Rheumat Flashcards

1
Q

ACR 1997 Classification criteria for SLE

*American College of Rheumatology

A

4 out of 11
SOAP BRAIN MD

  1. Serositis (Pleurisy, pericarditis)
  2. Oral ulcers
  3. Arthritis
  4. Photosensitivity
  5. Blood disorders (Leukopenia, Lymphopenia, Thrombocytopenia, Hemolytic anemia)
  6. Renal disorders
  7. ANA (High titre = >1/160)
  8. Immunological disorders (Anti-dsDNA, Anti-Smith Ab, Anti-phospholipid Ab)
  9. Neurological disorders
  10. Malar rash
  11. Discoid lupus erythematosus
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2
Q

Anti-dsDNA antibodies

A

Anti-double stranded DNA
(for SLE)

A group of ANA, target antigen is double stranded DNA Can detect by ELISA and immunofluorescence
Highly diagnostic of SLE
Implicated in the pathogenesis of lupus nephritis

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3
Q

ELISA

A

Enzyme-linked immunosorbent assay

酵素結合免疫吸附分析法

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4
Q

Temporal patterns of joint involvement in polyarthritis

A
  1. Migratory (rheumatic fever, GC arthritis)
    - Remit, then reappear in other joints
  2. Additive (RA, SLE)
    - Progressively worsen
  3. Intermittent (RA, polyarticular gout, palindromic rheumatism)
    - Complete remission in btn attacks
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5
Q

Ix for Chronic polyarticular Sx

A
  1. CBC
  2. ESR, CRP
  3. L/RFT
  4. Serum uric acid
  5. Urinalysis
  6. Synovial fluid analysis
  7. Relevant serological tests, e.g. ANA, RF, Anti-CCP
  8. Relevant radiological studies
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6
Q

Rheumatoid factor

A

IgM, an autoAb against IgG
Not very sensitive (70% positive in RA)
Not specific
High level = poor RA prognosis

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7
Q

Anti-CCP

A

Anti-cyclic citrullinated peptide Abs

  • Specific for RA (>90%)
  • Predictive of progressive and erosive disease
  • Allow early Dx
  • Not sensitive (60%)
  • Sensitivity better if screened tgt with RF
  • 100% sensitivity + specificity for RF + Anti-CCP tgt
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8
Q

Ankylosing spondylitis associations

A
HLA-B27
SI joints, spine
Reactive arthritis (Reiter's syndrome), psoriasis, IBD
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9
Q

Pharm tx for RA

A
  1. Pain relief
    • Panadol / NSAID / COX-2 inhibitor
  2. DMARDs (Disease-modifying anti-rheumatic drugs)
    • Start within 3m if poor result with NSAIDs
      1. Hydroxychloroquine
      2. Methotrexate
      3. Sulfazalazine
      4. Leflunomide
      5. Parenteral gold
      6. Penicillamine
      7. Azathioprine
      8. Cyclosporine A
  3. Biologics
    • TNF alpha blockers
      Etanercept (Enbrel)
      Infliximab (Remicade)
      Adalimumab (Humira)
      Golimumab
      Certolizumab pegol
    • IL-1 receptor blocker
      Anakinra
    • Anti-CD 20 Ab
      Rituximab (MabThera)
    • IL-6-receptor blocker
      Tocilizumab (Actemra)
    • T-cell co-stimulation blocker
      Abatacept (Orencia)
  4. Corticosteroids (local or systemic)
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10
Q

Allopurinol SE

A

Toxic epidermal necrolysis and vasculitis

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11
Q

Pseudo-gout

A

aka Calcium pyrophosphate deposition disease (CPPD)

Affect knees

Acute attack tx same as gout

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12
Q

FK-506

A

Tacrolimus
Calcineurin inhibitor –> decrease IL-2
SE: New onset DM, nephrotoxicity

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13
Q

Drugs that induce lupus

A

Procainamide

Hydralazine

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14
Q

Drugs that exacerbate SLE

A

Sulphonamide

OCP

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15
Q

Joint function history taking

RB

A
  1. Dressing
  2. Washing unaided
  3. Working in kitchen
  4. Climbing stairs
  5. Get in and out of car
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16
Q

Dermatomyositis classical signs

RB

A
  1. Heliotrope rash
  2. Periorbital edema
  3. Photosensitive rash on sun exposed areas, e.g. V neck sign
  4. Gottron’s patches: scaly, violaceous rash on dorsal MCPJ and PIPJ
  5. Signs of underlying malignancy
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17
Q

