Musculoskeletal, skin and connective tissues Flashcards

1
Q

20 - 50 female, 对称性、弥漫性肌肉痛 + insomnia + fatigue: Dx? Rx? 和 polymyalgia rheumatic, polymyositis 鉴别诊断

A

Fibromyalgia 纤维肌痛 病因不明 Rx:regular exercise + antidepressenets (TCAs, SSRIs) + anticonvulsants

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2
Q

> 50 yr, 双侧、对称性pain and stiffness in shoulders and hips, often with constitutional sym (night fever, malaise, weight loss): Dx? disease association? lab? Rx?

A

Dx: polymyalgia rheumatica 风湿性多肌病 disease association: temporal arteritis (50%颞动脉炎病人合并此病 - 怀疑颞动脉炎要马上给激素,防止blindness) lab: ↑ ESR, ↑ CRP, normal CK Rx: low-dose steroids

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2
Q

2nd MC skin cancer? cause? Cx? 好发部位?microscope? if metastasize? precursor disease (重要考点)? a variant disease? 其特点?

A

2nd MC skin cancer: SCC (squamous cell carcinoma) cause: sun, immunosuppression, arsenic exposure Cx: ulcerative lesions with frequent scale 好发部位: face, lower lip, ears, hands microscope: keratin “pearls” if metastasize: rarely, locally invasive, but may spread to lymph nodes precursor disease (重要考点): AK (actinic keratosis) 光化性角化病 a variant disease:keratoacanthoma 角化棘皮瘤 grow rapidly (4-6 wks), but may regress spontaneously over months

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3
Q

Acanthosis nigricans (AN): Cx? Disease association (最重要的考点!!)?

A

Cx: epidermal hyperplasia, symmetrical hyperpigmented, velvety thickening of skin, 好发部位在neck and axilla 腋窝 associated with: 2大类,分为良性、恶性: 1. benign AN: insulin resistance (DM, obesity, cushing syn) 2. malignant AN: visceral malignancy, especially GI and genitourinary neoplasm 如果是中老年病人,忽然出现黑色棘皮,一定要怀疑恶性肿瘤 最常见的肿瘤:gastric adenocarcinoma

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3
Q

Acetaminophen: 什么类型药? Mechanism? 可逆否?作用位点?inactivation位点? 是否解热?是否抗炎?Use? toxicity and the mechanism? antidote?

A

扑热息痛,和NSAID类似,都是可逆地抑制COX 作用位点: CNS inactivation位点: peripheral 是否解热? - yes (anti-pyretic) 是否抗炎?- No Use: use instead of aspirin in children to avoid Reye syn toxicity and the mechanism: hepatic necrosis; acetaminophen metabolite (NAPQI) depletes glutathione - form toxic tissue adducts in liver antidote: N - acetylcysteine (regenerate glutathione)

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4
Q

Albinism病因?黑色素细胞数目有无变化?

A

normal melanocyte #, with ↓ melanin production 病因: 1. ↓ tyrosinase activity 2. defective tyrosine transport 3. failure of neural crest cell migration during development ↑ risk of skin cancer

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4
Q

actinic keratosis: Cx? why important?

A

光化性角化病 Cx: small, rough (feel like “sand paper”) erythematous or red/brown papules on sun-exposed area; confined to epidermis premalignent to SCC! need to be monitored

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4
Q

acute gout drugs?

A
  1. NSAIDs: naproxen 萘普生; indomethacin 2. glucocorticoids (oral or intraarticular) 3. colchicine (stabilize microtubulin ; inhibit chemotaxis of leukocytes) GI side effects
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4
Q

Adalimumab: 什么类型药?同种药物还有什么? Mechanism? use? 要注意什么?

A

anti-TNFa McAb (同种药物还有Infliximab) use: IBD,RA, psoriasis, AS (ankylosing spondylitis) 要注意什么:prediposit for infection (reactive latent TB)

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5
Q

allergic contact dermititis: type ? hypersensitivity reaction major histological changes?

