There is no recognized effective cure for this disease. A variety of medications may be effective, but they are prone to relapse when discontinued. The goal of treatment is to control existing symptoms, prevent damage to vital organs, and slow disease progression. Different treatment programs are adopted according to different clinical manifestations. General treatment During the acute active period, bed rest should be given. During the interictal period, attention should be paid to the prevention of recurrence. Such as controlling mouth and throat infections, avoid eating irritating food. The accompanying infected person can be treated accordingly. Hebei Provincial People’s Hospital Rheumatology and Immunology Zhang Fengxiao 2, local treatment Oral ulcers can be localized with glucocorticoid cream, ice boron powder, tin-like powder, etc., genital ulcers with 1:5000 potassium permanganate wash with antibiotic ointment; ocular damage to ophthalmologists need to assist in the treatment of eye conjunctivitis, keratitis can be applied to glucocorticoid eye ointment or eye drops, ocular pigmentary membranitis must be applied to the pupil dilating agent in order to prevent inflammation of the postadhesive, severe Glucocorticoids can be injected under the bulbar conjunctiva in severe cases of uveitis. (1) Non-steroidal anti-inflammatory drugs (NSAIDs) have anti-inflammatory and analgesic effects. They are effective in relieving fever, skin nodules and erythema, genital ulcer pain and arthritis symptoms. A variety of NSAIDs are available (see Rheumatoid Arthritis Treatment). (2) Colchicine can inhibit neutrophil chemotaxis, and has a certain therapeutic effect on arthropathy, erythema nodosum, oral and genital ulcers, and uveitis, with a common dose of 0.5 mg 2-3 times daily. Adverse effects such as hepatic and renal damage and granulocytopenia should be noted. (3) Thalidomide is used for the treatment of oral and genital ulcers and skin lesions. 25~50mg/dose, 3 times daily. Contraindicated in pregnant women, may cause fetal malformation (see the standardized treatment of ankylosing spondylitis), in addition to the side effect of causing neuraxial degeneration (4) Dapsone (Dapsone) has bacteriostatic and immunosuppressive effects, inhibit neutrophil chemotaxis. It is used in the treatment of oral and genital ulcers, pseudofolliculitis, and erythema nodosum. The common dose is 100mg/d. Side effects include decreased hemoglobin, liver damage, gastrointestinal reactions. (5) Glucocorticosteroids are used according to the severity of organ involvement and disease, and sudden stopping of the drug is likely to lead to recurrence of the disease. Serious patients, such as severe ophthalmia, central nervous system lesions, severe vasculitis patients can use intravenous application of high-dose methylprednisolone shock, 1000mg / d, 3-5 days for a course of treatment, and immunosuppressant combined effect is better. Long-term application of glucocorticoids has adverse effects (see Treatment of systemic lupus erythematosus). (6) Immunosuppressants These drugs should be used when there is damage to vital organs. They are often used in combination with glucocorticoids. The side effects of these drugs are significant and should be closely monitored during administration. 1) Azathioprine (AZA) Dosage is 2~2.5mg/(kg-d) orally. It is the main drug for BD with multi-systemic lesions. May improve prognosis by suppressing mouth ulcers, eye lesions, arthritis, and deep vein thrombosis. Relapses occur easily after stopping the drug. It can be combined with other immunosuppressants, but should not be combined with interferon-α to avoid bone marrow suppression. During the application period, blood routine and liver function should be checked regularly. 2) Methotrexate (MTX) 7.5~15mg per week, orally or by injection. It is used for the treatment of neurological, skin and mucous membrane lesions, and can be taken in small doses for a long time. Adverse effects include bone marrow suppression, liver damage and gastrointestinal symptoms. 3) Cyclophosphamide (CYC) is used in combination with prednisone in acute central nervous system damage or pulmonary vasculitis or ophthalmitis, and can be administered orally or in large doses intravenously (0.5~1.0/m2 of body surface area per dose, once every 3~4 weeks, or 0.6/dose, once every 2 weeks). Patients should be advised to drink a lot of water to avoid the occurrence of hemorrhagic cystitis, in addition, there may be gastrointestinal reactions and leukopenia, etc. (see the treatment of systemic lupus erythematosus). 4) Cyclosporine A (CsA) is effective in ocular leukoaraiosis where colchicine or other immunosuppressive agents are ineffective. The dose is 3-5 mg/(kg-d). Because of its neurotoxicity, it may lead to central nervous system lesions, and is generally not used in patients with BD combined with central nervous system damage. Blood pressure should be monitored carefully, and renal impairment is the main side effect. 5) Sulfasalazine (SSZ) 3～4g/d, 3～4 times orally. It can be used in patients with intestinal leukemia or arthritis, and the adverse effects of the drug should be noted. 