风湿总论

,(General Introduction),2004 Spring Teacher：毕黎琦,Rheumatic Diseases,Rheumatology,Section 1 General Introduction(总论) of Rheumatic Diseases Requirements(要求)：1. To know (了解)the definition of rheumatic diseases and its common pathology. 2. To be familiar with (熟悉)the classification of rheumatic diseases and the common characteristics of connective tissue diseases. 3. To grasp(掌握) the features of joint manifestation of rheumatic diseases and clinical importance (意 义)of auto-antibodies detection.4. To be familiar with(熟悉) the classification and mechanism of 3 kinds of anti-rheumatic drugs.,Definition: 1、Rheumatism (风湿)：Its defined as the chronic pain of joint, peri-articular tissues,muscle and bone. （风湿：是指关节、关节周围软组织、肌肉、 骨出现的慢性疼痛。）,2、Rheumatic diseases(风湿性疾病)： Its a disease entity involving joints and peri-articular tissues with different causes.(风湿性疾病：是指一大类病因各不相同，但均累及 关节及其周围组织的疾病。) It encompasses diffuse connective tissue diseases, and other disorders involving joints and peri-articular tissues (including muscle,tendon, ligament, etc).(它包括：弥漫性结 缔组织病及各种病因引起的关节和关节周围软组织，包括肌、 肌腱、韧带等的疾病。,概念： 3、 diffuse connective tissue disease(弥漫性结缔组织病)， 简称结缔组织病(CTD). Its the main kind of rheumatic diseases. It belongs to organ nonspecific autoimmune diseases(器官非特异性自身免疫病). Its also called collagenic disease(胶原病).,Common characters of diffuse CTD: （1）pathogenesis :autoimmunity; （2）pathologic change :chronic inflammation of vessels and connective tissue; （3）multisystem involvement. Heterogeneous (4) course:onsetremissionrecurrence remission. Lifelong diseases. （5）gluco-corticosteroid hormone and immuno- suppressants; （6）Improved prognosis.,The nomenclature and classification of arthritis and rheumatic diseases from American society of Rheumatology in 1993 美国风湿病学学会对关节炎和 风湿性疾病的命名和分类 （Arth Rheum 26:10291032,1993）一、 Diffuse connective tissue diseases (弥漫性结缔组织病) 1、Rheumatoid arthritis，RA (类风湿关节炎) 2、Juvenile rheumatoid arthritis，JRA (幼年类风湿关节炎) 3、 Systemic lupus erythematosus，SLE (系统性红斑狼疮) 4、 Systemic sclerosis，SSc (系统性硬化病) 5、 Diffuse fasciitis，EF (弥漫性筋膜炎),6、 Polymyositis/Dermatomyositis，PM/DM (多肌炎/皮肌炎) 7、 Vasculitis (血管炎) 8、 Sjogren syndrome，SS (干燥综合征) 9、 Overlapping syndrome，OS (重叠综合征) 10、 others :Polymyalgia rheumatica ( 风湿性多肌痛)二、Arthritis associated with spodylitis (并发脊柱炎的关节炎) Seronegative spondyloarthropathies (血清阴性脊柱关节病) 1、Ankylosing spondylitis，AS (强直性脊柱炎) 2、 Reiter syndrome，RS (赖特综合征) 3、 Psoriatic arthritis，PA (银屑病关节炎) 4、 Enteropathic arthritis，EA (炎性肠病关节炎),三、 Osteoarthritis，OA (骨性关节炎) 四、Infection (感染所致风湿性综合征) 1、 Reactive arthritis (反应性关节炎) Rheumatic fever (Rheumatic aithritis)，RF (风湿热) 2、 Direct infection (直接) 五、Metabolic and endocrinologic diseases (伴有风湿性疾病的 代谢或内分泌疾病) 1、 Gout (痛风) 2、 Pseudogout (假性痛风) 六、Others (其他) 1、Osteoporosis (骨质疏松) 2、Fibromyalgia syndrome，FS (纤维肌痛综合征) 3、Palindromic rheumatism，PR (复发性风湿病),Epidemiology of rheumatic diseases in China The incidence some rheumatic diseases: SLE：0.07% RA:0.320.36% AS：0.25% pSS：0.3% OA：50%（50 years old） Gout：increased day by day Rheumatic arthritis：decreased markedly,History taking and physical examination (病史采集和体格检查),History taking：1.Articular and peri-articular tissue involvement (关节及其周围组织受累情况) involved joint(site); disease course; swelling; pain; morning stiffness; relieving and aggravation factors; reaction to therapy; and so on.2.Organ system involvement (各器官系统受累情况) Kidney, heart, brain, liver, gastro-intesting, respiratory and hematologic system, skin, muscle. (Tab 8-1-1, P1029),History taking and physical examination (病史采集和体格检查),Physical examination： the general physical examination 一般内科体格检查Muscle examination:（myagia, myasthenia, myotrophy, myodynamia -6 grades:0-5）2. Skin-mucosa examination:(rashes, mucous membrane lesions)3. Joints:(site, symmetric, effusion, redness, swelling, heat, tenderness, active and passive motion of the joints and spine).,Differentiating features of common arthritides RA AS OA Gout SLEperipheral arthritisOnset slow,insidious slow,insidious slow,insidious