Sclerosing Mesenteritis: A Concise Clinical Review for Clinicians.

Sclerosing mesenteritis (SM), an idiopathic nonneoplastic condition affecting 0.18% to 3.14% of the population, is characterized by chronic fat necrosis, inflammation, and fibrosis most commonly of the mesentery of the small intestine. Sclerosing mesenteritis typically presents in the fifth or sixth decade of life, where patients with a history of abdominal surgery and/or autoimmune disease may be at higher risk. While many patients are asymptomatic, clinical features and complications are related to the mass effect resulting from the inflammation and fibrosis involved in the pathogenesis of SM. When present, common signs, symptoms, and complications include abdominal pain, weight loss, diarrhea, palpable abdominal mass on examination, bowel obstruction, chylous ascites, and mesenteric vessel thrombosis. Although SM was historically diagnosed predominantly by biopsy, current practice has shifted away from this to computed tomography imaging of the abdomen, given the invasive nature of biopsy. However, certain conditions, including mesenteric neoplasia (lymphoma, metastatic carcinoid tumor, desmoid tumor, mesenteric carcinomatosis), can mimic SM on imaging, and if clinical suspicion is equivocal, a biopsy may be warranted for definitive diagnosis. Asymptomatic patients do not require treatment. For patients with pronounced symptoms or complicated SM, the combination of tamoxifen 10 mg twice daily and prednisone 40 mg daily is the first-line pharmacotherapy; no randomized controlled trial of this regimen has been performed. Rarely, surgery may be necessary in cases of persistent bowel obstruction refractory to medical management. Sclerosing mesenteritis has an overall benign course in most cases, but disease progression and fatal outcomes have been reported.

A review of 17 cases of mesenteric panniculitis in Zhengzhou Ninth People's Hospital in China.

PURPOSE: Mesenteric panniculitis (MP) represents the uncommon, benign and chronic inflammatory disorder affecting the mesenteric adipose tissues. Its etiology, diagnosis and treatment remain unnoticed. Our report focused on shedding more lights on this condition. PATIENTS AND METHODS: Seventeen MP patients were identified by searching the electronic medical record system in the Zhengzhou Ninth People's Hospital using the search terms "Mesenteric panniculitis" from October 2015 to March 2023. All cases were diagnosed with MP through computed tomography (CT). Their clinical features and treatments were analyzed. RESULTS: There were altogether 17 cases enrolled for this analysis. The male to female ratio was 8:9, and the median age at diagnosis was 64 (range: 37-96) years. There were 15 patients (88.2%) showing abdominal pain to varying degrees. The proportions of symptoms of nausea, vomiting and fever were 23.5%, 23.5% and 41.2%, respectively. Neoplastic disease was present in 3 patients (17.6%). Meanwhile, 9 patients (52.9%) had gallstones, 3 (17.6%) had cholecystitis and 1 (5.9%) had gallbladder polyps. Six patients (35.3%) received antibiotics treatment only and 1 (5.9%) received oral antibiotics and prednisone. One patient (5.9%) received antibiotics followed by prednisone treatment, because the symptoms were significantly relieved after antibiotic treatment, while the disease recurred soon after, and the symptoms improved again after prednisone treatment. The abdominal pain in 9 patients (52.9%) was relieved spontaneously. Two patients (11.8%) died, including one due to respiratory failure caused by pneumonia and the other one because of pancreatic cancer with lung and liver metastases. CONCLUSION: MP is a poorly understood chronic inflammatory disease. Patients often have abdominal pain as the main symptom, accompanied by comorbidities in the gallbladder, and the prognosis is usually good after correct diagnosis and treatment, Therefore, the present report aims to promote the awareness among clinicians of patients with non-classic abdominal symptoms, so as to avoid misdiagnosis or missed diagnosis.

Sclerosing mesenteritis due to Mycobacterium genavense infection: A case report.

