Intern hour Rheumatology 柯祈化醫師
Approach of ANA disease
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
ANA 基本型態
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
Peripheral pattern 與 Chromatin( 染色質 ) 有關的抗體 尤其是 Anti-dsDNA Ab Pattern specific for SLE
Coarse speckled pattern RNP Ab 與 MCTD, SLE, PSS, 雷諾氏現象有關 Smith Ab Specific for SLE
Fine speckled pattern 較無專一性, 也可說是最常見的型態 SSA (anti-ro) SSB (anti-la)
Cytoplasmic pattern Ribosomal-P Ab Neuropsychiatric lupus Anti-Mitochondrial Ab PBC Jo-1 Ab DMitis/antisynthetase syndrome
Discrete Speckled Pattern Centromere Ab ( 著絲點抗體 ) Limited type PSS, CREST syndrome Sjogren syndrome Primary biliary cirrhosis Pulmonary hypertension
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
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
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
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
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
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
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
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
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
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
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
ESR and CRP
ESR > 100 is found in GCA, CTD, SBE, osteomyelitis, TB, renal cell carcinoma, multiple myeloma, and paraporteinemias
Extracellular pattern-recognition receptors
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 35000 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
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
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.
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