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

Jo-1 Ab

DMitis/anti-synthetase syndrome

Anti-Mitochondrial

Ab

PBC

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

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

Cryoglobulinemia

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 Narcolepsy, multiple sclerosis,

Goodpasture’s syndrome

In human, MHC located on chr 6

HLA-A, B, Cclass I

HLA-D (M,O,P,Q,R)class II

寫法: class, family, chain

HLA-DR3 SLE, primary Sjogren’s syndrome,

Gluten-sensitive enteropathy,

chronic active hepatitis, dermatitis

herpetiformis, Graves’ disease,

type I DM, idiopathic membranous

GN, polymyositis-scleroderma

overlap

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