RATIONALE: Since its first identification in the early 1990s, Mycobacterium genavense has been considered and opportunistic pathogen. It mainly causes gastrointestinal symptoms, but also disseminated infections in severely immunosuppressed patients. Sclerosing mesenteritis is a long-term complication with high morbidity and mortality. As it is a rare condition, there are no specific guidelines for its management. We report a challenging case of persistent M. genavense infection, and propose surgery as an alternative treatment strategy. PATIENT CONCERNS: A 38-year-old Caucasian man presented to the emergency room with fever, abdominal pain, and night sweats for 3 months. HIV screening revealed a previously unknown HIV-1 infection, with a CD4 cell count of 216 cell/µL and viral load of 361.000 copies/mL at diagnosis. A body CT-scan showed mild splenomegaly as well as mesenteric and retroperitoneal enlarged lymph nodes. Fine needle aspiration revealed the presence of acid-fast bacilli, but mycobacterial cultures were negative. In the second sample, 16S RNA sequencing yielded a diagnosis of M. genavense infection. Despite 2 years of corticosteroids and antimycobacterial treatment excluding rifampicin due to a severe cutaneous reaction, there was no clinical improvement and an increase in the mesenteric lymph node size was observed, with a sclerosing transformation of the mesentery. A surgical approach was proposed to release small bowel loops and to remove fibrin. A second surgery was required due to an acute peritonitis ought to yeyunal segmental isquemia and perforation. Finally, the patient evolved favorably, and antimycobacterial drugs were suspended without relapse. LESSONS: Despite a prolonged multidrug strategy, some patients develop persistent M. genavense infection. Once sclerosing mesenteritis is established, clinicians have few treatment options. Surgery should be considered in patients with sclerosing mesenteritis or bowel obstruction. The combination of medical and surgical treatment could be a potential cure for these patients.

Bilateral pleural effusions as the first sign of mesenteric panniculitis.

A 38-year-old woman presented to the emergency department with a history of thoracic pain and anorexia for 1 week. Thoracic CT scan showed bilateral pleural effusion, a thoracentesis was performed revealing a transudate liquid with polymorphonuclears and predominance of eosinophils. After admission, the patient developed abdominal pain and the abdominal CT scan showed densification of the mesenteric fat characteristic of mesenteric panniculitis (MP). The patient went through investigation for secondary causes of panniculitis including infection, neoplasia and autoimmune diseases, and no abnormalities were found. The patient was treated with corticosteroids over a period of 3 months with complete resolution and without any signs of remission or secondary cause of MP. Moreover, the patient remained asymptomatic for 2 years after being discharged, which strengthens the diagnosis of MP that presented with eosinophilic pleural effusion.

Sclerosing Mesenteritis.

Sclerosing mesenteritis is a rare non-neoplastic disorder characterized by fat necrosis, chronic inflammation, and fibrosis typically of the small bowel mesentery. Our understanding of this disorder is limited by its rarity as well as inconsistent terminology used across the literature. While prior abdominal surgery or trauma, autoimmunity, infection, ischemia, and malignancy have been suggested to be involved in the pathogenesis of the disorder, it remains poorly understood. The clinical course of sclerosing mesenteritis is generally benign with a large proportion of patients diagnosed incidentally on imaging obtained for other indications. In a subset of patients, symptoms may arise from a mass effect on the bowel, lymphatics, or vasculature resulting in bowel obstruction, chylous ascites, or mesenteric ischemia. Symptomatic patients should be treated with a combination of corticosteroid and tamoxifen as first-line therapy based on retrospective case series and experience in other fibrosing disorders. Surgical intervention may be required in those with persistent obstruction despite conservative treatment, though complete resection of the mass is often not feasible given intimate involvement with the mesenteric vasculature. A careful use of terminology and communication between the radiologist, pathologist, and clinicians in the care of these patients will be essential to future efforts at understanding this disease.

Pediatric mesenteric panniculitis: three cases and a review of the literature.

Açari C, Ünsal E, Hakgüder G, Soylu A, Özer E. Pediatric mesenteric panniculitis: three cases and a review of the literature. Turk J Pediatr 2019; 61: 798-803. Mesenteric panniculitis is an inflammatory and fibrotic process in the mesenteric adipose tissue with unknown etiology. It is rarely seen in general, particularly in children. Etiology is unknown, and pathophysiology is not clear. Factors that trigger the disease are malignancy, tuberculosis, trauma, medications and past surgical interventions. There is no pediatric case series in the literature except single case reports. This paper consists of 3 cases: The first case is a 5-month-old girl, the youngest patient in the literature, who was referred to a pediatric surgeon with vomiting and abdominal distention. She had diffused intraabdominal fluid and mesenteric panniculitis documented by perioperative biopsy. The second case had acute abdominal pain with perforated appendicitis, who eventually had mesenteric panniculitis in the evaluation of the pathological specimen. The last case had a diagnosis of polyarticular juvenile idiopathic arthritis (JIA), successfully treated with etanercept, and has been in remission for 2 years. Interestingly, in one of her routine visits, she had pallor, anemia and renal failure. Bilateral hydronephrosis was detected. Magnetic resonance imaging (MRI) of the abdomen revealed retroperitoneal fibrosis, and mesenteric panniculitis was the histopathological diagnosis.

