风湿病与肾_精品文档.ppt

1、风湿病几种a风湿性疾病的分类 根据ACR标准1、弥漫性结缔组织病（类风湿性关节炎，系统性红斑狼疮等）2、与脊柱炎相关的关节炎（强直性脊柱炎，反应性关节炎等）3、骨性关节炎4、感染所致风湿性综合症5、伴有风湿性疾病的代谢性或内分泌性疾病（痛风等）6、与肿瘤相关的风湿病7、与神经血管疾病相关的风湿病（血管炎等）8、骨及软骨疾病（骨质疏松症等）9、关节外疾病10、其他有关节表现的疾病 免费热线：800-830-5160 会员热线：400-700-5160 从这里我们可以看出，风湿病是一个大的概念。风湿免疫病-多系统受累3 T,B cells,Macrophage TNFa a,IL1,IFNg g,

2、IC,C3 血管炎是多系统受累的核心ACR/EULAR criteria for the classification of SSclThese criteria are applicable to any patient considered for inclusion in an SSc study.The criteria are not applicable to patients with skin thickening sparing the fingers or to patients who have a scleroderma-like disorder that bette

3、r explains their manifestations.lPatients with a total score of 9 are classified as having definite SSc.ItemSub-item(s)WeightSkin thickening of the fingers of both hands extending proximal to the MCP joints(sufficient criterion)9Skin thickening of the fingers(only count the higher score)Puffy finger

4、sSclerodactyly of the fingers(distal to MCP joints but proximal to PIP joints)24Fingertip lesions(only count the higher score)Digital tip ulcersFingertip pitting ulcers23Telangiectasia-2Abnormal nailfold capillaries-2Lung involvement(maximum score is 2)Pulmonary arterial hypertensionInterstitial lun

5、g disease22Raynauds phenomenon-3SSc-related autoantibodies(maximum score is 3)AnticentromereAnti-topoisomerase IAnti-RNA polymerase III3Van den Hoogan F et al.Arthritis Rheum 2013;65:2737-47Major criteria -Raynauds phenomenon -Antibodies (ANA,anticentromere,anti-topo I)-Diagnostic nailfold videocapi

6、llaroscopyAdditional criteria -Calcinosis -Puffy fingers -Digital ulcers -Dysfunction of the oesophageal sphincter -Telangiectasia -Ground glass at chest HRCTDiagnosis is made by satisfaction of:all 3 major criteriaor 2 major plus one of additional criteriaMatucci-Cerinic M et al.Ann Rheum Dis 2009;

7、68:1377-80Provisional criteria for diagnosis of very early SSc EULAR Scleroderma Trial and Research group(EUSTAR)Preliminary criteria for the diagnosis of very early diagnosis of SScKoenig M et al.Arthritis Rheum 2008;58:3902-12Sensitivity 47%Specificity 98%ACAanti-topo Ianti-Th/Toanti-RNAPIII肾脏肾脏轻度

8、或间歇性蛋白尿轻度或间歇性蛋白尿少有红细胞或白细胞少有红细胞或白细胞70的蛋白尿患者的蛋白尿患者高血压高血压肾功能衰竭肾功能衰竭SSc“肾危象肾危象”：Sclerosis Crisis肾损害发展急剧肾损害发展急剧突然出现急进性高血压突然出现急进性高血压演变为肾功能衰竭演变为肾功能衰竭SSc重要的死亡原因之一重要的死亡原因之一SRC的临床表现的临床表现急骤进展的重度高血压急骤进展的重度高血压头痛、视力下降、急性左心衰头痛、视力下降、急性左心衰ARF少尿、蛋白尿、血尿、颗粒管型少尿、蛋白尿、血尿、颗粒管型微血管性溶血性贫血微血管性溶血性贫血患者血浆肾素水平升高患者血浆肾素水平升高dcSSclcSS

9、cTherapy:VascularIdentification and treatment of severe organ-based complicationsTherapy:Vascular Immunosuppressive(Antifibrotic)SYSTEMIC SCLEROSISOverlap SScPakozdi et al J Rheum 2011Overlap features 332 of 1700(20%)SSc casesMyositis43%Arthritis 32%Sjogrens 17%SLE 8%Manage according to severity and