Sjogren’s syndrome classical signs

RB

A

Wiki: Long-term AI disease in affecting moisture-producing glands

  1. Dry eyes, gritty sensation, may be cx by infection
  2. Positive Schimer’s test: sterile strip of filter paper hooked onto temporal side of lower eyelid; positive if mositure extends <5mm in 5 mins
  3. Dry mouth
  4. Synovitis of MCPJ and PIPJ
  5. Lymphadenopathy
  6. Post-auricular mass, parotid and submandibular swelling
  7. Raynaud’s phenomenon
  8. RA in 50% patients
  9. May transform into lymphoma
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18
Q

Ix findings for Sjogren’s syndrome

RB

A
  1. CBC (low WBC, low lymphocyte)
  2. Increase ESR
  3. Electrolytes, e.g. HypoK due to secondary renal tubular acidosis (type 1; distal)
  4. Increase serum globulin, IgG
  5. Immune markers: ANA, Anti-Ro, Anti-La, RF
  6. Abnormal salivary duct scintigraphy, biopsy
  7. FNAC parotid/submandibular mass
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19
Q

Ix for SLE

A
  1. ANA (Anti-dsDNA, ENA, Antiphospholipid only when ANA is positive or highly suspect)
  2. RF (to rule out RA/Sjogren)
  3. Complements
  4. CBC w/ D
  5. RFT, including urine exam
  6. Serum albumin, globulin
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20
Q

CREST syndrome

A
  • Calcinosis cutis
  • Raynaud’s phenomenon
  • Esophageal dysmotility
  • Sclerodactyly
  • Telangiectasia

Asso w/ Anti-centromere Ab
Spare kidneys

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21
Q

Sjogren Abs

A

Ro(SS-A) and La(SS-B)

Female (9:1)

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22
Q

Xanthine oxidase inhibitor

A
  1. Allopurinol

2. Febuxostat

23
Q

SE of Febuxostat

wiki

A
Nausea
Diarrhea
Arthralgia
Headache
Elevated LFT
Rash

CI: Azathioprine at same time

24
Q

New criteria for RA

ACR/EULAR 2010

A

> =6 out of 10 = positive

Joint involvement
   1 large --> 0
   2-10 large  --> 1
   1-3 small --> 2
   4-10 small  --> 3
   >10 (at least 1 small) --> 5

Serology
RF and ACPA (Anti-Citrullinated protein Ab) normal –> 0
Any low positive (1-3x ULN) –> 2
Any high positive (>3x ULN) –> 3

Duration
<6wk –> 0
>= 6wk –> 1

Acute phase reactant
ESR/CRP normal –> 0
Any abnormal –> 1

Joints: exclude DIP, 1st MTP, 1st CMC
Small joints: MCP, PIP, MTP 2-5, thumb IP, wrist

