作者: M. den Braber-Ymker , M. C. Vonk , K. Grünberg , M. Lammens , I. D. Nagtegaal
DOI: 10.1007/S10067-020-05325-8
关键词: Rheumatology 、 Immunohistochemistry 、 Medicine 、 Interstitial cell of Cajal 、 Autopsy 、 Enteric nervous system 、 Pathogenesis 、 Histology 、 Fibrosis 、 Pathology 、 Internal medicine
摘要: The pathogenesis of intestinal involvement in systemic sclerosis (SSc) is thought to be a sequential process (vascular, neuronal, and consecutive muscular impairment), but understanding the underlying histological changes how they translate symptoms, still lacking. Therefore, we systematically investigated characteristics SSc intestines, compared controls. Autopsy material from small bowel colon was used for semiquantitative evaluation vasculature, enteric nervous system, interstitial cells Cajal (ICC), muscle layers, using combination histochemical immunohistochemical stainings, according guidelines Gastro 2009 International Working Group. Vascular were most frequently encountered, represented by intima fibrosis both arteries vessels, venous dilatation. Second, generalized circular layer significantly more found patients than Third, reduction submucosal nerve fibers myenteric neurons shown four patients, which may explain severe symptoms dysmotility. density ICC network decreased patients. postulated processes could not supported our evaluation. interpatient diversity suggests that parallel occur, explaining variety features clinical symptoms.