Tsuchiya M, Hibi T, Mizuno Y, Ono A, Morita A
Gastroenterol Jpn. 1976;11(2):88-99. doi: 10.1007/BF02776704.
Lymphangiectasia of the small intestine was demonstrated in 9 to 18 cases with protein losing gastroenteropathy and in 10 of 26 cases with Behçhet's disease. Protein losing gastroenteropathy was compared to Behçet's disease in view of immunological aspects. Immunoglobulin containing cells in the jejunal mucosa of protein losing gastroenteropathy were decreased, whereas Behçet's disease had normal or increased distribution. This suggested that suggested that immunoglobulin may be lost into the intestinal lumen or that production of those cells may be inhibited in protein losing gastroenteropathy. Decreased DNCB skin reaction and impaired blastoid transformation of peripheral lymphocytes in both of two diseases suggested that there should be immunological defect in those diseases. However, in Behçet's disease, investigation of the thymus disclosed hyperplasia and the presence of lymphoid follicle which does not appear in normal state. In conclusion, protein losing gastroenteropathy is in immunological deficiency state due to the congenital or acquired disorders of lymphatics which implies abnormal protein loss, decreased immunoglobulin containing cells in the jejunal mucosa and decreased sensitivity. On the contrary, in Behçet's disease hyperimmune state is present based on thymic hyperplasia.
在9至18例蛋白丢失性胃肠病患者以及26例白塞病患者中的10例中发现了小肠淋巴管扩张症。从免疫学角度对蛋白丢失性胃肠病和白塞病进行了比较。蛋白丢失性胃肠病患者空肠黏膜中含免疫球蛋白的细胞减少，而白塞病患者的分布正常或增加。这表明免疫球蛋白可能丢失到肠腔中，或者在蛋白丢失性胃肠病中这些细胞的产生可能受到抑制。两种疾病的二硝基氯苯皮肤反应降低和外周淋巴细胞母细胞转化受损表明这些疾病存在免疫缺陷。然而，在白塞病中，胸腺检查显示增生以及出现正常状态下不存在的淋巴滤泡。总之，蛋白丢失性胃肠病处于免疫缺陷状态，这是由于淋巴管的先天性或后天性疾病导致蛋白质异常丢失、空肠黏膜中含免疫球蛋白的细胞减少以及敏感性降低。相反，白塞病基于胸腺增生而处于高免疫状态。