25
Old criteria for RA | ACR 1987
4 out of 7 1. Morning stiffness >1h 2. Arthritis >= 3 joints 3. Arthritis of hand joints 4. Symmetrical arthritis 5. Rheumatoid nodule 6. RF 7. Radiological changes suggestive of joint erosion
26
Poor prognostic factor for RA What to do? HH
1. High disease activity at onset (>= 18 joints) 2. High baseline joint damage (erosive disease) 3. Persistent high CRP 4. Positive IgM RF or Anti-CCP 5. Positive FH of RA 6. Nodular disease 7. Extra-articular manifestations ==> Early aggressive use of DMARDs
27
Extra-articular manifestation of RA ESNMHLHK (Ed suc nip in MH Les HK)
1. Eye: Scleritis, keratoconjunctivitis (secondary Sjogren) 2. Skin: thinning, ulceration, Rheumatoid nodules 3. Nerve: Peripheral neuropathy, CTS 4. Muscle: wasting 5. Hemat: anemia (of chronic disease, PU due to NSAID/steroid), thrombocytopenia, lymphadenopathy, splenomegaly 6. Lung: Fibrosis, pleurisy, pleural effusion 7. Heart: Pericardial effusion/pericarditis 8. Kidney: AA amyloidosis
28
Rheumatoid nodules
- Non-tender, firm, SC nodules - Usu over bony prominence (but can be everywhere) - Usu on extensor surface, pressure areas (ulnar border of elbow/forearm, knuckles) - May have ulceration --> infection
29
Pre-Tx Ix and screening for RA
1. CBC 2. LRFT 3. CXR 4. Viral hepatitis serology 5. Comorbidities 6. Extra-articular manifestations 7. Infections (TB, hepatitis) 8. Vaccination status 9. Pregnancy and lactation
30
Tx algorithm for RA
Dx, Pre-Tx Ix and screening --> Check for poor prognostic factors Yes --> start combination DMARD +/- steroid No --> MTX monotherapy If poor response after 6m after combination --> MTX + Biologic or Triple therapy If still poor --> taper and stop steroid; then stop biologics; titrate to lowest possible dose of DMARD
31
RA drug classes
1. Analgesics 2. Conventional DMARDs (HCQ, MTX, Sulfasalazine, Leflunomide) 3. Biologics (TNF alpha blocker, IL-1/6 blocker, Anti-CD20, T cell co-stimulation blocker) 4. Corticosteroids
32
SE of Methotrexate MTX | Antimetabolite, folic acid analog
1. Pneumonitis (can lead to fibrosis) 2. Mucositis 3. Intestinal 4. Hepatotoxicity (cirrhosis) 5. BM suppression 6. Lymphoma 7. Alopecia 8. Teratogenicity, not safe for BF
33
SE of Hydroxychloroquine | Anti-malarial; also for RA, SLE
1. Bull's eye maculopathy 2. Corneal deposition 3. Skin pigmentation esp exposed to sunlight 4. Peripheral neuropathy 5. Proximal myopathy 6. Cardiomyopathy
34
Sero-negative Spondyloarthropathy
Inflammatory back pain - Young (<40y) - Insidious onset - Nocturnal pain - Improve with exercise Type [PEAR] 1. Ankylosing spondylitis 2. Psoriatic arthritis 3. Reactive arthritis / Reiter's syndrome 4. Enteropathic arthropathy asso w/ IBD (UC>CD)
35
Ankylosing spondylitis
- Chronic, inflammatory disorder of axial skeleton, affect SIJ and spine - Strong genetic asso w/ HLA-B27 - Usu ascending involvement
36
Psoriatic arthritis
- DIP, peripheral arthritis, spondyloarthritis - 5 patterns 1. Distal arthritis 2. Asymmetrical oligoarthritis 3. Symmetrical polyarthritis (RA-like) - most common in HK 4. Spondyloarthritis (AS-like) 5. Arthritis mutilans (rare) - Seronegative (RF, ACPA neg) - Phase 1: Pain relief: NSAID, local steroid injection - Phase 2: 1st line DMARD, i.e. MTX - Phase 3: Anti-TNF or another DMARD (avoid HCQ) - Phase 4: Biologics
37
Gout
- Dx by urate crystals in synovial fluid / tophus - M; 30-60y/o - Precipitated by Thiazides - Asymptomatic hyperuricemia --> acute gouty arthritis --> chronic gouty arthritis
38
RF for Gout
1. Hyperuricemia 2. FH 3. Male 4. PM women 5. Obesity 6. Alcohol 7. High purine diet 8. Drugs, e.g. Thiazide, Loop diuretic, Pyrazinamide, Cy A 9. Hyper-TG
39
Tx of Gout
Asymptomatic hyperuricemia - Drug not indicated Acute attack - Mild/Moderate: Monotherapy with Oral NSAID or oral colchicine or IA/systemic steroid - Severe polyarticular: combination of NSAID/Steroid + Colchicine - NSAID: Indomethacin; avoid Aspirin If repeated acute attacks (>=2 in 1y), tophus, renal disease, need to take diuretics --> start urate lowering drugs (XOI, Uricosuric) *1-2wk after acute attack subsided - XOI: Allupurinol, Febuxostat - Uricosuric: Probenecid
40
SE of Colchicine
1. Acute: GI upset, esp diarrhea | 2. Chronic: Myelosuppression, renal impairment
41
SLICC Classification criteria 2012 for SLE | AC ON SSRN HLT