A

type IV histological changes: spongiosis 棘细胞水肿

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6
Q

Allopurinol抗痛风的机制?同样的药物还有? 如果用于lymphoma, leukemia机制?要注意什么?

A

Allopurinol抗痛风机制:inhibit XO (xanthine oxidase) 同样的药物还有Febuxostat。急性发作时是禁忌症(应用NSAIDs) 如果用于lymphoma, leukemia机制: prevent tumor lysis-caused irate nephropathy 要注意如果和azathioprine, 6-MP联用抗肿瘤,会↑ 这两种药物concentration: (both metabolized by XO), 所以如果联用,一定要降低抗肿瘤药物的剂量!!!!

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8
Q

Ankylosing spondylitis: Cx? XR? HLA (class I or II?) 累及其他的器官表现?

A

Cx: chronic dull back pain, morning stiffness, can be relieved by over-counter pain killer; 一生中不断缓解和复发 XR: “bamboo spine”- vertebral fusion HLA-B27 (class I; class II are DR, DP, DQ) 除了脊柱之外常累及: 1) respiratory: pain limits chest expansion - hypoventilation: 病人随访中必须监测chest expansion 2) cardiac: ascending aortitis ⇒ dilation of aortic ring, aortic insufficiency - AR (aortic regurgitation) 3) eye: uveitis: pain in the eyes, blurred vision, 结膜充血

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8
Q

Aspirin: Mechanism? 作用可逆否? Clinical use? Toxicity? 对respiratory center作用?

A

Mechanism? 作用可逆否: covalently acetylate cyclooxygenases (both COX1 and COX2), ⇒ ↓ TXA2, PGs Clinical use: 1. low dose (< 300 mg/day): anti-platelet aggregation (↑ bleeding time until new platelets are made: ~ 7 d; no change in PT, PPT) 小剂量aspirin用于prevention of transient ischemic attack, MI, stroke [对于PE不如wafarrin有效,对于DVT不如heparin有效】 2. intermediate dose (300 - 2400 mg/d): anti-pyrectic, analgesic 3. high dose (> 2400 mg/day): anti-inflammation Toxicity: 1. in adults: gastric ulcer, tinnitus (CN VIII), chronic use caused GI bleeding, nephritis, acute renal failure 2. to treat kids for viral infection: Reye syn stimulate respiratory center⇒ hyperventilation ⇒ respiratory alkalosis

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9
Q

atopic dermatitis又名? 诊断hallmark? associated with diseases?

A

atopic dermatitis又名eczema 湿疹 诊断hallmark: intense pruritis, can’t Dx without it 常在5-6岁(甚至婴儿期起病),start on the face in infancy, and often appears in the antecubital fossae thereafter; 终身反复, often associated with other atopic diseases (asthma, allergic rhinitis - UW 考点)

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9
Q

biphosphonates (Alendronate, other -dranotes) 什么类型药? Mechanism? clinical use? toxicity? 如何预防?

A

什么类型药:pyrophosphate analogues Mechanism: inhibit osteoclast activity by binding to hydroxyapatite in bone clinical use: osteoporosis, 恶性高钙血症, Paget disease toxicity - corrosive esophagitis; osteonecrosis of the jaw 如何预防- pts take lot of water and remain upright for 30 min;

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10
Q

Dx: fever + generalized erythematous rash + sloughing of the upper layers of the epidermis that heals completely; seen in newborns and children

A

Dx: Staphylococcal scalded skin syndrome exotoxin destroys keratinocyte attachments in the stratum granulosum (颗粒层,皮肤从外到里第3层), ⇒ destroy the epidermal dermal junction

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10
Q

blistering skin disorders有哪两种? 区别: 1. 严重程度? 2。Ab against? 3. oral mucosa involvement? 4. 荧光染色location? pattern? 5. Nikolsky sign positive/negative?

A

PA vs. bullous pemphoid, see “notes for printing”

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10
Q

Dx: multiple types of skin lesions (macules, papules, vesicles, “target lesions”-targets with rings and a dusky center showing epithelial disruption) associated with?

