RHEUM: Ank spond + scleroderma Flashcards
What gene are the Spondyloarthropathies associated with?
HLA - B27
How would a patient with Ankylosing Spondylitis Present?
- Typical pt
- Presenting symptoms
1. Typical pt Young male (teens - mid 30s)
2. Presenting symptoms Gradual development over 3 months Bilateral buttock pain (sacroilliac) lower back pain/ stiffness - wakes pt up in morning- improve with activity Chest wall pain Thoracic pain Often 'flare ups' of symptoms
Examining a pt with Ankylosing Spondylitis
- Early examination vs later on
- What test might you perform and what is normal?
- Early examination - often normal
Later examination
- reduced chest wall expansion
- loss of lumbar lordosis and increased thoracic kyphosis - Schober’s test - to see if decreased lumbar spine range of movement
Mark skin 10cm above and 5cm below the L5 vertebrae. Ask pt to bend forward with straight legs. If distance increases < 20 cm supports diagnosis as restricted movement
Examining a pt with Ankylosing Spondylitis
- Early examination vs later on
- What test might you perform and what is normal?
- Early examination - often normal
Later examination
- reduced chest wall expansion
- loss of lumbar lordosis and increased thoracic kyphosis - Schober’s test - to see if decreased in lumbar spine range of movement
Mark skin 10cm above and 5cm below PSIS. Ask pt to bend forward with straight legs. Distance should increase >20cm = normal test.
What management for a pt with Ankylosing Spondylitis?
Drug:
NSAIDS
TNF inhibitors - e.g. infliximab (Monoclonal AB)
IL-17 inhibitors e.g. Secukinumab (Monoclonal AB) if TNF and NSAIDs don’t work
Non Drug: Physio stop smoking Bisphosphonates if osteoporosis exercise treat complications
Patients with which _S_____ and __S_____ can develop myositis ?
Patients with which __Scleroderma__ and __SLE__ can develop myositis ?
A patient is diagnosed with systemic sclerosis. A medical student sat with you in clinic wants to know what that condition is. Summarise the condition for her
- It is an autoimmune disease which affects multi-systems of the body.
- There is increased activity of fibroblasts (which usually make connective tissue), so you get ABNORMAL growth of connective tissue.
- This causes vascular damage and fibrosis.
What are the two subtypes of Systemic sclerosis (SSc) aka scleroderma?
Limited SSc, diffuse SSc
What other name is given to limited SSc?
CREST syndrome
What does CREST in CREST syndrome (i.e. limited SSc) stand for?
Calcinosis Cutis Raynaud's phenomenon Eoesophageal dysmotility Sclerodactyly Telangiectasia
What is the usual pattern of symptoms in Limited SSc/ crest syndrome?
Have Raynaud’s symptoms first, then get scleroderma (thickening of the skin). After about 10 years of these symptoms, get Pulmonary Arterial HTN.
How is Diffuse scleroderma/SSc characterised?
Sudden onset of skin involvement. Proximal to the elbows and the knees (- so above elbow/nearer shoulder and above knee - so nearer thigh. )
What investigations would you do for pt attending rheumatology clinic with thickening of the skin and PMH of fingers that turn very pale with cold and stress?
They might have CREST syndrome - as have scleroderma and raynauds features. Investigations to do include:
* ECG and ECHO - check for PA HTN, HF, myocarditis,arrythmias
Antibody screen - check for
* positive ANA (+ve in 90% of patients),
* Anti-centromere antibody in limited SSc,
* Scl-70 and anti-RNA polymerase III antibodies in diffuse SSc
* XR of hands - check for calcinosis (the c in crest!)
* CXR, PFT, high resolution CT of lungs - check for pulmonary disease
How is systemic sclerosis managed?
No cure for this condition - may need psych support to deal with this.
Manage the symptoms:
* Raynaud’s symptoms - calcium antagonist, sildenafil, iloprost infusion
* skin thickening - methotrexate and mycophenolate can reduce this.
* HTN crisis - ACEi
* Renal failure (related to scleroderma renal crisis) - ACEi
* Flare ups - prednisolone
* GI symptoms - PPIs
Compare and contrast limited (CREST) and diffuse scleroderma
Limited - gradual onset. Diffuse - sudden onset
Limited - lower mortality. Diffuse - higher mortality
