NORD gratefully acknowledges Eli D. Ehrenpreis, MD, Associate Director for Research, Advocate Lutheran General Hospital Internal Medicine Residency, Park Ridge, Illinois; Professor of Medicine, Rosalind Franklin University Medical School and Jeffery Prochet, MD, Advocate Lutheran General Hospital Internal Medicine Residency, Park Ridge, Illinois for the preparation of this report.
- sclerosing mesenteritis
- mesenteric lipodystrophy
- retractile mesenteritis
Mesenteric panniculitis, also known as sclerosing mesenteritis, belongs to a spectrum of rare diseases of the fatty (adipose) tissue of the mesentery. Mesenteric panniculitis is characterized by fat degeneration and necrosis, chronic inflammation, and at times, scarring and fibrosis of fatty tissue within the mesentery. This inflammatory and at times progressive condition is most consistent with autoimmune disorders. There is currently limited understanding of the progression of events that lead to the development of mesenteric panniculitis.
The mesentery is a fold of tissue within the peritoneum that supports and attaches the small and large intestines to the walls of the abdomen. The mesentery contains fat, blood vessels, lymphatic tissue, lymphatic vessels and other forms of connective tissue. Anatomists previously considered it to be a fragmented collection of intra-abdominal connective tissue. Recently, the anatomy of the mesentery has been clarified and the mesentery has been found to represent a continuous organ that extends from the duodenojejunal flexure to the mesorectum. The portion of the mesentery that is adjacent to the small intestine is most often affected in mesenteric panniculitis. Although the exact cause of mesenteric panniculitis remains unknown, the disease has been associated with a variety of other conditions, including neoplasms, other autoimmune diseases and abdominal trauma.
Clinical symptoms of mesenteric panniculitis are highly variable. Some individuals have few or no noticeable symptoms; others may be greatly affected by a variety of complaints including abdominal pain, nausea/vomiting, bloating, early satiety, loss of appetite and diarrhea or constipation. Systemic symptoms, especially fatigue, commonly occur in patients with mesenteric panniculitis. A computerized tomogram (CT) or other imaging of the abdomen shows thickening of the mesentery, sometimes with lymph node enlargement. Due to its variable clinical presentation and rarity, the diagnosis of mesenteric panniculitis is often delayed. Tissue biopsy is required to secure the diagnosis of mesenteric panniculitis and rule out neoplastic infiltration of the mesentery. However, not every patient suspected of having the disease will require a biopsy. There is limited information on the natural history of mesenteric panniculitis, but a stable clinical course is generally anticipated. Due to the rarity of mesenteric panniculitis, there is little prospective data available on its treatment. Nonetheless, corticosteroids and other medications directed at lowering the degree of inflammation and other medications that improve symptoms are felt to be the mainstay of treatment for mesenteric panniculitis.
Mesenteric panniculitis is a rare disorder that is part of a spectrum of diseases affecting the mesentery, a continuous organ that extends from the duodenojejunal flexure to the mesorectum that supports and attaches the intestines to the abdominal wall. Individuals with mesenteric panniculitis develop inflammation and necrosis of the fatty tissue of the mesentery, especially in the area of the small bowel. The condition progresses to cause chronic inflammation of the mesentery. In some patients, ongoing inflammation can result in scarring (fibrosis) of the mesentery.
Mesenteric panniculitis was first described in the medical literature in 1924 as “retractile mesenteritis”. Since that time, alternative names have been used to describe the condition including mesenteric panniculitis, retractile mesenteritis and mesenteric lipodystrophy. These names denote the predominant features of the disease process in the mesentery. Mesenteric panniculitis refers to the manifestation of the disease with a predominance of inflammation, sclerosing mesenteritis (or retractile mesenteritis) is the term that refers to a form of the disease with a predominance of fibrosis; and mesenteric lipodystrophy refers to disease with a histologic predominance of fat necrosis.
Emory et al. examined three entities (sclerosing mesenteritis, mesenteric panniculitis, and mesenteric lipodystrophy) and confirmed that there was a great deal of histologic overlap between the three entities and suggested that they are all manifestations of the same condition. The authors of this historic study also concluded that the most appropriate term for the process was “sclerosing mesenteritis” based on the presence of some degree of fibrosis. The authors also hypothesized that the condition might be a progressive process moving from lipodystrophy to mesenteric panniculitis to retractile mesenteritis. At present, mesenteric panniculitis is the most commonly used and recommended name for the condition. Overall, additional names that have been used for this disorder include mesenteric fibrosis, mesenteric sclerosis, liposclerotic mesenteritis, mesenteric Pfeifer-Weber-Christian disease, mesenteric lipogranuloma, xanthogranulomatous mesenteritis, inflammatory pseudotumor, retroperitoneal xanthogranuloma and isolated lipodystrophy.
