Rheumatology Flashcards
What is Felty syndrome?
RA + splenomegaly + neutropenia
SOAP BRAIN MD
Serositis (pleuritis, pericarditis)
Oral ulcers
ANA +
Photosensitivity
Blood disorder - anaemia, leukopenia, thrombocytopenia
Renal disorder (proteinuria, haematuria)
Arthritis
Immunological - APLS, anti-dsDNA, anti-Smith
Neuro - seizures, psychosis
Malar rash
Discoid rash
(Generally need 4 out of 11 features)
SLE.
a) Pathogenesis
b) Risk factors
c) Presentation - general symptoms
d) Diagnostic criteria
e) Joint involvement
f) Typical skin involvement
g) Overlap syndromes
a) Auto-immune connective tissue disease
b) - Chinese/SE Asian/Afro-Caribbean; female
c) Fever, fatigue, malaise, splenomegaly, lymphadenopathy, weight loss
d) SOAP BRAIN MD - need 4/11
e) - Inflammatory small-joint polyarthritis (morning stiffness, improves with use)
- May have reversible deformities (ie. Jaccoud’s arthropathy)
f) (note: all rashes may be photosensitive)
- Malar - butterfly on face; spares naso-labial folds
- Discoid - in sun-exposed areas; scaling
- Livedo reticularis - purple mottled reticular pattern
- Mild alopecia
- Vasculitic rashes
g) Other CTDs:
- vasculitis, myositis, APLS, Sjogren’s, scleroderma, Raynaud’s, etc.
SLE management.
a) Investigations for suspected SLE
b) Non-drug management
c) Drug management
d) In pregnancy/ contraception
a) - Bedside: urine dip (and send for microscopy), ECG, pregnancy test
- Bloods: FBC (pancytopenia), CRP/ESR (raised), U+Es (renal involvement),
- Antibodies: ANA, dsDNA, Smith; cardiolipin and lupus anticoagulant (APLS); histone (drug-induced lupus), ANCA (vasculitis)
- Imaging: joint XR, ?renal USS
- Special tests: complement (C3 and C4 depletion), red cell microscopy, renal biopsy (lupus nephritis)
b) - Patient education: empowerment, self-management (avoid sun exposure), adherence to medication
- Allied health professionals: PT/OT/orthotics (and devices), nurses, counselling, social work, etc.
c) - Symptom control: analgesia, NSAIDs (beware in renal involvement) and steroids (topical, intra-articular, IM, oral)
- Hydroxychloroquine (antimalarial: 1st line in mild SLE)
- Cyclophosphamide and mycophenolate (used in life-threatening disease- eg. vasculitis, nephritis)
- Other DMARDs
- Biologics: eg. rituximab or belimumab (B-cell inhibitors)
d) - COCP contraindicated if: Hx of migraine, HTN, VTE or anticardiolipin antibody positive
- Pregnancy should be delayed until disease is quiescent for > 6 months
- Complications of SLE/APLS in pregnancy: worsens SLE, foetal loss and abnormality, maternal VTE
- If APLS in pregnancy - LMWH and aspirin
Drug-induced lupus.
a) Common causes
b) Clinical features (vs. SLE)
c) Antibody associated
d) Management
a) Procainamide, chlorpromazine, methyldopa, hydralazine, isoniazid, d-penicillamine, anti-TNF drugs
b) Arthralgia most commonly (90%);
less end-organ damage than SLE; renal and central nervous system (CNS) involvement is uncommon
c) Anti-histone
d) Stop the offending drug and it should resolve
Antiphospholipid syndrome (APLS).
a) 2 types and complications
b) Antibodies associated
c) Causes
d) Who should be screened?
e) Management
a) - Obstetric: recurrent miscarriage, stillbirth, IUGR, pre-eclampsia, pre-term birth, etc.
