Intern hour

Size: px

Start display at page:

Download "Intern hour"

炮庞
5 years ago
Views:

1 Intern hour Rheumatology 柯祈化醫師

5 Approach of ANA disease

6 抗核抗體 ANA 效價 : 以螢光指數表示不論型態時 :>1:160 以上較有意義高效價 : 紅斑性狼瘡 SLE 混合性結締組織病變 : MCTD 低效價老年人 Autoimmune disease: S.S., RA, SAE Infection, 尤其是慢性感染 ( 如 TB 等 ) Malignancy Drug-induced: 高血壓藥, 抗結核藥

7 ANA 基本型態

8 Diffused pattern 與 Chromatin( 染色質 ) 有關的抗體 Anti-dsDNA Ab Specific for SLE Anti-Histone Ab Drug induced lupus 有關的抗體

9 Peripheral pattern 與 Chromatin( 染色質 ) 有關的抗體尤其是 Anti-dsDNA Ab Pattern specific for SLE

10 Coarse speckled pattern RNP Ab 與 MCTD, SLE, PSS, 雷諾氏現象有關 Smith Ab Specific for SLE

11 Fine speckled pattern 較無專一性, 也可說是最常見的型態 SSA (anti-ro) SSB (anti-la)

12 Cytoplasmic pattern Ribosomal-P Ab Neuropsychiatric lupus Anti-Mitochondrial Ab PBC Jo-1 Ab DMitis/antisynthetase syndrome

13 Discrete Speckled Pattern Centromere Ab ( 著絲點抗體 ) Limited type PSS, CREST syndrome Sjogren syndrome Primary biliary cirrhosis Pulmonary hypertension

14 Nucleolar pattern 染在核仁上有許多種特有抗體本例為 anti-scl-70 Ab (DNA topoisomerase Ab) 與廣泛性硬皮症 (dffused type PSS) 相關 Anti-Scl70 Ab

16 抗磷脂質抗體症候群 APS 抗體 Lupus Anticoagulant (LA, 紅斑抗凝血質 ) 與 thromboembolism 相關性高 Anticardiolipin Ab ( 抗心質抗體 ) Pathogenic Ab ( 致病性抗體 ) ACA-IgG & IgA 與疾病較有關聯 ACA-IgM 一般與感染較有關 Cofactor Ab AB2GPI Ab: 與 thromboembolism 相關性高 APhL Ab: 目前認為最 sensitive 的檢查

21 Detection of ANCA ANCAs were originally described based on their immunofluorescence patterns cytoplasmic (c-anca) and perinuclear (p-anca) The antigens responsible for these patterns have also been identified proteinase 3 (PR3) for c-anca myeloperoxidase (MPO) for p-anca

25 C 7. 全身性紅斑狼瘡病人血中可能偵測到許多不同的自體抗體 ; 請依出現率 (prevalence) 從高至低排列下列抗體. 1.ANA 2.Anti-Sm 3.Anti-DNA 4.Anti-phospholipid. 請問哪一個排列是正確的? A.1>2>3>4 B.1>3>2>4 C.1>3>4>2 D.3>1>4>2 E.3>2>1>4

26 A 試問下列的自體抗體與自體免疫疾病的配對中, 何者不正確? A. Anti-Jo-1---PSS B. Anti-centromere ---CREST syndrome C. canca---wegener's garnulomatosis D. Anti-ds DNA --- SLE E. Anti-U1 RNP --- MCTD

27 類風濕因子 Rheumatoid Factor Anti-human IgG Ab 一般是指 anti-igg IgM (RF-IgM) 許多狀況均可呈陽性, 不是類風濕因子陽性加關節炎就是類風濕關節炎 Autoimmune disease: RA, S.S.,SLE Infection:Bacterial:SBE, HCV-infection, TB/Fungal infection Malignancy 1:160 is positive

