or autoimmune pancreatitis in some patients, suggesting a possible role for IgG4-related immunopathologic processes in the development of sclerosing mesenteritis

●Paraneoplastic syndrome – Among patients with sclerosing mesenteritis, an underlying malignancy has been reported from 1 to 75 percent . Non-Hodgkin lymphoma is the most commonly associated malignancy, however, others include breast cancer, neuroendocrine tumor, melanoma, squamous carcinoma and adenocarcinoma of the lung, renal carcinoma, multiple myeloma, hepatocellular carcinoma, prostate adenocarcinoma, ovarian carcinoma, endometrial carcinoma, cervical carcinoma, angiosarcoma, and gastrointestinal adenocarcinomas . While it has been hypothesized that sclerosing mesenteritis represents a nonspecific response to an underlying malignancy and is a paraneoplastic syndrome, other studies have not demonstrated an association

●Ischemia and infection – A process mimicking sclerosing mesenteritis has been replicated in laboratory animals by a variety of methods, such as interference with the mesenteric vascular supply and injection of bacteria and bacterial toxins into the mesentery. A possible infectious etiology is consistent with case reports of sclerosing mesenteritis in patients with a history of typhoid fever, dysentery, tuberculosis, syphilis, malaria, influenza, and rheumatic fever.

Clinical features — Approximately 10 to 15 percent of patients with sclerosing mesenteritis are asymptomatic or have minimal symptoms .

The most common presenting features are abdominal pain (30 to 70 percent), systemic symptoms including fever, malaise and weight loss (20 to 23 percent), and altered bowel habits with either constipation or diarrhea (20 percent) . Based on the severity, the duration of the symptoms varies from days to years prior to the diagnosis .

An abdominal mass can be palpated in 35 to 50 percent of patients. Masses tend to be deep seated and poorly defined . Other findings include abdominal tenderness in approximately one-third of cases and distension in approximately 10 to 15 percent. Signs of peritoneal inflammation and ascites (usually chylous) are rare.

Patients may also have signs and symptoms attributable to associated conditions, such as underlying malignancy (eg, pleural effusion, fever, edema due to protein-losing enteropathy, hemolytic anemia, jaundice due to biliary obstruction)

* Non-obstructive symptoms include abdominal pain, systemic symptoms including fever, weight loss, and altered bowel habits.
¶ Patients with bowel obstruction are managed conservatively when feasible. Surgical bypass is indicated to alleviate symptoms in those whose symptoms are dominated by focal intestinal obstruction. 
Δ Treatment with azathioprine is an alternative approach.
◊ Treatment with pentoxyfylline is an alternative approach.
§ Treatment with colchicine is controversial.