Rheum quickfire Flashcards
Limited vs diffuse scleroderma
- skin, joint, lung, renal disease
- antibodies
- treatments
Limited scleroderma (CREST):
- Skin thickening limited to below elbows and knees (+/- face or neck)
- Calcinosis more prominent, Raynaud’s, Esophageal dysmotility (Rx: prokinetic), Scleroderma, Telangiectasia
- Arthralgia/arthritis more common
- Lungs - pulmonary HTN, but fibrosis less common
- May cause dysmotility in the small/large intestines also due to defective peristalsis, and 40% have SIBO
- Do NOT have renal involvement
- Antibodies: anti-centromere*, 50% have no antibodies
Diffuse scleroderma:
- Skin thickening including above knees/elbows
- Lungs - fibrosis more common (treat with cyclophosphamide or mycophenolate), do NOT get pulmonary HTN
- Renal crisis possible (histologically causes thrombotic micro-angiopathy, glomerulus will be normal)
- Antibodies: anti-Scl-70** (anti-topoisomerase), anti-RNA polymerase 3***, 50% have no antibodies
Mnemonics:
Centromere = Crest
**Scl = Scleroderma + Scarring of the lung (pulmonary fibrosis)
**RNA P3 = Renal crisis
Treatments for scleroderma:
- Not any good DMDs
- ACE-I to prevent (and treat) renal crises
- Bosentan (endothelin antagonists), prostanoids (iloprost) or PDE5I (sildenafil) can be used to treat PAH
Drug-induced lupus
a) Common causes
b) Presentation vs lupus
c) Lab findings
d) Management
a) Procainamide, hydralazine, isoniazid, quinidine, D-penicillamine, TNF-inhibitors, antipsychotics, anticonvulsants
b) - Generally a milder form of SLE
- may still cause nephritis, percarditis, etc.
c) - Anti-histone antibodies common
- Positive ANA in most cases
- Negative anti-dsDNA in most cases
d) - Remove offending drug
- NSAIDs, steroids, chloroquine, etc if needed (e.g. nephritis, skin inflammation)
Ank spond.
a) Diagnostic criteria
b) Other clinical features (7 As)
c) Most specific radiological finding, best early finding and later radiological findings
d) HLA-B27 positivity rate in AS vs general population (what about in black population?)
e) Management
a) Sacro-iliitis on XR/MRI, plus one out of:
- Inflammatory lower back pain >3 months
- Limitation in lumbar spine forward and lateral flexion (often with retention of lumbar lordosis during flexion)
- Limitation in chest expansion (often due to costochondritis)
b) Arthritis (peripheral arthritis in 25%), apical lung fibrosis, Aortic regurgitation, Achilles tendonitis, Anterior uveitis, Amyloidosis (renal), Atlanto-axial subluxation
c) - Most specific: Bilateral sacroiliac erosions
- Earliest: subchrondral sclerosis on iliac side of sacro-iliac joints
- Later: syndesomophytes, disc sclerosis, vertebral fusion, bamboo spine
d) 90% in AS, 8% in general pop
- Very low prevalence in black population, so in a black patient with inflammatory lower back pain, consider other diagnoses (e.g. HIV)
e) - TNF-inhibitors e.g. etanercept, infliximab (note - if undergoing surgery, these should be stopped 2-4 weeks in advance)
- NSAIDs for symptom control
Sjogren’s
a) Primary vs secondary
b) Investigation findings
c) Presentation
d) Management
a) Primary - more likely to have anti-Ro antibodies
b) FBC usually normal
Rheumatoid factor almost always positive
ANA usually positive
Anti-Ro (70%) and Anti-La (70%)*
Schirmer test positive
Oral labial biopsy - lymphocytic infiltrates of minor salivary glands
*Risk of fetal hydrops and neonatal heart block if these are present during pregnancy
c) - Dry mouth, dry eyes
- Dysphagia
- Dry cough (xerotrachia), ILD, pneumonitis
- Raynaud’s
- Arthralgia
d) Symptomatic:
Artificial tears and saliva
Pilocarpine (pro-cholinergic)
DMD:
NSAIDs
Steroids
Hydroxychloroquine, methotrexate
Cyclophosphamide, Rituximab
Paget’s
a) Clinical features
b) % inherited
c) Investigations and diagnostic findings
d) Management
e) Complication in 1%
a) OA hip, bone pain, abnormal bone remodelling (lumbar spine, femur, pelvis, skull, ear), sensorineural deafness, high output cardiac failure
- most common over 55 years old
b) 40% autosomal dominant
c) - Raised Alk Phos with normal Calcium and Phosphate
- Limited skeletal survey - evidence of lytic and sclerotic lesions
- Bone biopsy (not usually required): multinucleated osteoclasts
d) Bisphosphonates
e) Osteosarcoma
Mixed connective tissue disorder
a) What is it?
b) Antibody associated
c) Characteristic clinical feature
a) - Overlap syndrome of multiple rheumatological conditions, e.g. RA, SLE, systemic sclerosis and polymyositis
- Many also have Sjogren’s
b) Anti-U1 ribonucleoprotein (Anti-U1RNP)
c) “Mechanics hands”
- Dry, fissured, hyper-keratotic
Seronegative arthritis
a) Meaning
b) 10% reactive arthritis have what skin condition on soles of feet?
c) Gonococcal arthritis features
d) Other causes of reactive arthritis
e) Enteropathic arthritis vs. IBD with inflammatory arthritis
a) Rheumatoid factor negative
b) Keratoderma blenorrhagica - intense scaling of soles of feet (psoriasiform)
c) Dermatitis-polyarthritis-tenosynovitis syndrome: Swelling near the joints/tendons, with vesiculopustular rash.