A

Dx: erythema multiforme associated with: 1. infection - mycoplasma pneumoniae, HSV 2. drugs (sulfa, beta-lactams, phenytoin) 3. cancers 4. autoimmune

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10
Q

Dx: multiple plaques with collarette scale (蜀黍红疹), “herald patch” followed days later by “X’mas tree” distribution?

A

Pityriasis rosea 玫瑰糠疹 self-resolving in 6-8 wks

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12
Q

Dx: progressive symmetric proximal muscle weakness (常考的描述是“can’t comb hair, can’t raise from chair, 说明累及shoulders and hips):最可能的诊断? activation of which immune cells? release cytokines?

A

Dx: polymyositis endomysial inflammation with CD8+ Th1 cells: activate macrophage to release IL-1, TNFa

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13
Q

Dx: proximal muscle weakness (can’t comb hair, can’t clim up stairs) + autonomic sym (dry month, diplopia, impotence)? associated with which disease? 病因?和什么疾病鉴别?

A

Dx: Lambert-Eaton myathenic syndrome (LEMS) associated with small lung cancer (i.e.: CXR 可见mass in upper lung) 病因: autoantibodies to presynaptic Ca2+ channel ⇒ ↓ Ach release (improve with muscle use) 和什么疾病鉴别: MG (myasthenia gravis) - ab against Ach postsynpatic receptors, worsen with muscle use, associated with thymoma, sym can be reversed with AchE inhibitors)

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14
Q

Dx: pruritic papules, bullae on elbow; 和什么病鉴别? which Ab deposit at where? disease association?

A

dermatitis herpetiforms 和PV (pemphigus vulgaris), bullous pemphipgoid鉴别 IgA deposited at tips of dermal papillae; associated with celiac disease

16
Q

Dx: skin rash (骨关节突出部位) + progressive muscle weakness? Lab? Rx?

A

dermatomyositis malar rash, Gottron papules (手指骨关节的红斑)-特征性病变 Heliotrope rash (upper eye lids或者眶周红斑) -特征性病变 CD4+ T cells Lab: ↑ CK (-特征性病变), anti-Jo-1 (-特征性病变), anti-SRP (specific but not characteristic), anti-Mi-2 Rx: steroids

17
Q

Dx: white, painless plaques on the tongue, can’t be scraped off caused by which virus? in which population of pts?