- Thrombotic: recurrent arterial/venous thrombosis
b) Anti-cardiolipin, anti-b2-glycoprotein I antibody
(+ lupus anticoagulant)
c) - SLE (30% have APL antibodies; not all have APLS)
- Other AI disease (RA, Bechet’s, seronegative SpA, etc.)
d) Thrombotic: < 50 years old with ischaemic stroke
- Obstetric: recurrent pregnancy loss (≥ 3)
e) - Conservative: healthy lifestyle (smoking, diet, etc.), pre-pregnancy counselling, manage other RFs
- APLS in pregnancy - LMWH and low-dose aspirin
- Non-pregnant: warfarin (switch to LMWH and aspirin if they become pregnant)
Rheumatoid arthritis.
a) What is it?
b) Risk factors
c) Pattern of arthritis (site, number, features)
d) Tenosynovitis in RA - explain
e) Investigations in suspected RA
f) Give some poor prognostic factors in RA
a) Multisystem autoimmune disease, primarily causing an inflammatory arthritis (synovitis)
b) - Female, smoking, FHx, other AI disease
- Presence of RhF/ anti-CCP antibodies; HLA DR1 + DR4
c) - Usually symmetrical peripheral small-joint polyarthritis
- MCPJ, wrist, PIPJ, MTPJ; also elbows, knees, shoulders
- Inflammatory (red, hot, swollen, stiffness > 30 mins in the morning, improves with activity)
d) Tenosynovitis may be first sign - painful tendons and limited function, but joints non-inflamed (no synovitis)
e) - Bloods: FBC, CRP/ESR, uric acid, LFT/U+E (baseline)
- Antibodies: RhF (+/- anti-CCP), ?ANA, ?HLA-B27, etc.
- Imaging: XR hands and feet (peri-articular osteopenia, erosions, loss of joint space, soft tissue swelling)
- Aspirate any hot swollen joints - ?septic, ?gout
f) - Younger age of onset (< 30)
- Double sero-positive (RhF+ and anti-CCP+)
- Delay in starting DMARD (> 3 months)
- Persistently active disease
- Extra-articular features
- Early joint erosions on XR
RA: extra-articular features
- head to toe
- Neuro: peripheral nerve entrapment (eg. CTS), atlanto-axial subluxation (+ cervical myelopathy), polyneuropathy, mononeuritis multiplex.
- Eyes: secondary Sjögren’s syndrome, scleritis and episcleritis.
- Resp: pleural involvement, pulmonary fibrosis, Caplan’s syndrome
- CV: ischaemic heart disease, pericarditis, endocarditis, vasculitis, Raynaud’s
- Kidneys: analgesic nephropathy, amyloidosis (rare).
- Liver: mild hepatomegaly and abnormal LFTs (more likely methotrexate-related)
- Skin: subcutaneous nodules, rashes, nail fold infarcts, ulcers (especially in vasculitis/ Felty syndrome)
- Haem: anaemia, Felty syndrome (neutropenia, splenomegaly), increased infections
Other: constitutional symptoms, thyroid disorders, osteoporosis, depression
RA: classic signs on examination
- by site
- Elbows: nodules, fixed flexion
- Wrists: subluxation, fusion (if surgical - evident scar), deviation (ulnar or radial)
- Dorsal: guttering, loss of furrowing between knuckles
- Palmar: palmar erythema
- Thumb: Z-deformity
- Fingers: MCPJ subluxation (+/- tendon ruptures), MCPJ ulnar deviation, Boutonniere’s, swan-neck
Rheumatoid nodules
a) Consistency
b) Common sites
c) What are they?
a) Rubber-like
b) - Subcutaneous: olecranon, finger joints
- Other: lung, heart, vocal cords
c) Granulomas
RA management.
a) Indications for urgent rheumatology referral
b) Monitoring disease activity
c) Other things to check at annual review
d) Non-drug
e) Drugs
f) Surgery - indications (including urgent)
g) Surgery - types
a) - The small joints of the hands or feet are affected.
- > 1 joint is affected.
- Delay > 3 months between onset and presentation
(note: refer even if normal CRP/ESR and negative RhF)
b) Disease activity score (DAS-28):
- No. of tender/swollen joints (shoulders, elbows, wrists, MCPJ, PIPJ, knees)
- ESR
- Patient ‘assessment of global health’
c) - Assess compliance and for any complications
- Assess for: HTN (BP), coronary heart disease (QRISK), osteoporosis (DEXA/FRAX) and depression (PHQ-9); start therapies if necessary
d) Non-drug.