10、 activity of overlap features arthritis,myositis,lupusManagement of common morbidityRaynauds,upper GI,anorectal disease,erectile dysfunction,calcinosis,telangiectasiaManagement of systemic sclerosisPulmonary arterial hypertensionScleroderma renal crisisSystemic sclerosis as a vascular diseaseSRCSRC的

11、的ACEIACEI治疗治疗早期积极使用早期积极使用ACEI可以使部分病人避免透析治疗可以使部分病人避免透析治疗即使病人肌酐升高也要使用即使病人肌酐升高也要使用50的已透析的已透析SRC病人最终可以脱离透析病人最终可以脱离透析SRC脱离透析的病人的预后同未发生脱离透析的病人的预后同未发生SRC的的dSSc病人一样好病人一样好SRC预后预后20年随诊年随诊无肾脏损害表现者，死亡率无肾脏损害表现者，死亡率10有肾损害者死亡率有肾损害者死亡率60应用应用ACEI以前以前SRC的一年存活率的一年存活率22五年存活率五年存活率18应用应用ACEI之后之后SCR的一年存活率的一年存活率76五年存活率五年存活

12、率65Is there a role for ACEi prophylaxis?ACEi have no benefit for RP or digital ulcers QUINS trial1Use of ACEi/ATII blockers prior to SRC associated with trend towards worse outcome2,3Pooled data analysis from two studies gives OR 2.4(1.0-5.7 CI)and p=0.059 Chi-squared value 3.64Recent prospective in

13、ternational cohort study raised similar concernsNo dialysisDialysis and recoveryDialysis without recoveryTotalPre-SRC ACEi/ATII7(35%)3(15%)10(50%)20 No pre-SRC ACEi/ATII20(42%)14(29%)14(29%)48 Total27172468Odds ratio0.750.432.5Odds ratio 95%CI0.26-2.20.12-1.60.85-7.0Fishers exact test p value0.780.3

14、60.161.Glidden,et al.Arthritis Rheum 2007;55:3837-46.2.Penn,et al.QJM 2007;100:485-94.3.Teixeira,et al.Ann Rheum Dis 2008;67:110-6.4.Penn and Denton.Curr Opin Rheum 2008;20:692-6.5.Hudson et al.Int J Rheumatol.Epub 2010.糖皮质激素糖皮质激素/免疫抑制剂免疫抑制剂有内脏损害的弥漫型有内脏损害的弥漫型SSc患者患者肌炎、间质性肺炎、心肌病变、心包积液、肾炎肌炎、间质性肺炎、心肌病变

15、、心包积液、肾炎 强的松强的松3040mg.d-1，连用，连用34周后逐渐减量，以周后逐渐减量，以15mg.d-1维持维持不能阻止本病的进展不能阻止本病的进展 大剂量可能诱发大剂量可能诱发SRC 对弥漫型对弥漫型SSc尤其是伴有肾脏、肺脏等内脏损伤的患者，在给予强的松的同时需联合使用尤其是伴有肾脏、肺脏等内脏损伤的患者，在给予强的松的同时需联合使用免疫抑制剂免疫抑制剂 Rheum Dis Clin North Am.2003 May;29(2):409-26.常用的有环磷酰胺常用的有环磷酰胺 Clin Rheumatol.2003 Oct;22(4-5):289-94.Arthritis Rh

16、eum.2003 Aug;48(8):2256-61.依木兰依木兰 Clin Rheumatol.2004 Aug;23(4):306-9.CyA Intern Med.2004 May;43(5):397-9雷公藤等雷公藤等 造血干细胞移植造血干细胞移植（Expert Opin Biol Ther.2003 Oct;3(7):1041-9.）强化疗强化疗自体干细胞移植自体干细胞移植可维持皮肤和脏器功能稳定可维持皮肤和脏器功能稳定3年年欧洲最早报告欧洲最早报告47例，移植相关死亡例，移植相关死亡17%欧洲多中心研究欧洲多中心研究57例，死亡率例，死亡率8.7%（美、法研究，相似结果）（美、法研究，相似结果）Farge D.Ann Rheum Dis.2004 Aug;63(8):974-81.多中心前瞻性研究：重症、致死性损害多中心前瞻性研究：重症、致死性损害比较移植和传统治疗的优劣比较移植和传统治疗的优劣免疫重建免疫重建Best Pract Res Clin Haematol.2004 Jun;17(2):233-45免疫抑制剂免疫抑制剂 对弥漫型对弥漫型SSc尤其是伴有肾脏、肺脏等