Signs & Symptoms
In general, mesenteric panniculitis is a chronic, benign disorder with a favorable prognosis that occasionally resolves on its own (spontaneous regression). Nonetheless, symptoms of mesenteric panniculitis may be severe in some patients and can result in significant effects on quality of life. The clinical presentation of mesenteric panniculitis is highly variable. Some patients have few or no noticeable symptoms. The diagnosis of mesenteric panniculitis may be made incidentally following a CT scan of the abdomen, generally for the evaluation of abdominal pain. Symptoms of mesenteric panniculitis fall into two categories. Some symptoms, such as abdominal pain, are due to the mass-like effect of mesenteric inflammation, and potentially involvement of adjacent structures including the small intestine. The second group of symptoms occurs in the presence of chronic inflammation and may include weight loss, fever, and fatigue. Some affected individuals may develop complications such as small bowel obstruction or acute abdomen. Small bowel obstruction prevents the passage of food through the intestines and can cause a variety of nonspecific gastrointestinal symptoms as well as a nutrient malabsorption.
The most common symptom of mesenteric panniculitis is abdominal pain. The pain is generally located in the middle portion of the abdomen but can be present in other areas of the abdomen or pelvis as well. Other common symptoms include nausea, vomiting, early satiety, anorexia, fatigue, fever, unintended weight loss and altered bowel habits (either constipation or diarrhea). In some patients a tender mass may be detected in the middle portion of the abdomen mass. Abdominal distension from chylous ascites has also been described. A thorough examination to rule out peripheral lymphadenopathy or other signs of neoplasm is necessary in all patients.
There is little information available on the cause of mesenteric panniculitis. Many autoimmune diseases are believed to occur when patients with genetic predisposition to the diseases are exposed to an environmental factor that triggers an inappropriate immunologic response. This response ultimately leads to chronic inflammation. To this end, many conditions have been associated with and possibly predispose to the development of mesenteric panniculitis. These include surgery, acute pancreatitis, other autoimmune conditions and trauma.
Multiple papers have cited a relationship between mesenteric panniculitis and abdominal trauma or surgery. A review of the literature suggests a relatively low rate of association (4.76%).
Multiple case reports of mesenteric panniculitis have detailed the history of chronic infections including tuberculosis, histoplasmosis, Whipple’s disease, typhoid fever, cholera and syphilis that have possibly led to the development of sclerosing mesenteritis.
Evidence suggests that mesenteric panniculitis is an autoimmune disorder. Autoimmune diseases occur when the body’s natural defense mechanisms such as antibodies and lymphocytes, (that are normally in place for prevention of infectious diseases and cancer), instead cause a reaction and damage to the patient’s own healthy tissue. In general, genetic and environmental factors play a role in the development of autoimmune diseases. A number of factors support the hypothesis that mesenteric panniculitis is an autoimmune disease. These include the fact that biopsies of affected areas show chronic, ongoing inflammation, (although inflammation is not specific for autoimmune processes).
Additionally, systemic symptoms that are characteristic of other autoimmune diseases such as rheumatoid arthritis and Crohn’s disease including fever and fatigue can occur in patients with mesenteric panniculitis. Patients with mesenteric panniculitis also frequently often have a strong family history of autoimmune diseases. Finally, elevation of inflammatory markers that are measured in the blood, such as the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) are often found in patients with mesenteric panniculitis. Because mesenteric panniculitis occurs in some patients after certain medications, infections, abdominal surgery or trauma, other theories have been proposed to explain this disorder including post inflammatory reactions to acute inflammation or infection, or a deficient blood supply (ischemia) to the mesentery. However, these conditions probably develop secondary to an autoimmune reaction.
A significant number of patients with radiographically diagnosed “mesenteric panniculitis” have a variety of known or soon to be diagnosed cancers. A case control study from 2013 suggested that the appearance of “mesenteric panniculitis” often represents a paraneoplastic process. A significant number of patients having mesenteric abnormalities consistent with mesenteric panniculitis on CT scan will have an underlying form of cancer. The most common cancers with mesenteric panniculitis like abnormalities on CT scan are lymphomas. Other cancers associated with this finding include carcinoid tumor, colon, renal and prostate cancers. Mesenteric thickening and inflammation often appeared to represent a paraneoplastic syndrome that was not due to the physical presence of cancerous tissue in the affected area. In one study, few of the areas owed increased uptake in positron emission scanning and these abnormalities were generally stable in patients with cancer.
Other Fibrosing Conditions
There is also a known association between mesenteric panniculitis and other fibro-sclerotic disorders. This suggests that mesenteric panniculitis belongs to a larger spectrum of diseases in which inflammation and fibrosis affect multiple organ systems of the body. Fibro-sclerotic disorders that have been reported to occur with mesenteric panniculitis include retroperitoneal fibrosis, Sjögren’s syndrome and sclerosing pancreatitis.
The epidemiology of mesenteric panniculitis has not been fully defined. One study reported that findings consistent with mesenteric panniculitis occurred in 359 patients (0.24%) from a total of 147,794 abdominal computed tomography (CT) examinations undertaken for over a 5-year period in a large community based medical system. Of these, 100 patients (28%) had known malignancy or were later diagnosed with cancer. The incidence of mesenteric panniculitis from recent studies has shown a range from 0.16% – 3.4%. This range is dependent on the method of diagnosis and whether it is histologic versus radiologically diagnosed.