28 補體 Complement C3, C4 下降 : SLE, Vasculitis, 先天性補體缺乏, 肝功能障礙上昇 : 懷孕, 發炎性關節炎, 感染

31 Cryoglobulin ( 冷澱球蛋白 ) 遇冷沈澱, 回溫溶解的免疫球蛋白複合體可沈澱在血管及組織中引起發炎病變

33 Cryoglobulinemia 類型免疫球蛋白類風濕因子相關疾病 I 單株 monoclonal 對單株 monoclonal 無骨髓瘤 II 單株 monoclonal 對多株 polyclonal IgG 有單株 IgM 急性及慢性感染 C 型肝炎自體免疫疾病 III 多株 polyclonal 對多株 polyclonal 有多株 IgM 自體免疫疾病淋巴瘤 A disease which predominantly affects middle-aged persons Females affected more often than males: F/M ratio 3:1 HCV positivity in more than 90% of cases Histologically: small vessel leukocytoclastic vasculitis Clinically: dependent purpura, appears in almost all patients. Other common features: weakness, arthralgias, liver involvement, Raynaud's phenomenon and multiplex mononeuritis

36 HLA-A29 Birdshot choroidretinitis HLA-DR4 RA, type I DM, IgA nephropathy, Pemphigus valgaris (Jews), MCTD HLA-B5 Behcet s disease, ulcerative colitis, polycystic kidney disease HLA-DR5 Pauciarticular juvenile RA, Pernicious anemia, Hashimoto s thyroiditis HLA-B27 Seronegative spondyloarthropathy HLA-DR7 Congenital adrenal hyperplasia HLA-B38 Psoriatic arthritis HLA-DQ Polymorphisms associated with various autoantibodies produced in SLE patients HLA-DR1 RA HLA-DQ3,2 Type I DM HLA-DR2 HLA-DR3 Narcolepsy, multiple sclerosis, Goodpasture s syndrome SLE, primary Sjogren s syndrome, Gluten-sensitive enteropathy, chronic active hepatitis, dermatitis herpetiformis, Graves disease, type I DM, idiopathic membranous GN, polymyositis-scleroderma overlap In human, MHC located on chr 6 HLA-A, B, C class I HLA-D (M,O,P,Q,R) class II 寫法 : class, family, chain

37 A 1. 下列有關風濕性疾病與 HLA 的相關性配對中, 何者有對? A. Reiter's syndrome --- HLA-B27 B. Systemic lupus erythematosus --- HLA-DR4 C. Behcet's disease --- HLA-DR3 D. Rheumatoid arthritis --- HLA-DR2 E. Type I diabetes mellitus --- HLA-B51

38 ESR and CRP

40 ESR > 100 is found in GCA, CTD, SBE, osteomyelitis, TB, renal cell carcinoma, multiple myeloma, and paraporteinemias

41 Extracellular pattern-recognition receptors

43 A 6. 一位 28 歲男性病人主訴右膝關節腫痛已經一個月, 並無伴隨發燒發冷. 抽取之關節液為混濁色, 其中 WBC cells/mm3, Seg. 佔 85% ; 並無發現針狀之結晶物,Gram stain 也為陰性, 請問最不可能的診斷為何? A. Osteoarthritis B. Gouty arthritis C. Reactive arthritis D. Gonococcal arthritis E. Septic arthritis

44 SYNOVIAL FLUID ANALYSIS synovial fluid is an ultrafiltrate of plasma plus hyaluronate; it lubricates joint surfaces and nourishes articular cartilage analysis provides definitive diagnosis for infectious, inflammatory, and crystalline disease normal synovial fluid is colourless or straw-coloured and has <200 WBC/mm3 Three Most Important Tests of Synovial Fluid (The Three Cs) Cell count and differential Crystal examination Culture and Gram stain

45 A simplified algorithm for analyzing synovial fluid samples and initiating a plan of management. CPPD, calcium pyrophosphate dihy- drate; NSAID, nonsteroidal anti-inflammatory drug; WBC, white blood cell.

49 negatively birefringent = yellow when parallel to axis of red rhomboid-shaped positively birefringent = blue when parallel to axis of red compensator Cholesterol crystals in a synovial fluid sample

untitled

untitled (CIP) / - :, 2004 7 ( ) ISBN 7801943155 R593 21 CIP ( 2004) 042664 : : : : : : : : : : 22 3 :100842 : (010) 66882586() 51927290( ) : (010) 68222916() 66882583( ) : www.pmmp.com.cn : : :787mm1092mm 1/ 36