abrupt uncertainFirst metacarpophalangeal knee knee,lumber, 1st metatarso- handInvolved joint,proximal inter- hip distal inter- phalangeal joint orJoint phalangeal joint,wrist ankle phalangeal joint joint others Pain persistent pain pain at severe pain uncertainFeature pain at rest at rest activity night pain Swelling soft tissue soft tissue bony redness,heat lessFeature hypertrophy swelling common Deformity common and obvious most in less rare rare joint function is limited hip joint commonChanges symmetric asymmetric weight-bearing recurrent multiarthritis lower limbs jointAxial skeleton occationaly must present lumber hyperplasia no noAnd/or sacro- joint function condensing osteitis iliac joints is limited lip-like change,常见关节炎的关节特点 关节 RA AS OA 痛风 SLE周围关节炎 起病 缓 缓 缓 急骤 不定 首发 MCP 膝、髋、 膝、腰、 大拇趾 手关节或 PIP、腕 踝 DIP 其他部位 痛性质 持续性 休息后 活动后 痛剧烈 不定 休息后加重 加重 加重 夜间重 肿性质 软组织为主 软组织为主 骨性肥大 红、肿、热 少数 畸形 常见，明显 多见于髋 小部分 少见 偶见 影响功能 演变 对称性 不对称 负重关节 反复发作 多关节炎 下肢大关节 症状明显脊柱炎和（或） 偶有 必有 腰椎增生 无 无骶髂关节病变 功能受限 唇样变,Laboratory test 实验室检查 1. Special examination Autoantibody examination： (1) ANA and ANA spectrum（抗核抗体谱） Anti ds-DNA SLE(50%) high specificity Anti-histone drug-induced lupus(95-100%),SLE(70%),RA(30%) Anti-Sm SLE(20-30%), marker antibody Anti-U1RNP MCTD(100%), SLE,SSc,etc Anti-SSA(Ro) SS(60-76%), SLE(30-40%) Anti-SSB(La) SS(50-60%), SLE(30-40%) Anti-Scl-70 SSc(15-20%) marker antibody Anti-Centromere SSc(CREST)(80%) marker antibody Anti-Ku SLE(10%), PM+SS overlap(55%) Anti Jo-1 PM(31%), marker antibody ,Laboratory test 实验室检查 （2） ANCA(antineutrophil cytoplasmic antibody,抗中性粒细胞胞浆抗体) 。 c-ANCA (cytoplasmic, 胞浆染色) proteinase 3(PR3) p-ANCA (perinuclear, 核周边染色) MPO diagnosis of vasculitis c-ANCA for Wegener granulomatosis (30-90% sensitivity, 98% specificity) （3）Antiphospholipid antibodies (抗磷脂抗体): include: Anticardiolipin antibody (抗心磷脂抗体) Lupus anticoagulant (狼疮抗凝物) False-positive serologic test for syphilis (假阳性梅毒血清试验) 1.multiple thromboses positive Antiphospholipid 2.thrombocytopenia + anti- phospholipid antibody syndrome 3.recurrent spontaneous antibody (抗磷脂综合征) abortion,Laboratory test 实验室检查 （4）RF (Rheumatoid factor, 类风湿因子 ) 80-90% RA. HLA-B27: 90% AS. Complement: CH50(serum total complement) ,C3, C4 for SLE and vasculitis. Analysis of synovial fluid: Common items: routine test(WBC count and differential count) biochemistry test(glucose, chloride) smear and culture for bacteria test RF Crystal test under micropolariscope(偏振光显微镜),Classification and differentiation of synovial effusions Diseases WBC differentiation neutrophil micro- count of WBC (%) polariscope 1. Normal 0-200 10 2. Non- OA 1000-2000 monocyte/ inflammation lymphocyte 20 3. Mild SLE 2000-5000 neutrophil 30 inflammation 4. Severe RA 5000-50000 neutrophil -70 inflammation Gout 5000-75000 neutrophil -70 crystal of urate (noninfectious) Pseudogout 5000-75000 neutrophil -70 crystal of CPPD* *CPPD:calcium pyrophosphate dihydrate 5. Severe inflammation Bacterial (infectious) infections 5000-250000 neutrophil -90,2. General examination Blood routine for WBC,RBC, pletlet count Urine routine to test WBC, RBC, casts in urine, also glucose, protein, crystal, pH, gravity, bacteria, bilirubin, urobilinogen,etc. Hepatic function test(AST, ALT) Renal function test (BUN-blood uric nitrogen, creatinine),2. Radiographic studies 1. Plain film： convenient,economic; but samll erosion of joint may be missed. 2. CT： for sacro-iliac joint (sacroiliitis in AS) for brain (SLE) for lung(interstitial fibrosis) 3. MRI (magnetic resonance imaging) : expensive,for brain,spinal cord,muscle,bone,small erosion. 4. Angiography (血管造影) expensive and traumatic; for vasculitis (polyarteritis nodosa, Takayasus arteritis大动脉炎）,Pathology (病理) Biopsy : renal biopsy-lupus nephritis(SLE) muscle biopsy-myositis(PM/DM) labial mucosa,parotid gland-cheilitis lupus band test-SLE 1. Inflammatory reaction： Its manifested by infiltration gathering of lymphocytes, macrophages and plasma cells due to immunologic reaction