Immunoglobulin G4 Unrelated Idiopathic Mesenteric Sclerosis / 대한소화기학회지

Sclerosing mesenteritis is a rare benign disease with a prevalence of 0.16–3.4% and is characterized by chronic nonspecific inflammation and extensive fibrosis in the adipose tissue of the mesentery although the exact pathogenesis is still elusive. A 65-year-old woman was referred with suspicion of an abdominal mass and biliary stones on abdominal ultrasonography and CT. Bile duct stones were confirmed by endoscopic ultrasonography and successfully treated by endoscopic retrograde cholangiography with stone removal. Furthermore, a 4.7 cm conglomerated mass on small intestinal mesentery was suspected as sclerosing mesenteritis based on the features on abdominal MRI. However, because it could not be differentiated from malignancy without histologic examination, laparoscopic excisional biopsy was performed; it showed only inflammatory cells with extensive fibrosis. Therefore, the abdominal mass was confirmed as sclerosing fibrosis and the patient was followed-up without any treatments because no mass-related symptoms accompanied the findings. Six months later, abdominal CT showed no significant change in the mass. Herein, we report a rare case of incidentally found idiopathic sclerosing mesenteritis.

Analysis of the cause of MSCT misdiagnosis in sclerosing mesenteritises / 实用放射学杂志

Objective To summarize the MSCT signs and the key points of differential diagnosis of sclerosing mesenteritises (SM) which were misdiagnosed by clinical and CT,to reduce the misdiagnosis rate.Methods Clinical and MSCT data of 23 misdiagnosed SM patients were analyzed retrospectively.The misdiagnosed diseases,misdiagnosed causes,and differential diagnosis were analyzed.Results SM were mainly misdiagnosed as tumor and infective inflammation.The main causes of misdiagnosis and differential diagnostic features were listed as follows.(1)SM didn't have specific clinical manifestations.(2)The density of the adipose tissue in mesentery increasedmisty mesentery.A clear demarcation between the lesion and the surrounding normal fatty tissue could be differentiated from infective inflammation.(3)The mass-like false capsule had space-occupying effect of displacement of the surrounding structures.However, the blood vessels were encapsulated by the mass-like false capsule with fat ring around, which could be differentiated from fat-containing tumors.(4)The soft mass was formed at the root of the mesentery.The fat halo sign and mild enhancement of the mass can be differentiated from lymphoma and carcinoid.Conclusion SM is easily misdiagnosed both in clinical practice and medical imaging.Recognition of differential diagnostic features of MSCT can reduce the misdiagnosis rate.

Paniculitis mesentérica asociada a enfermedad relacionada con IgG4 como causa de fiebre de origen desconocido / Mesenteric panniculitis associated with IgG4-related disease as a cause of fever of unknown origin

La paniculitis mesentérica es un raro desorden inflamatorio de la grasa mesentérica, de la cual hay, hasta ahora, aproximadamente 200 casos reportados en la literatura. Se presenta en adultos a partir de la tercera década de la vida y su etiología es desconocida, pero es sabida su asociación con neoplasias gastrointestinales, genitourinarias y enfermedades reumatológicas. Entre sus manifestaciones clínicas están el dolor abdominal, las alteraciones del tránsito intestinal, la pérdida de peso, la fiebre y los vómitos. El diagnóstico definitivo es fundamentalmente histopatológico, también existen algunos estigmas tomográficos que podrían sugerir su presencia. Debe ser tratada a la mayor brevedad posible y los corticosteroides son los medicamentos a elegir. Se presentó un paciente con diagnóstico de paniculitis mesentérica idiopática, como resultado del estudio de una fiebre de origen desconocido, en el cual logramos además demostrar la asociación de la paniculitis con la enfermedad relacionada con IgG4, desorden recientemente descubierto, caracterizado por lesiones inflamatorias seudotumorales, que cursan con infiltración hística por células plasmáticas IgG4 positivas.

Mesenteric panniculitis is a rare inflammatory disorder of the mesenteric fat, of which there is, so far, about 200 cases reported in the literature. It occurs in elderly adults and its etiology is unknown but its association with gastrointestinal tumors, genitourinary and rheumatological diseases is known. Among its clinical manifestations are abdominal pain, altered bowel movements, weight loss, fever and vomiting. The definitive histopathological diagnosis is fundamentally, there is some tomographic stigma that might suggest its presence. It should be treated as soon as possible and corticosteroids are the drugs of choice. A patient diagnosed with idiopathic mesenteric panniculitis as a result of the evaluation of fever of unknown origin, which we further demonstrate the association of panniculitis with related disease IgG4, disorder recently discovered, characterized by lesions in flammatory pseudotumoral occurs, that occur with tissue infiltration IgG4 positive plasma cells.

Sclerosing mesenteritis - rare cause of abdominal pain and intra-abdominal mass: a case report.

Sclerosing mesenteritis is a rare, benign, and chronic fibrosing inflammatory disease with unknown etiology that affects the mesentery of small bowel and colon. The disease has two well-established histological types: the acute or subacute form known as mesenteric panniculitis and the chronic form known as retractile or sclerosing mesenteritis. Because sclerosing mesenteritis lacks special clinical manifestations and typical signs, the patients are very easily misdiagnosed. The correct diagnosis of sclerosing mesenteritis depends on pathological examination after laparotomy. We report a case of sclerosing mesenteritis in a 55-year-old male who presented with chronic abdominal pain and intra-abdominal mass. He was misdiagnosed as lymphoma by Computed Tomography and then underwent exploratory laparotomy. Histopathological examination revealed it to be sclerosing mesenteritis. This patient went well and lives without recrudescence till date.