>=4 (at least 1 clinical, 1 immunologic) Clinical x11 1. Acute/subacute cutaneous lupus 2. Chronic cutaneous lupus 3. Oral/nasal ulcers 4. Non-scarring alopecia 5. Synovitis (>=2 joints) 6. Serositis 7. Renal 8. Neurologic 9. Hemolytic anemia 10. Leukopenia 11. Thrombocytopenia Immunologic x6 1. ANA 2. Anti-dsDNA 3. Anti-Smith Ab 4. Anti-phospholipid Ab 5. Low complement (C3/C4/CH50) 6. Direct Coombs' test (in absence of hemolytic anemia)
42
Tx for SLE
1. NSAID, Anti-pyrexial drugs 2. Anti-malarials: Chloroquine, Hydroxychloroquine - Useful for arthritis, cutaneous manifestations, constitutional symptoms - May reduce systemic complications - Retinopathy is rare but irreversible - Corneal deposition is more common and reversible 3. Steroids - Very useful but numerous SE for long term use - Variable doses for different manifestations - Small dose for skin rashes, arthritis, serositis - Moderate dose for resistant serositis, hematological abnormalities and class 5 GN - High dose for severe/resistant hematologic changes, diffuse GN, major organ involvement - Whenever steroid is indicated, consider a steroid sparing agent (including anti-malarial agents) or an immunosuppressive drug 4. Immunosuppressives - Azathioprine (hepatotoxicity) - Cyclophosphamide (infertility, hemorrhagic cystitis) - Cy A (renal toxicity) - MTX (hepatotoxicity) - Leflunomide (hepatotoxicity) - MMF (GI related SE, e.g. abd pain, better tolerated) - Tacrolimus [FK-506] * All can cause BM suppression, immunosuppression; potentially carcinogenic, teratogenic 5. Biologic agents - Rituximab (Anti-CD20 monoclonal Ab – off label use) - Belimumab (Anti-BAFF *B cell activating factor)
43
Cause of SOB in SLE
1. RS - Pleural effusion, infective pneumonia (esp on steroid/immunosuppressants), ILD, BOOP, Shrinking lung syndrome (rising diaph, neuromuscular, myopathy) 2. CVS - Pericardial effusion, myocarditis, HT heart disease (HT due to uremia/steroid), IHD (arteritis of coronary a.) 3. Vascular - PE (antiphospholipid syndrome, NS, immobility), pul HT, fluid retention 4. Anemia - Due to chronic disease, part of SLE pancytopenia, hemolytic anemia, due to uremia, GIB due to steroid/NSAID, BM suppression due to immunosuppressants (e.g. Aza)
44
High CRP in SLE
Suspect infection | Could also be: active arthritis, serositis, vasculitis
45
Specific tx strategy for SLE
Conservative - for MSS Sx, Cutaneous lupus, systemic upset, serositis - MSS: NSAID, HCQ, Low dose prednisolone - Cutaneous: Avoid sunlight, topical steroid, HCQ Aggressive - for life-threatening Cx, major organ involvement - Ind: Lupus nephritis, Cerebral lupus, Transverse myelitis, peripheral neuropathy, pneumonitis/pul hemorrhage, severe and refractory serositis, severe hemolysis - Start with high dose steroid, then cytotoxic agents e. g. high dose IV steroid, then oral steroid for maintenance and rapidly tapered - Lupus nephritis now use MMF (instead of Aza) - Cyclophosphamide for more severe and life threatening Cx
46
Poor prognostic factors for SLE
1. Race (Black) 2. Late disease onset 3. Socio-economic status 4. Time btn onset and Dx of SLE 5. Acute disease at presentation 6. Major organ involvement (renal, cerebral) 7. Severe thrombocytopenia
47
Sulfasalazine
For RA, IBD | Breaks into 5-ASA (Mesalazine)
48
Biologics SE
1. Infection: Reactivation of HBV, Latent TB (Etanercept has Lower risk of TB Reactivation) 2. Malignancy 3. Others: Eg. Injection site reaction, Infusion reaction TNF Blockade: Infection, Neutropenia, Demyelinating disease, HF, Malignancy, AI disease
49
Behçet's disease
Classical triad: Mouth ulcer, genital ulcer, uveitis Young (20-40y); affect BVs of all sizes Dx: at least 3 episodes of mouth sores in a year together with at least 2 of: genital sores, eye inflammation, skin sores, a positive skin prick test (Pathergy test)
50
Pathergy test
Behçet's disease
51
Gout and Polycythemia vera
Related due to increase RBC breakdown
52
RA radiological features | Rev
``` Soft tissue swelling Periarticular osteopenia Joint space narrowing Periarticular bony erosions Chronic deformities ```
53
SE of Biologics (anti-TNF) | Rev
Infections, particularly mycobacterial, fungal, viral Reactivation and de novo infection Tumors Non-melanotic skin cancer Heart failure Autoimmunity Development of anti-drug Abs