成人Still病诊断中的问题和鉴别诊断思路__培训课件.ppt

NSAIDs 不缓解：+糖皮质激素 强的松0.5-1mg/ kg /d 缓解：单用NSAIDs 缓解：NSAIDs+糖皮质激素 不缓解或激素减量复发：+ MTX 缓解：逐个减DMARDs下台阶策略 不缓解或重型AOSD MP冲击治疗、必要时生物制剂， MTX基础上联合DMARDs上台阶策略 1-3月 6-12个月 1-3月 缓解：单用MTX 6-12个月 不缓解：逐个加DMARDs上台阶策略 1-3月 6-12个月 *上台阶备选药物：来氟米特、硫唑嘌呤、环孢素A、环磷酰胺、羟氯喹、柳氮磺吡啶、霉酚酸酯等（一种或多种渐加用）。 中华医学会风湿病学分会，2010 顽固发热 重要脏器损害 严重血管炎 血沉极快 规律DMARDs联合治疗半年以上效差 重型成人斯蒂尔病 AOSD诊治指南.中华医学会风湿病学分会，2010 1）糖皮质激素冲击治疗 病情严重者需用大剂量激素，也可用MP冲击治疗。 2）生物制剂 难治、复发、重症和高度活动的AOSD的治疗新途径 抗TNF-α制剂 抗IL-1制剂 抗IL-6制剂等 治 疗 临床综合征：发热、皮疹、关节炎 无特异性 血清阴性 排他性诊断 激素治疗有效 成人斯蒂尔病 * * * * * * * * * * * * * * * * * * * * * * * Inherited inflammatory disorders are each associated with defects in specific gene products that are related to the functioning of the innate immune system. Most disorders present with episodes of inflammation – including fever, rash and elevation of acute phase proteins – but there are subtle differences between the different disorders.1–3 Features of CAPS include deafness, urticarial rash, conjunctivitis, visual problems and arthritis (see slide 6 for further details). Most patients have a mutation in the NLRP3 gene and inheritance is usually autosomal dominant (see slide 13 for further details).1 Familial Mediterranean fever (FMF) is the most common inherited periodic fever syndrome occurring in more than 100,000 people worldwide. It is most prevalent in Jewish, Arab, Armenian, Turkish and Italian populations. It is associated with mutations in the gene encoding pyrin, a protein believed to be involved in regulation of IL-1β production. The gene is located on chromosome 16p13.3, and inheritance is generally autosomal recessive. Attacks usually last 1–3 days, and distinguishing clinical features include serosal inflammation and amyloidosis. Most patients respond to treatment with colchicine.1–3 Tumour necrosis factor (TNF) receptor-associated periodic syndrome (TRAPS) is associated with mutations in the gene encoding the TNF receptor 1, located on chromosome 12p13 and show