- MDT support: GP, rheumatologist, nurse specialist, PT, OT, dietician, podiatrist, pharmacist, social worker
- Devices (eg. walking aids, tap-turners, orthoses)
- Patient education and self-management
- Exercise
e) Drug.
- NSAIDs/ steroids - short-term
- Other analgesia
- DMARDs - methotrexate, sulfasalazine
- Biologics (after 2x trials of 6m of DMARDs fail to control): anti-TNF, CD-20 inhibitor, IL-6 inhibitor
f) - Urgent: cervical myelopathy, septic arthritis
- Other: worsening function or persisting pain, tendon rupture/imminent rupture, nerve entrapment (eg. CTS)
g) - Nerve decompression / cord decompression
- Fusion of joints
- Replacement of joints
- Tendon repairs/ tendon transfer
Methotrexate.
a) MoA
b) Side effects
c) Monitoring
d) How to take?
a) Folate antagonist; cytotoxic (also chemotherapeutic)
b) Nausea (usually less if given SC), mouth ulcers
- More severe: hepatotoxicity, agranulocytosis, TERATOGENIC
c) - FBC, U+Es, LFTs (plus intermittent CRP/ESR)
- Women must be on contraception; pregnancy tests
d) Once per week (oral or SC)
- Give folic acid on day(s) not taking methotrexate (to reduce the side effects
Biologics:
- 4 main classes
- give examples
- give the diseases its used in
- contraindications/cautions
TNF-inhibitors.
- Etanercept, infliximab, adalimumab, certolizumab
- Used in: RA, AS, IBD (inflix), PsA
Interleukin inhibitors.
- IL-6: tocilizumab (used in: JIA, RA)
- IL-12/23: ustekinumab (used in PsA)
- IL-17: secukinumab (used in PsA)
B-cell (CD-20) inhibitors.
- Rituximab, belimumab
- Used in: NH lymphoma (B-cell lymphoma), RA, SLE
T-cell inhibitors.
- Abatacept
- Used in: RA
Biologics side effects.
- Infection risk
- Can activate latent TB or Hep B
- Antibodies against drug produced - effect may wear off over time
Ankylosing spondylitis.
a) What is it?
b) Causes/risk factors
c) Presentation
d) Possible signs o/e
e) Diagnostic criteria
f) Other investigations
a) Chronic seronegative spondyloarthropathy, primarily involving the axial skeleton (ie. sacroiliitis and spondylitis)
b) HLA-B27+, FHx, male, younger age (20 - 30)
c) - Inflammatory back/buttock pain
- Insidious onset/ relapsing-remitting pattern
- Systemic symptoms (eg. fever, weight loss, fatigue)
- Extra-articular symptoms
d) - Inspection - cervical kyphosis and loss of lumbar lordosis (question mark posture), buttock atrophy
- Palpation - tender sacro-iliac joints, tender entheses
- Movement - restricted lumbar flexion (< 5cm increase in Schober’s test indicates restriction); reduced chest
expansion
e) - At least one clinical finding of AS (inflammatory back pain, reduced lumbar flexion or chest expansion)
- Radiological findings (sacro-iliitis, etc.)
f) - Bedside: pulmonary function tests
- Bloods: FBC, ESR/CRP, antibodies (HLA-B27, RhF, ANA)
- Imaging: XR +/- MRI
- For complications (if suspected): DEXA (osteoporosis), ECHO (AR), CXR (fibrosis)
g) - Conservative: physiotherapy, hydrotherapy, encourage activity, monitor for complications, spinal extension and deep breathing exercises
- NSAIDs for symptom control
- Anti-TNF (etanercept or adulimumab; NOT infliximab) for severe AS not controlled by NSAIDs (note: classic DMARDs like methotrexate/5-ASAs don’t work in AS)
- Surgery if required
Features of inflammatory pain.
- Red hot, swollen, tender joints
- Stiffness > 30 mins in the morning
- Improves with activity
- Worse/not relieved by rest
- If back pain - insidious onset at age < 40 years
