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

Abstract

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.

Lesiones ocupantes de espacio en pared abdominal (no herniaria). La visión del patólogo

“…Puede aparecer en cualquier región anatómica excepto las vísceras, aunque tiene predilección por las extremidades y el tronco [9][10][11][12][13][14] . La FN debe incluirse en el diagnóstico diferencial, especialmente en pacientes con una lesión en partes blandas de rápido crecimiento o con una historia reciente de traumatismo.…”

“…La FN se caracteriza histológicamente por una proliferación de fibroblastos y miofibroblastos asociados generalmente a un estroma mixoide. Las células no exhiben atipia citológica ni mitosis atípicas [9][10][11][12][13][14] .…”

Sclerosing Mesenteritis-Update on Diagnostic and Therapeutic Approach

“…The ischemic lesions are described correlated with the previous surgery, abdominal trauma, mesenteric thrombosis and mesenteric arteriopathy [3]. The association with infections proved to be more evident in patients with personal history of infection with typhoid, dysentery, tuberculosis, syphilis, malaria, influenza and rheumatic fever [3][4][5]16]. Initially a degenerative process of mesenteric fat, followed by inflammation (panniculitis).…”

Sclerosing mesenteritis: update on diagnostic and therapeutic approach

“…2,[5][6][7] Yet studies describe an intrinsic connection between the increase of serum IgG4 antibodies and the intestinal inflammatory process, suggesting a relationship between SM and IgG4-related diseases. 15 With regard to the paraneoplastic syndrome, the proportion of cases associated with underlying malignancy, varied in studies. Several neoplasms were reported, including lymphoma, breast cancer, melanoma, squamous carcinoma and pulmonary adenocarcinoma, renal carcinoma, multiple myeloma, chronic lymphocytic leukemia, mesothelioma thoracis, hepatocellular carcinoma, adenocarcinoma of the prostate, ovarian carcinoma, endometrial carcinoma, cervical carcinoma, angiosarcoma, gastrointestinal adenocarcinomas and carcinoid tumor.…”

“…As a result, the consumption of nicotine not only produces a sense of satisfaction that influences the neural reward system, but also has a wide variety of independent effects, such as an increased inflammatory response in the digestive tract. [13][14][15] The SM has been described in association with a number of conditions, including autoimmune thyroiditis, presumably of riedel, primary sclerosing cholangitis, retroperitoneal fibrosis, and orbital pseudotumor. In addition, can be related with paintings of autoimmune hemolytic anemia, minimum damage nephropathy, pulmonary fibrosis, lupus erythematosus and relapsing polychondritis.…”