6) Nitrogen mustard phenylbutyrate (chlorambucil, CB1348) Due to the large side effects, the current application is less. It can be used to treat retinal, central nervous system and vascular lesions. It is given as 2mg three times a day. Continue for several months until stabilization and then reduce the dose for maintenance. Eye damage should be considered for more than 2-3 years to avoid recurrence. Side effects include secondary infections, menopause or spermatogenesis or azoospermia in long-term use. (7) Biological agents 1) Interferon-alpha-2a (IFN-alpha-2a) IFN-alpha-2a has a high efficacy rate in joint damage and skin and mucous membrane lesions, and has been reported to be effective in the treatment of patients with refractory uveitis and retinal vasculitis. The initial treatment is 6 million units of IFN-α-2a subcutaneous injection per day, and after effective treatment, the dosage is gradually reduced, and the maintenance dosage is 3 million units three times a week, and some patients can stop the drug. Side effects include depression and hematopoiesis, avoid combining with AZA. 2) TNF-α antagonists Infliximab, etanercept and adalimumab have been reported to be effective in the treatment of leukemia. It can be used for DMARDs-resistant leukemia patients with skin and mucosal lesions, uveitis and retinitis, arthritis, gastrointestinal injuries, and central nervous system involvement, etc. TNF-alpha antagonists have a rapid onset of action but are prone to relapse after discontinuation of the drug, and reapplication in patients with relapses is still effective. Attention should be paid to the prevention of infection, especially tuberculosis infection. (8) Others ① Ranunculus preparations can be used in the treatment of oral ulcers, subcutaneous nodules, arthropathy, and ophthalmia. It is less effective for intestinal symptoms. ② Antiplatelet drugs (aspirin, Pansentin) and antifibrin therapies (urokinase, streptokinase) have no direct evidence of use in the treatment of thrombotic disorders in BD, and they should be used with caution to avoid rupture and bleeding of hemangiomas. Recently formed thrombi with a clear diagnosis can be thrombolyzed and anticoagulated. Thrombolysis can be applied intravenously with streptokinase and urokinase; anticoagulation can be used with subcutaneous injection of low molecular heparin or oral administration of Warfarin 2～8mg/d (need to monitor the prothrombinogen time and maintain the INR at 2～2.5). Thrombolytic anticoagulation is forbidden for patients with bleeding tendency, stroke, surgery, uncontrolled hypertension, hepatic and renal dysfunction, and retinal hemorrhagic lesions. ③ If the patient has tuberculosis or a history of tuberculosis, PPD skin test is strongly positive (5IU with blisters), try anti-tuberculosis treatment (triple) for at least three months, and observe the efficacy. Surgery is generally not advocated for surgical treatment, and surgical treatment can be considered for aneurysms with the risk of rupture. Patients with chronic stage should first use glucocorticoid combined with CYC treatment. When severe intestinal leukoaraiosis is complicated by intestinal perforation, emergency surgical treatment is feasible, but the recurrence rate after surgery can be as high as 50%, so the choice of surgical treatment should be careful. After surgery for vascular lesions, aneurysms may also form again at the postoperative anastomosis, and the use of interventional therapy can reduce surgical complications. The use of interventional therapy can reduce the complications of surgery. The use of immunosuppressive drugs should be continued after surgery to reduce recurrence. Ocular blindness with persistent pain can be removed surgically. 5. Reference treatment plan for BD major organ involvement: (1) Ophthalmology Systemic glucocorticosteroids and early application of AZA are required for the treatment of any BD inflammatory ophthalmopathy. glucocorticosteroids, AZA combined with cyclosporine A or biologics are recommended for severe ophthalmopathy with ≥ grade 2 loss of visual acuity and/or retinopathy. Glucocorticoids should be used to prevent secondary cataracts and glaucoma. (2) Macrovascular lesions There is insufficient evidence from controlled studies to guide the treatment of macrovascular lesions in BD. Acute DVT is recommended to be treated with glucocorticoids in combination with immunosuppressive agents such as AZA, CYC, CsA. peripheral aneurysms are at risk of rupture and can be treated with surgery in combination with immunosuppressive agents. Pulmonary aneurysm surgery has a high mortality rate, mainly treated with immunosuppressants, and aneurysm embolization can be tried in emergencies. (3) Gastrointestinal tract lesions Except for emergencies requiring surgery, glucocorticoids, SSZ, AZA should be used as the first step, and TNF-α antagonists or thalidomide can be used in refractory cases. If necessary, partial ileocolonic resection should be performed, but the postoperative recurrence rate and secondary surgery rate are high, and AZA can be used for postoperative maintenance therapy to reduce the rate of secondary surgery. (4) Neurologic lesions Brain parenchymal damage can be treated with glucocorticoids, MTX, AZA, CYC, IFN-α and TNF-α antagonists. In the acute stage, high-dose glucocorticoid shocks (commonly used intravenous methylprednisolone 1000mg/d 3-7 shocks) are required, followed by oral glucocorticoid maintenance therapy for 2-3 months. Combined use of immunosuppressive drugs can prevent recurrence and slow down the progression of the disease. (5) Mucocutaneous lesions may be treated with specialized local therapy. AZA, thalidomide, and biologics may be used for refractory mucocutaneous lesions.