Clinical and radiological features of mesenteric panniculitis: a critical overview.

The mesenteric panniculitis is a rare form of inflammation that mainly involves the mesenteric adipose tissue. The etiology remains unknown and the disease has been associated with various conditions such as cancer, abdominal trauma, previous surgery, autoimmune diseases and obesity. Mesenteric panniculitis can be divided into two main groups: the mesenteric panniculitis with only the inflammation and degeneration of the mesenteric fat, and the retractile panniculitis, mainly fibrotic, with retraction of the surrounding structures. From a radiological point of view, there are two main signs: the fat ring sign, which is the presence of normal fat around vessels and lymph nodes, and the pseudocapsula around the lesion. In this paper, we present the imaging and clinical features of mesenteric panniculits with particular reference to the differential diagnosis and the possible etiological associations. (www.actabiomedica.it).

Sclerosing mesenteritis: a systematic review of 192 cases.

INTRODUCTION: Sclerosing mesenteritis includes a spectrum of inflammatory disorders involving the adipose tissue of the bowel mesentery. AIM: To perform a systematic review of previously reported cases of sclerosing mesenteritis (SM) to determine the epidemiology, risk factors, methods of diagnosis, treatment patterns and outcomes for this disease. METHODS: Medline, PubMed, Google Scholar and Cochrane database were searched using keywords mesenteric panniculitis, retractile mesenteritis, mesenteric lipodystrophy and sclerosing mesenteritis. Data was collated into a single excel database, transferred into SPSS (Version 21.0) and analyzed. RESULTS: Patients diagnosed with SM were between ages of 3 and 88 with a mean age of 55 ± 19.2 years. SM was more common in Caucasians (n = 28, 60.8% of those reporting ethnicity) and men (n = 133, 69.3%) with a male to female ratio of 2.3:1. 28.6% (n = 55) of patients reported a prior abdominal surgery or abdominal trauma, 8.9% (n = 17) had a history of malignancy, and 5.7% (n = 11) of autoimmune disease. 85.4% (n = 164) underwent surgical abdominal exploration (open or laparoscopic); 41.7% (n = 80) had surgery with resection of the involved bowel and mesentery. 34.9% (n = 67) of patients received medical treatment with the majority of them receiving steroids (n = 56, 83.5%). Symptom duration of more than a month (66.7% vs 40.4%, p < 0.05), underlying autoimmune disorder (14.3% vs 4.0%, p < 0.05) or low protein (14.3% vs 4.0%, p < 0.05) at presentation were seen more frequently in those with poor treatment response whereas patients with tender abdomen (45.0% vs 19.0%, p < 0.05) or leukocytosis (20.5% vs 0.0%, p < 0.05) at presentation were likely to have good response to therapy. The most common complications included bowel obstruction/ileus/ischemia (n = 10, 23.8%) and obstructive uropathy/renal failure (n = 10, 23.8%). There were a total of 14 deaths, 12 (85.7%) of which were secondary to SM related complications. CONCLUSION: SM is a poorly understood chronic inflammatory disease. Our study is the first systematic review of the published cases of SM. Future work is required to better understand this disease and its optimal therapy.

Sclerosing mesenteric panniculitis in a young patient : common cause of diagnostic dilemma and treatment refractoriness.

BACKGROUND: Sclerosing mesenteric panniculitis (SMP) is an idiopathic chronic fibroinflammatory disorder of the intra-abdominal fat. CASE PRESENTATION: Herin, we report a case of SMP, involving the omentum, mesentery and peri-colic fat in a 18 year old male, who presented with significant and recurrent abdominal distension for 4.5 years. Computed tomogram revealed ascites, with nodular and irregular omental thickening and foci of calcification. Non-specific radiological and histological features made an accurate diagnosis extremely difficult. After a thorough work up and exclusion of other differentials, diagnosis of a nodular SMP (Weber Christian disease) was given. After showing resistance to chemotherapeutic agents, slow response was noted with cyclophosphamide, followed by rapid symptomatic improvement with mesenterectomy. CONCLUSION: SMP is an uncommon benign mesenteric/ omental inflammation, and is a diagnosis of exclusion. As treatment refractoriness is common, management should be individualized and continued for along period. Surgical omentectomy may be helpful.