A

Dx: hairy leukoplakia 毛状白斑 caused by EVB occur in HIV+ pts

18
Dx: yo male, fever, malaise, macropapular rash on palms and soles?病原体? screen test? 确诊test?
Dx: 二期梅毒 病原体:Treponema pallidum screen test: RPR(VDRL) test: add pt's serum to a mix of cardiolipin + lecithin + cholesterol: flocculation (= aggulation) occurs 用于筛查,不特异 确诊test: FTA-ABS
19
erythema nodosum Cx? Disease association
结节性红斑 Cx: painful, inflammatory lesions, 多发于腿部 extremely non-specific findings, associated病实在太多了!! sarcoidosis, coccidiodomycosis, histoplasmosis, TB, streptococcal infection, leprosy, Crohn disease
20
Etanercept: 什么类型药? Mechanism? use? 要注意什么?
依那西普 TNFa decoy receptor (fusion protein of TNGa receptor + IgG1 Fc) use: RA, psoriasis, AS (ankylosing spondylitis) 要注意什么:prediposit for infection (reactive latent TB)
21
febuxostat: 什么类型药? Mechanism?
gout prevention 机制和allopurinol 类似,inhibit XO (xanthine oxidase)
22
Gout的2种原因?和pseudogout的鉴别?
Gout : 90%: underexcertion of uric acid - 原发性较多,也可能由medication加重(thiazide diuretics) 10%: overproduction of uric acid, caused by: Lesch-Nyhan, von Gierke (糖原储积病,G-6-phophatase deficiency), PRPP, ↑ cell turnover (tumor lysis syn) birefringent偏振光: gout: negative (crystals are yellow under parallel light; blue under perpendicular light) pseudo gout:positive; crystals are blue under parallel light;
24
Infectious arthritis: MCCs?
In US: gonococcal arthritis是最常见的感染性关节炎!!! "STD" = synovitis 滑膜炎,tenosynovitis 腱鞘炎,dermatitis presents as migratory arthritis, asymmetric; affected joint is swollen, red and painful S. aureus, Streptococcus, Neisseria gonorrhoeae
25
Infliximab: 什么类型药? Mechanism? use? 要注意什么?
anti-TNFa McAb use: IBD,RA, psoriasis, AS (ankylosing spondylitis) 要注意什么:prediposit for infection (reactive latent TB)
26
Lichen Planus: Dx: 6P? Mucosal involvement manifest? patho见什么细胞浸润皮肤什么部位? Disease association
Dx: 6P: pruritic, purple (在黑皮肤上不一定是紫色),polygonal, planner papule or plaque Mucosal involvement manifest: reticular white lines (Wickham striae) patho见什么细胞浸润皮肤什么部位: sawtooth infiltration of lymphocytes at dermal-epidermal junction Disease association: hepatitis C
27
MC skin cancer? cause? Cx? microscope? if metastasize?
BCC (basal cell carcinoma) cause: sun exposure Cx: pink, pearly nodules, central crusting or ulceration; non-healing ulcers, or scaling plaque microscope: nests of basaloid cells; palisading (栅栏样的) nuclei if metastasize: almost never; only locally invasive
29
myositis ossificans 骨化性肌炎 什么病?常见部位?
metaplasia (组织转化) of skeletal muscle to bone following trauma 常见部位:upper or lower extremity present as suspicious mass, 或者XR偶然发现
31
osteopetrosis: defecit in? X-ray表现?对骨髓、颅神经的影响?potential Rx?
deficit: mutations (eg: carbonic anhydrase II 碳酸酐酶) ⇒ osteoclasts deficit!! can't reabsorb bones X-ray表现: thickened, dense bones prone to fracture ("bone in bone" 对骨髓、颅神经的影响: fill BM ⇒ pancytopenia, extra medullary hematopoiesis; if narrowed foramina: can result in cranial nerve impingement and palsies potential Rx: BMT
32
osteoporosis: 哪种骨受累?Dx? Types, 各自骨折最容易发生部位, 以及损伤血管? Rx?
哪种骨受累:trabecular (spongy) bone loses mass and interconnections; [normal bone mineralization: so normal lab values: serum Ca2+, PO3-正常】 Dx: DEXA (bone mineral density test) with a T-score \< 2.5 Types: Type 1: postmenopause, due to ↓ E2; MC seen in femoral neck fracture 【损伤medial femoral circumflex A, distal radius fracture; Type II: senile osteoporosis, in \> 70-yr old, MC in spine [compression fracture] Rx: - prophylaxis: regular weight-bearing exercise, adequate calcium and VitD intake Rx with biphsphonates, PTH, SERMs, denosumab (McAB against RANKL)
