Rheumatology Flashcards
4 main presentations of lupus
Discoid lupus - scarring, depigmentation, 80% above neck
Subcutaneous lupus (SCLE) - photosensitive, non-scarring rash, cape distribution
Systemic lupus - low risk: malar rash, patchy alopecia
Systemic lupus - high risk: more systemic involvement
Renal and CSN lupus ab
Anti-SM
3 specific SLE Abs
DsDNA
Anti-Sm
SLE - monitor disease activity
Anti-dsDNA - lupus nephritis
C3/C4
ESR
anti-Ro
Subacute cutaenous lupus, neonatal heart block
Anti-U1RNP
MCTD
Anti-dendritic cell biologic
Litifilimab
Anti-BAFF/BlyS (b cell activating/b lymphocyte)
Belimumab
Biologic blocking IFN 1 signalling
Anifrolumab
SLE treatment principals
All patients get hcq
Mild - usually hcq alone
Moderate - Pred, possibly aza or MTX as steroid sparing
Severe = renal and cns
- Induction - Pred and Cyclophosphamide/MMF
- Maintenance - MMF or AZA, biologics
Lupus nephritis Rx
Steroid pulse + MMF/Cyclo
Rescue Ritux
APLS anticoagulation in pregnancy
LMWH and aspirin
Anti-histone
Drug induce lupus
Common drugs causing lupus
Anti-TNF
Minocycline
PPIs
Hydralazine
Rheumatoid - HLA association
HLA-DRB1 04
Ab in < 1% autoimmune disease
Anti dfs70
Felty syndrome triad
RA + neutropenia + splenomegaly
RA - what cytokine causes erosions
TNF
Major cytokines in RA
Erosions - TNF
Inflammation - IL-1, IL-6, IL-17, TNF
Eye manifestations RA
Scleritis, episcleritis (whole or segmental), peripheral ulcerative keratitis
Common neuro finding of RA
CTS
Most common haem finding of RA
Anaemia
Diagnostic criteria for RA
Inflammatory arthritis (usually peripheral) > 3 joints
Positive RF and/or anti-CCP
Elevated ESR/CRP
Duration > 6 weeks
No other CTD
Poor prognostic features for RA
Early erosive XR changes
Extra-articular features
Sustained CRP elevation
CCP positivity
Which DMARD does not slow radiographical progression RA
HCQ
Treatment ladder RA
MTX 1st
Triple therapy 2nd - LFL,SLZ, MTX
Add biologics third
Add JAKi 4th
MTX A/E
Myelosuppression
Nephrotoxicity - need urinary alkalinisation and leucovorin rescue
Hepatotoxicity
A/E HCQ
Retinopathy
Leflunomide A/E
Antidote -
Myelosuppresion
Teratogenic
Antidote - cholestyramine
SZL A/E
Myelosuppression, high risk in G6PD
Sulfur drug - rash
TNFi A/E
reactiviation opportunistic infections
Cardiac failure
Reversible lupus
A/E of JAK inhibitors
Increased Zoster infection
Increased overall cancer risk
Increased CVS risk
IMS okay in pregnancy
PASH TNF
Pred, Aza, SZS, HCQ, TNF
Dose of pred associated with worse outcomes/mortality
Daily dose > 8mg (aim to wean below this as safely possible)
Most common eye sign of RA
Keratoconjunctivitis sicca
Eye signs of ra in order of severity
Keratoconjunctivitis sicca -dry, gritty eyes
Episcleritis - red sclera
Scleritis - painful red sclera (can be scleromalacia perforans or peripehral ulcerative keratitis, needs opthal
PR3
GPA
MPO
EGPA 65%
MPA 80%
ANCA vasculitis associated with RPGN
MPA
ANCA vasculitis associated with trachela stenosis
GPA
Management of ANCA vasculitis
GC + Cyclophosphamide
Ritux as salvage
Urticaria > 72 hours
Antic1q Ab
Urticarial vasculitis
Investigation of PAN
Biopsy - segmental necrotising vasculitis medium vessels
CT angiographa
Features of Behcet’s
Oral ulcers
2 of
- genital ulcers
- SKin lesions
- Pathergy - skin sensitivity to trauma
- Ocular inflammation
Visual symptoms and GCA Rx
Methylpred
Complications of GCA
Vision loss
Aortic dissection
Anti-topoisomerase - other name?
Anti-Scl70
RNA polymerase III?
Diffuse SSc
- Renal
- malignancy
Anti-centromere
Limited SSc
- Pulm HTN
SSc renal crisis Rx
ACEi
SSc ILD Rx
Cyclophosphamide
New evidence for nintedanib
Resorption of terminal tuft
Acro-osteolysis = SSc (diffuse)
Leading cause of death SSc
ILD
Probenecid
- CI
Nephrolithiasis
Non gout drugs with:
-ULT
- Urate elevating properites
Losartan - lowers urate
Aspirin - increases urate
Anti-Jo
Anti-synthetase syndrome
Anti Pl 7
Anti Pl 12
Anti synthetase syndrome
Anti Mi
Dermaomyositis
MDA-5
MDA-5 associated ILD
Cancer associated Abs
Anti TIF1
Anti NXP2
Anti Pm-Scl
Scleroderma overalp myositis
Anti-Ku
Scleroderma overlap myositis
Ro60 vs Ro52
Ro60 - Subacute lupus, Sjogren’s
Ro52 - scleroderma overlap
Spontaneous activity in EMG
- Signs
- Significance
Fibrilliation, positive waves, complex repetitive discharges
Significance - chronic partial/full nerve lesions
Motor unit recruitment on EM
- Amplitude
- Early recruitment?
Amplitude
- Low - primary muscle disease
- High - chronic partial dennervation with re-innervation
Early recruitment = primary muscle disease
MND EMG findings
Spontaneous activity with chronic partial dennervation all 4 limbs
Characteristic pathology of Sjogren’s
Lymphocyte infiltration of exocrine glands
Muscle biopsy - necrosis but no inflammation
Necrotising myopathy - Immune/statin etc.
Managemene of FMF
1st - colchicine
2nd line - Anakinra IL-1 inhibition
Have U1RNP and Anti-Sm/Anti-dsDNA
Lupus - lupus trumps MCTD
What is RF?
Found in
Antibody against Fc portion of IgG.
Found in Sjogren’s, MCTD, cryoglobuinaemia
ANCA positive but with mismatch of pattern to antibody - cause?
Cocaine/levamisole
Main lifestyle RF for RA
Smoking
A/E of tocilizumab
LFT
Cholesterolaemia
Staining pattern for ANA positive, dsDNA psotive but ENA negative
Homogenous (nucleus stains one colour)
Most prevalent CNS complication lupus
Cognitive dysfunction = “brain fog”
Difference between Jaccoud’s and RA
Jaccound (in SLE) shows no erosive arthritis
RA will show erosive arthritis
Faber test for hip pathology
Flexion, external rotation and abduction
90% sensitive and specific
Most common cause of coronary artery aneurysm
Kawasaki disease
Negative birefringence
Needle shaped
Uric acid/gout
Positive birefringence
Rhomboid
CPPD (Ca)
Central necrosis surrounded by palisading macrophages
Rheumatoid nodule
Cause of gout from increased uric acid synthesis.
- which enyme deficiency?
Hypoxanthine-guanine phosphoribosyl transferase (HGPRTase)
Necrotising myopathy antibodies
Srp
Hmg coa reductase
Ab most associated with myocarditis in myositis
SRP
IgG antiC1nA
Inclusion body myositis