May also have fever, urethritis, uveitis
d) Chlamydia, gastroenteritis (campylobacter, salmonella, shigella, yersinia)
e) Enteropathic arthritis - joint flares occur in conjunction with IBD flare
APLS
a) Causes
b) Antibodies
c) Presentation
d) Management
a) Primary
Secondary - SLE, HIV, Hep C, syphilis, drugs (e.g. chlorpromazine)
b) - Anti-cardiolipin
- Anti-beta-2-glycoprotein (also positive in 25% Behcet’s)
- Lupus anticoagulant (50% prevalence in SLE)
c) - Recurrent miscarriage
- DVT/PE, renal vein thrombosis, arterial thrombosis
- Thrombocytopenia, paradoxically raised APTT
- Livedo reticularis
- Other: epilepsy, migraine
d) Pregnancy (especially if previous VTE or miscarriage) - heparin and aspirin to 34 weeks
VTE not in pregnancy:
- anticoagulation for 6 months (INR 2.5)
- recurrent or arterial thrombosis - consider lifelong and higher INR
Psoriasis treatments
a) Good for joint but not skin involvement
b) Good for both skin and joint
c) When 2 DMARDs don’t work, what is the next stage?
a) Sulfasalazine
b) Methotrexate
c) TNF-inhibitors
Behcet’s disease
a) Epidemiology
b) Presentation
c) Skin test to assist in diagnosis
d) Lab findings
e) Management
a) 20s-30s, Mediterranean (Turkey esp) and Japan
b) Triad of ulceration, skin and eye disease:
- Recurrent oral and genital ulcers (DDx: herpes)
- Skin - erythema nodosum, acne like rash, petechiae/purpurae, PATHERGY (pathognomonic sign - pustule at site of venepuncture/trauma)
- Eyes - uveitis
- Other features include neuro (headache, movement disorder, MS-like, venous sinus thrombosis, stroke, cognitive deficit), cardiovascular (vasculitis, thrombosis, phlebitis), arthritis, abdo pain/ diarrhoea/ ulceration, rarely GN
c) Skin prick (pathergy) test - inflammatory healing - 2mm pustule in 24-48 hours following skin prick
d) - FBC - normocytic anaemia common
- APLS antibodies in 25%
- HLA-B51 positive in 20%
e) - Skin: Topical steroids
- Systemic: steroids, DMARDs, biologics
Palindromic rheumatism
a) What is it?
b) Investigations and management
a) Episodes of rheumatism with asymptomatic intervals, often affecting larger joints, episodes lasting around 1-3 days and responding well to NSAIDs
b) - Check for anti-CCP/Rheumatoid factor - presence predicts higher risk of progression to RA
- Open access to rheumatology clinic - during episode, aspirate joint to rule out crystal arthropathy
- NSAIDs are 1st line
- Steroids
- DMARDs - hydroxychloroquine preferred
Polymyositis
a) Epidemiology
b) Cause
c) Presentation (vs PMR and vs IBM)
d) Lab findings
e) EMG findings
f) Treatment
a) 30-60 years
b) May be associated with malignancy or connective tissue disease
c) - Proximal muscle weakness and wasting with distal muscle sparing, (vs. PMR which causes proximal muscle pain + stiffness in those >50, and IBM which causes distal muscle involvement esp finger flexors in those >50)
- Dysphagia (pharyngeal weakness, oesophageal spasm)
- Small joint arthralgia
- Only 1/3 have pain
- Dermatomyositis - causes characteristic rashes (e.g. Gottron’s papules, heliotrope rash) and 1/3 have Ca
- Interstitial lung disease may be present (vs IBM which rarely causes lung involvement)
d) - Normocytic anaemia
- Raised CK
- Positive ANA, RhF and anti-Jo1 (40%)
- Confirm with EMG and muscle biopsy
e) Polyphasic motor potentials, spontaneous fibrillations, high frequency repetitive discharges
f) Steroids
- If anti-Jo 1 positive, will need long term immunosuppression
SLE
a) Markers of disease activity
b) CRP levels
c) Management of skin/arthralgia vs synovitis vs severe systemic involvement
d) ANA level to consider diagnosis
e) SLE and CVD
f) Rash and other skin features
g) SOAP BRAIN MD
a) - Clinical - fever, joint pain, etc.
- Biochemical - low complement, raised ESR, raised dsDNA, falling blood counts
(note - ANA has no bearing on disease activity but higher levels generally indicate more severe SLE)
b) Typically normal unless there is:
- Infection
- Serositis
Classic condition where ESR is high but CRP is normal
c) - Skin rash/ arthralgia - hydroxychloroquine
- Arthritis (synovitis/swelling) - methotrexate
- Severe systemic (renal, neuro, heart, lung, eyes) - steroids +/- cyclophosphamide
d) In presence of symptoms, ANA titre present at dilution 1:80 or greater is significant
e) - Women aged 40 with SLE are 50x more likely to have CVD event compared to controls without SLE
- Secondary prevention should be used
- If event manifests at young age (e.g. MI / CVA/ VTE at age 40), should screen for secondary APLS
f) - Butterfly rash - involves nasal ridge (unlike rosacea)
- Photosensitive rash, discoid rash, livedo reticularis, scarring alopecia
g) Serositis
Oral ulcers
Arthritis
Photosensitivity
Blood disorders
Renal involvement
ANA+
Immunological disorders
Neurological disorders - CNS (confusion, stroke, seizures, psychosis, myelopathy), PNS (neuropathy), Autonomic
Malar rash
Discoid rash
Rituximab
a) Risk of reactivation of which virus?
b) Screening test and treatment if positive
a) Hep B
b) Hep B core antibodies - treat with lamivudine if positive
Hydroxychloroquine
a) Does it need to be stopped in acute infection?
a) No, as not an immunosuppressant per se