In some reports, mesenteric panniculitis has a male predominance of 2:1. Mesenteric panniculitis most often appears during the sixth and seventh decade of life, and its incidence appears to increase with age. Children and adolescents are rarely affected, possibly related to a lesser amount of fat in their mesentery, but more importantly because of specific characteristics of this form of autoimmunity.
Symptoms of mesenteric panniculitis mimic those of a number of other benign and malignant conditions. Because of this, it is important to rule out other diseases prior to confirming the diagnosis of mesenteric panniculitis. Infectious causes range from viral, bacterial (including V. cholerae) and parasitic infections. There are also a variety of diseases that are associated with mesenteric abnormalities on abdominal imaging. Such conditions include primary or metastatic mesentery cancer, gastrointestinal lymphoma, desmoid tumors, inflammation of the pancreas (pancreatitis), Crohn’s disease, idiopathic nodular panniculitis, locally advanced pancreatic adenocarcinoma, retroperitoneal fibrosis and other sclerotic diseases as mentioned above.
The diagnosis of mesenteric panniculitis is made based upon identification of suggestive symptoms, a detailed patient history, and a thorough clinical evaluation.
Clinical Testing and Work-Up
Affected individuals may have non-specific laboratory abnormalities such as reduced red blood cell counts (anemia). Laboratory markers of inflammation such as erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) can also be elevated.
These five diagnostic signs that have been felt to be specific for mesenteric panniculitis and separate it from other causes of abdominal masses include the presence of a well-defined “mass effect” on neighboring structures (sign 1) constituted by mesenteric fat tissue of inhomogeneous higher attenuation than adjacent retroperitoneal or meso-colonic fat (sign 2) and containing small soft tissue nodes (sign 3). It may typically be surrounded by a hypo-attenuated fatty “halo sign” (sign 4) and a hyperattenuating pseudo-capsule may also surround the entity (sign 5). However, these diagnostic criteria were not histologically verified, but they have overall come to represent the closest general standards available.
Radiographic studies such as computerized tomography (CT) scanning or magnetic resonance imaging (MRI) reveal characteristic findings in the abdominal or pelvic mesentery. Imaging reveals characteristic thickening, fat necrosis and calcification of the mesentery. Mild cases are referred to a “misty mesentery”. Enlargement and calcification of mesenteric and pelvic lymph nodes are commonly present. Because mesenteric panniculitis is not an invasive disorder, blood vessels within the mesentery appear to be spared from the inflammatory mass. This is referred to as the “halo sign” and is highly characteristic of mesenteric panniculitis as opposed to malignancy of the mesentery.
Surgical biopsy and microscopic study of affected tissue is required to completely rule out other conditions and to confirm a diagnosis of mesenteric panniculitis.
Most treatment recommendations are based on case reports or small case series. The only clinical prospective study of a treatment for mesenteric panniculitis has been performed using the drug thalidomide. The goals of treatment for mesenteric panniculitis are reduction of mesenteric inflammation and the control of symptoms of the disease.
Generally, individuals with no symptoms are not treated, but are regularly monitored to see whether the disorder progresses on abdominal imaging (watch and wait approach). A decision regarding biopsy is made during this time. In most patients, the disease remains asymptomatic. The mesenteric mass is generally stable or even regresses on its own.
For patients with symptoms related to chronic mesenteric inflammation, anti-inflammatory agents, especially corticosteroids are the initial treatment of choice. Additional anti-inflammatory drugs that have been used to treat mesenteric panniculitis include colchicine, azathioprine, cyclophosphamide, infliximab and pentoxifylline. A prospective clinical trial has demonstrated that the drug thalidomide improves symptoms and reduces blood levels of ESR and CRP in patients with mesenteric panniculitis. Low dose naltrexone (LDN) is also a promising new therapy for mesenteric panniculitis. LDN appears to work by modulating the immune system and by increasing blood levels of enkephalins and endorphins. Tamoxifen and other hormonal therapies have been proposed to treat patients with mesenteric fibrosis due to their anti-fibrotic effects. Unfortunately, serious side effects may occur with these medications including the development of thromboembolic phenomena and secondary malignancies. Because of the rarity of mesenteric panniculitis, few controlled clinical studies of medical therapies for this condition are likely to be performed in the future.
When individuals with mesenteric panniculitis develop small intestinal obstruction, surgery may be required. In general, surgery should be avoided in patients with mesenteric panniculitis and there should never be an attempt to surgically remove the mesenteric mass for the purpose of curing the disease.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov . All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.
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Ginsburg PM, Ehrenpreis ED. A pilot study of thalidomide for patients with symptomatic mesenteric panniculitis. Aliment Pharmacol Ther. 2002;16:2115-22. http://onlinelibrary.wiley.com/doi/10.1046/j.1365-2036.2002.01383.x/pdf
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