白塞病诊疗指南（The white - plug disease clinic guide）.doc

白塞病诊疗指南（The white - plug disease clinic guide） Whites disease 【 summary 】 Whites disease (Bechet s diseases, BD) is a chronic inflammatory disease vessels, main clinical manifestation for recurrent oral ulcers, genital ulcers, eye and skin damage, can also be affected blood vessels, nervous system, the digestive tract, joints, lungs and kidneys, epididymis and other organs, is a systemic disease. Most of the patients had good prognosis, and the prognosis of the eyes, central nervous and large vascular subjects was poor. EB virus, herpes simplex virus, streptococcus, tuberculosis, and immune genetic factors (hla-b51) may be associated with the disease. About half of the patients were resistant to oral mucosal antibody and circulating immune complex. Patients peripheral blood lymphocyte subsets imbalance, CD4 + / CD8 + ratio inversion, the lack of CD45RA + cells, lymphocytes autocrine TNF alpha, IL - 6, IL - 8 and IL increase soluble IL - 2-1 beta receptors, showed that the disease with autoimmune and cellular immune abnormalities. Unlike other vasculitis diseases, it is involved in all the large, medium and small blood vessels in the body, with the greatest number of veins. Histopathological changes are the infiltration of lymphocytes around the blood vessels, which have IgG, IgM and C3 deposits, and the large vein thrombosis, which is a vascular tumor formed by degeneration and necrosis. Vasculitis with effusion and hyperplasia of two kinds of pathological changes, exudative change for vascular cavity hemorrhage, wall edema, the swelling of the endothelial cells, fibrin deposition, etc., hyperplastic lesions is the endothelial cells and outer membrane cell hyperplasia, wall thickening, sometimes with granuloma formation. The disease has a high incidence in east Asia, the Middle East and the Mediterranean region, known as the silk road disease. There is no exact information about the incidence of disease in China, and the peak age of the disease is between 16 and 40