白塞病诊疗指南(国外英文资料).docVIP

白塞病诊疗指南 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 patients have a good prognosis, and the prognosis of the eyes, the central nerve and the large blood vessels is poor. Epstein, herpes simplex virus, streptococcus, tuberculous infection, 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 angioinflammatory diseases, it is involved in all the large, medium and small blood vessels in the body, with the highest number of veins. Histopathological changes is lymphatic mononuclear cells infiltration around a blood vessel walls can have IgG, IgM and C3 deposition, vena cava thrombosis, artery caused by degeneration, necrosis and form of hemangioma. 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 is more prevalent in east Asia, the Middle East and the Mediterranean. It is known as the silk road disease. The incidence of disease in our country is not exact, any age can be ill, the peak age of the disease is 16 ~ 40 years old. Our country is mostly female,