A Case of IgG4-Related Sclerosing Mesenteritis Associated with Crohn's Disease / 대한소화기학회지

Sclerosing mesenteritis (SM) is a rare disease characterized by chronic nonspecific mesenteric inflammation and fibrosis of unknown etiology. Some tumefactive SM shows diffuse accumulation of IgG4-positive plasma cells and is considered as a part of the spectrum of IgG4-related disease. An association between inflammatory bowel disease and IgG4-related disease has been indicated. A 45-year-old woman visited our hospital due to weight loss with intermittent lower abdominal discomfort. Pelvic ultrasound revealed a mass-like lesion in the abdominal wall and pelvis MRI demonstrated a 5.9 cm sized wall-enhancing mass with heterogeneous signal intensity from right adnexa to the abdominal wall. Tumor resection and adhesiolysis was done because of severe adhesion with the small bowel, colon, bladder, uterus, and abdominal wall. Appendectomy was also performed due to adhesion and edematous change. Histological examination of the resected mass showed findings that were compatible with IgG4-related SM. The resected appendix showed chronic granulomatous inflammation without evidence of tuberculosis. She was diagnosed with Crohn's disease after undergoing colonoscopy and CT enterography. Herein, we report a rare case of IgG4-related SM that occurred in conjunction with Crohn's disease.

A Case of Idiopathic Sclerosing Mesenteritis with Retroperitoneal Fibrosis / 대한소화기학회지

Sclerosing mesenteritis is a rare inflammatory disease of the bowel mesentery. It produces tumor-like masses of the mesentery composed of varying degrees of fibrosis, chronic inflammation, and fat necrosis. It has been described variously as fibrosing mesenteritis, retractile mesenteritis, mesenteric Weber Christian disease, and systemic nodular panniculitis. The etiology and pathogenesis of the disease are as yet unknown, but autoimmune disorder, previous abdominal surgery, trauma, and ischemia could play a role. The clinical features include abdominal pain, vomiting, diarrhea, and constipation. Occasionally, patients with this condition may present with bowel obstruction. Rarely, It can be associated with other idiopathic inflammatory disorders such as retroperitoneal fibrosis, sclerosing cholangitis, and orbital pseudotumors. We report a case of idiopathic sclerosing mesenteritis with retroperitoneal fibrosis in a 58-year-old man.

Paniculite mesentérica pseudotumoral: aspectos tomográficos de um caso / Pseudotumoral mesenteric panniculitis: tomographic findings in a case

A paniculite mesentérica representa um processo inflamatório do mesentério de ocorrência rara e etiologia desconhecida, que apenas em alguns poucos casos pode se manifestar sob a forma de pseudotumores abdominais. Descreve-se, enfatizando os aspectos tomográficos, um raro caso de paniculite mesentérica que se apresentou inicialmente como um pseudotumor que envolvia a região peripancreática.

Mesenteric panniculitis is a rare inflammatory process of the mesentery whose etiology is unknown that in only very few cases may present as an abdominal pseudotumor. The authors report a rare case, emphasizing the tomographic findings of mesenteric panniculitis that initially presented as a pseudotumor involving the peripancreatic region.

Four Cases of Postoperative Sclerosing Mesenteritis

Mesenteric panniculitis is an extremely rare inflammatory condition of the adipose tissue of unknown etiology. It is characterized by extensive, progressive fibrosis of the mesenteric fat tissue, leading to tumor formation. Clinical manifestations vary according to the process involving the structures. Abdominal pain accompanied by nausea, malaise, pyrexia, and weight loss and a poorly defined mass are common presentations. Major pathologic changes include (1) degeneration of mesenteric fat, (2) an inflammatory reaction, and (3) fibrosis of the adipose tissue. We present four patients with an aggressive form of mesenteric pannicultis with characteristic histopathologic features. We discuss the relationship between the histopathologic features and the surgical intervention in these cases.

Sclerosing Mesenteritis

Sclerosing mesenteritis is a rare condition; it's an idiopathic nonspecific inflammatory process in the adipose tissue of the small bowel mesentery. It often develops into huge masses that contain necrotic fat, and these masses mimick malignancy. It has two pathologically different variants: mesenteric panniculitis (acute or subacute) and retractile-mesenteritis (chronic). Although infection, trauma, local ischemia, surgery and malignancy have been implicated in the etiology of this disease, the exact causes are unknown. The disease usually has a favorable prognosis. The diagnosis is confirmed by biopsy. A 59 year-old man visited in our hospital with vague abdominal pain and diarrhea that he had suffered with during the previous three months. A computed tomography (CT) scan showed a dilated small intestine and also masses in the small bowel mesentery. On the operative findings, fibrous masses were noted in the mesentery. The pathologic report revealed sclerosing mesenteritis. We report here on a case of sclerosing mesenteritis along with a review of the literatures.

Cases of Postoperative Mesenteric Panniculitis

Mesenteric panniculitis is a rare inflammatory condition of mesenteric adipose tissue in which the mesentery is replaced with fibrosis. The frequent symptoms of mesenteric panniculitis are palpable mass, abdominal pain and gastrointestinal obstructive symptoms. In the majority of cases, its course is self-limiting and the prognosis is favorable. 3 cases of mesenteric panniculitis are described that presented with obstructive symptoms of gastrointestinal tract, which occurred in 2 weeks following colectomy of colonic tumors. And reviewed the symptomatology, pathology, treatment, and outcome of this disorder.