34
Paget disease: Patho: which cell(s) involved? Stages of disease? Lab? Cx? 累及部位? Biopsy可见? ↑ risk for which cancer?
bone remodeling deficit, common Patho: which cell(s) involved: both osteoclasts and -blasts, 主要是osteoclast activity ↑, too much bone absorption ⇒ bone pain Stages of disease: (先破后立) - lytic: osteoclasts - mixed: osteoclasts + osteoblasts - sclerotic: osteoblasts quiescent: minimal osteoblasts/osteoclast activity Lab: normal serum Ca2+, PO3-, PTH ↑ ALP, ↑ activity of M-CSF, RANK-L (to stimulate osteoclasts) Cx: bone pain, long bone chalk-stick fractures; can affect skull - hearing loss Biopsy可见: mosaic pattern of woven and lamellar bone, multinucleate cells (破骨母细胞), ↑ risk for which cancer: osteogenic sarcoma
35
Probenecid:什么类型药? Mechanism? 要注意什么?
丙磺舒, 抗痛风(预防和慢性治疗)急性发作时是禁忌症(应用NSAIDs) Mechanism: inhibit reabsorption of uric acid in PCT; also inhibit secretion of penicillin
36
psoriasis: 皮肤哪(几)层厚度变化? microscopic change? associated with diseases? Rx?
↑ stratum spinosum (棘层变厚,第四层);↓ stratum granulosum (颗粒层【第三层】变薄) parakeratotic scaling; pinpoint bleeding when scales are scraped off associated with diseases: nail pitting, psoriatic arthritis Rx:
38
Reactive arthritis (Reiter syn): Cx? HLA? Cause?
post GI infection or Chlamydia (衣原体)感染, 例如腹泻后2周出现关节炎症状:symmetric large joint arthritis, 关节炎培养无细菌(sterile synovial fluid on joint aspiration) Cx: classic triad (Can't see, can't pee, can't bend my knee) 1.Conjunctivitis and anterior uveitis 葡萄膜炎 2.urethritis 3. arthritis HLA-B27 病原体:SSYCC Shigella, Salmonella, Yersinia, Campylobacter 弯曲杆菌, Clamydia
39
Sarcoidosis: Cx? CXR? patho? Ca2+变化,什么原因?哪些immune cells involved?
Cx: african American + bilateral hilar adenopathy on CXR, patho 见noncaseating granulomas —— 考题里绝对是诊断为sarcoidosis!! Ca2+ ↑ , caused by activated macrophages synthesized too much 1, 25-VitD ⇒ suppress PTH secretion Immune cells: Th1 cell-mediated immune reaction: these T cells secrete IL-2 and INFƔ
40
Scleroderma (systemic sclerosis) 病因?Cx? 好发人群?分型及ab association?
病因:excessive deposition of fibrosis and collagen throughout the body Cx: 1. 皮肤表现:puffy and taut skin, without wrinkles 2.其他器官:sclerosis of renal, pulmonary (MCC of death), cardiovascular, GI 好发人群: females 2 types: 1) diffuse scleroderma: rapid progression + early visceral involvement, associated with anti-Scl-70 Ab (anti-DNA topoisomerase I ab) 2) limited scleroderma: CREST, more benign course associated with anti-Centromere Ab (C for CREST)
41
seronegative spondyloarthropathies: definition? HLA association? 包括哪些疾病?
definition: rheumatoid factor negative (rheumatoid factor: anti-Ig IgM antibody) HLA association: HLA-B27 (encodes HLA MHC class I) 包括哪些疾病: PAIR 1. psoriatic arthritis: t bend my knee
43
Sjögren's syndrome: 什么病?特征?好发人群? Cx? Ab markers有哪些?腮腺的表现? complications?
Autoimmune, destruction of exocrine glands, especially lacrimal and salivary glands 好发人群: 40-60 females Cx: 1. xerophthalmia (↓ tear ⇒ damage to cornea) 眼干燥 2. xerostomia ((↓ saliva) 口腔干燥 3. bilateral parotid enlargement Ab markers有anti-nuclear Ab : SS-A (anti-Ro), SS-B (anti-La) 腮腺: bilateral enlargement (和并发症里MALT lymphoma的单侧腮腺肿大区别) complications: 1)dental caries 2) MALT lymphoma (mucosa-associated lymphoid tissue); may present as unilateral parotid enlargement
44