[Mesenteric panniculitis]. / Paniculitis mesentérica.

Mesenteric panniculitis is a condition characterized by chronic nonspecific inflammation of the mesentery. There is little and often confusing information about its characteristics and the approach to take once it has been demonstrated by an imaging test. We propose to describe the epidemiological, clinical, laboratory, radiological and pathological features of the patients with mesenteric panniculitis reported in the literature, as well as possible disorders causal or associated with mesenteric panniculitis, in the opinion of the authors of each study. Finally, we will review the different therapeutic options used and the response to them. To that end a literature search was performed from the main medical databases selecting ítems with information on these aspects. This information was collected on a database stored in SPSS software for further analysis and summary.

Mesenteric panniculitis: an update.

In 1924, mesenteric panniculitis was first described in the medical literature by Jura et al. as 'retractile mesenteritis.' It represents a spectrum of disease processes characterized by degeneration, inflammation and scarring of the adipose tissue of the mesentery. The clinical presentations vary according to the stage of the disease and they include abdominal pain, weight loss, nausea and vomiting. Computed tomography findings are usually diagnostic. The gross findings include thickening of the mesentery, mass lesions and adhesion to the surrounding organs. Histologically, there is a chronic inflammatory process involving the adipose tissue with fat necrosis, inflammation and fibrosis. Herein, the authors address the clinicopathological features, course, treatment and pathogenetic mechanisms of mesenteric panniculitis.

Sclerosing mesenteritis: A rare case of large bowel and rectum involvement.

Sclerosing mesenteritis (SM) is an uncommon non-neoplastic inflammatory process in the mesentery that is seen as a pseudotumour, usually involving the small-bowel mesentery and, less commonly, the mesentery of the large bowel. The disease has two well-established histological types: the acute or sub-acute form known as mesenteric panniculitis and the chronic form known as retractile or SM. Because SM lacks special clinical manifestation and typical signs, the possibility of misdiagnosis is very high. The correct diagnosis of SM depends on pathological examination. Here in, we report a case of a 41-year-old male patient with SM of the large bowel.

[Mesenteric panniculitis]. / Panniculite mésentérique.

Mesenteric panniculitis is a nonspecific inflammatory process affecting the fatty tissue at the root of the mesentery. This term is also used to describe the clinical and imaging findings in this disorder. Mesenteric panniculitis can be a misleading term: it is commonly misused to design an increased density of the mesentery without prejudice regarding the etiology. Pain is the main clinical symptom. Half of the patients are asymptomatic. There is a palpable mass in half of cases. Laboratory tests sometimes reveal an acute phase reaction of varying intensity. Mesenteric panniculitis is suspected when CT scan shows increased density of the mesenteric fat. Nevertheless, only histological examination could establish the diagnosis. Histologic examination may reveal various stages: lipodystrophy (the first stage when fat necrosis is predominant), mesenteric panniculitis (a majority of infiltrating lymphocytes), sclerosing mesenteritis (the end stage when fibrosis is predominant). Histopathologic differential diagnoses are lymphomas, lipomas, liposarcomas that can mimic mesenteric panniculitis on CT scan. Mesenteric panniculitis is associated with various diseases, especially with intra-abdominal inflammatory process. It also can be idiopathic. Rare complications can occur with vascular or digestive tract compressions. Empirical treatment is only useful in symptomatic patients. Colchicine, corticosteroids or immunosuppressive agents can be used. The only interest of surgery is the histological confirmation of the diagnosis. A better understanding of the pathophysiology of the immunoregulatory functions of adipose tissue will improve mesenteric panniculitis management.

IgG4-related sclerosing mesenteritis: a rare mesenteric disease of unknown etiology.

Sclerosing mesenteritis is a rare inflammatory and fibrosing disorder of unknown etiology, while IgG4-related disease (IgG4-RD) consists of mass-forming, fibroinflammatory lesions characterized by high serum IgG4 levels and tissue infiltration of many IgG4-positive plasma cells; obliterative phlebitis is common. This report describes a case of sclerosing mesenteritis that was considered a manifestation of IgG4-RD. A 53-year-old man underwent right hemicolectomy because of an ileocecal mass that did not improve with conservative therapy. The ill-defined fibroinflammatory lesion extended in the mesentery with storiform fibrosis, obliterative phlebitis, and infiltration of many IgG4-positive plasma cells. The ratio of IgG4-positive/IgG-positive cells was 64%, and the ratio of forkhead box protein 3 (FOXP3)-positive/CD4-positive cells was elevated (13%). It is likely that at least some cases of sclerosing mesenteritis are a manifestation of IgG4-RD. It is important to investigate this relationship because steroid therapy may benefit such cases.