Skin biopsy 见stellar cells with intracytoplasmic granules (shape: tennis racquet): what are these cells? express which MHC? co-stimulatory molecule? derived from ? lineage?
Langerhans cells (dendritic cells, APC) express MHC II co-stimulatory molecule: B7 derived from myeloid lineage
46
SLE: Cx? 哪种免疫细胞介入? MCC for death? 心脏、肾脏累及的表现? Rx?
Cx: RASH OR PAIN Rash Arthritis Soft tissues/serositis Hematologic disorder (cytopenias) Oral /nasopharyngeal ulcers Renal disease, Raynauld phenomenon Photosensitivity,Positive VDRL/RPR Antinuclear Abs (ANA-sensitive but not specific, anti-dsDNA - specific, poor prognosis, indicating renal disease; ) Immunosuppressants (↓ C3, C4, CH50 due to immuoplex formation) Neurologic disorders (seizures, psychosis) 哪种免疫细胞介入: Th1 (例如Fas gene mutation, Th1 over activated) MCC for death: 1. Cardiovascular diseases 2. infection 3. renal disease 心脏累及的表现: 1. pericarditis, 胸膜炎 【pain radiates to back, shoulders; inspiration加重,relived by sitting up] 2.Libman-Sacks endocarditis: wart-like vegetations on both sides of valve 肾脏累及的表现: lupus nephritis (type III hypersensitivity) 1. Nephritic - diffuse proliferative glomerulonephritis 2. neprhotic - membranous glomerulonephritis Rx: NSAIDs, steroids, immunosuppressants, hydroxychloroquine
47
specific COX2 inhibitor? why use selective inhibitor against COX2? clinical use? COX2有什么特点?
celecoxib 考昔 nonspecific COX inhibitors (NSAIDs) cause GI bleeding and renal damage celecoxib does not have the corrosive effects on the GI lining; spare platelet functions (TXA2 production is NOT dependent on COX-1) Used in RA and osteoarthritis, or pts with gastritis or ulcers COX2有什么特点:72 KDa enzyme, inducible, very specifically expressed in inflammatory cells and vascular endothelium; almost undetectable in other tissues !
48
Stevens-Johnson syn: Cx: Association? If \> 30% skin involved: Dx?
Cx: fever + bulla formation and necrosis + sloughing of skin [high mortality] 一般至少有2种mucous involved If \> 30% skin involved: Dx for toxic epidermal necrolysis
49
sunburn中 UVA dominant in which cases? wavelength? protected by? UVB? wavelength? protected by?
UVA dominant in: tanning, photoaging; 320 - 400 nm, protected by avobenzone 阿伏苯宗 UVB: sunburn; 290- 320 nm, protected by PABA (para-aminobenzoic acid) 对-氨基苯甲酸 Zinc oxide protects against both
50
urticaria [er:ti'karia]: patho?
荨麻疹 mast cell degranulation superficial dermal edema, lymphatic channel dilation
51
very painful skin infection lesion + honey-colored crusting, or bullae: Dx? 病原体?- 这些病原体还引起什么常见的皮肤病? 是否传染? 最常见病原体的virulence factor, 其作用?
1. Dx: impetigo [impi'taigou] 脓疱病 caused by S. aureus or S. progenies highly contagious,在皮肤表浅处感染 2. S. aureus or S. progenies如果在dermis and subcutaneous tissues感染, often starts with a break in skin from trauma or another infection: cellulitis 蜂窝织炎 3. S. aureus or S. progenies如果在皮肤深处感染,还常常引起necrotizing fasciitis 坏死性筋膜炎 最常见病原体S. aurues的virulence factor: protein A 其作用: bind the Fc portion of IgG ⇒ impaired complement activation (↓ C3b), opsonization, phagocytosis
52
最容易转移的皮肤癌? tumor marker? Cx? gene mutation? Rx?
melanoma: associated with sun exposure; light skin: ↑ risk ["Greys' anatomy"里Izzy case] tumor marker: S-100 Cx: "ABCDE" Asymmetry Border irregularity Color variation Diameter \> 6 mm Evolution over time gene mutation: BRAF kinase Rx: 1) excision with wide margins 2) if can't resect or metasize + BRAF V600E (Val ⇒ Glutamic acid) mutation: Vemurafenib (BRAF kinase inhibitor)
53
确诊RA需要什么Ab?
Not RA!!! anti-CCP ab (cyclic citrullinated peptides)