Sclerosing mesenteritis presenting with complete small bowel obstruction, abdominal mass and hydronephrosis.

Sclerosing mesenteritis is an uncommon and poorly understood inflammatory condition of the bowel mesentery which can often be confused with neoplasia, Crohn's disease and other inflammatory conditions. We describe a case of complete small bowel obstruction and right sided hydronephrosis due to sclerosing mesenteritis.

Cutaneous sclerosis: a previously undescribed manifestation of sclerosing mesenteritis.

BACKGROUND: Sclerosing mesenteritis is a rare disease of unknown etiology that is characterized by self-limited, nonspecific inflammation and fibrosis of the mesenteric adipose tissue. Histologic classification characterizes 3 main stages in the evolution of the fibroinflammatory process: mesenteric lipodystrophy (ML), mesenteric panniculitis (MP), and sclerosing (retractile) mesenteritis (SM). OBSERVATIONS: A 68-year-old woman with biopsy-proven MP presented with multiple asymptomatic, indurated subcutaneous nodules on both arms, as well as 2 indurated plaques on her abdomen. The cutaneous changes preceded the diagnosis of SM by roughly 3 years. The arm lesions were centrally depressed with a prominent groove and a peau d'orange appearance. Biopsy findings revealed a subcutaneous process with almost total replacement of adipocytes by zones of woody sclerosis and fat necrosis identical to that observed in the mesentery. To our knowledge, this manifestation of sclerosing mesenteritis has not been reported previously. CONCLUSIONS: Sclerosing mesenteritis has rarely been associated with extra-abdominal idiopathic fibrosclerotic disorders, but a cutaneous component of SM has never been characterized. The fact that the cutaneous lesions were histopathologically identical to the mesenteric changes and their presence prior to the recognition of intra-abdominal disease suggests that future patients with such lesions might be evaluated for this disorder leading to earlier recognition.

Transudative chylothorax associated with sclerosing mesenteritis.

Transudative chylothorax is an uncommon type of chylous pleural effusion, typically secondary to chyle leak and a coexisting disorder such as heart failure or liver cirrhosis. Sclerosing mesenteritis is a rare inflammatory disease of the small bowel mesentery, and has once previously been reported as a cause of chylothorax. We present the case of an 81-year-old man with a right-side transudative chylothorax associated with congestive heart failure and sclerosing mesenteritis. We discuss potential mechanisms.

Sclerosing mesenteritis involving the pancreas: a mimicker of pancreatic cancer.

Sclerosing mesenteritis (SM), also known as mesenteric lipodystrophy, rarely involves the parenchyma of the pancreas. When SM does involve the pancreas, it can mimic pancreatic carcinoma both clinically and radiographically with pain, obstructive jaundice, a mass lesion, and even the appearance of vascular invasion. We report 6 patients with SM involving the pancreas (mean age 43.2 y, 5 female), and review their clinical presentation, radiographic findings, pathology, and outcome. Five of these 6 patients were originally thought to have a primary pancreatic neoplasm. Initial presenting clinical information was available for each patient: all 6 reported abdominal or epigastric pain, 3 reported weight loss, and 2 reported one or more of the following: back pain, fever, abdominal bloating/distention, nausea with/without vomiting, and anorexia. The lesions formed masses with an infiltrative pattern and all had 3 key histologic features: fibrosis, chronic inflammation, and fat necrosis-without a known etiology. The inflammatory infiltrate was composed of a mixture of lymphocytes, plasma cells, and scattered eosinophils. Of the 5 patients with post-treatment clinical information available, 4 had at least a partial response to treatment with steroids, tamoxifen, azathioprine, resection, or a combination of these, and 1 did not respond. A dramatic response to immunosuppressive therapy is illustrated by the case of a 46-year-old woman who presented with the presumptive diagnosis of an unresectable pancreatic cancer. Distinguishing SM from pancreatic carcinoma is crucial to appropriate management, as patients with SM may benefit from immunosuppressive therapy.