RHEUM: Ank spond + scleroderma Flashcards Preview

Year 3 Medicine Block > RHEUM: Ank spond + scleroderma > Flashcards

Flashcards in RHEUM: Ank spond + scleroderma Deck (17)
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1
Q

What gene are the Spondyloarthropathies associated with?

A

HLA - B27

2
Q

How would a patient with Ankylosing Spondylitis Present?

  1. Typical pt
  2. Presenting symptoms
A
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
3
Q

Examining a pt with Ankylosing Spondylitis

  1. Early examination vs later on
  2. What test might you perform and what is normal?
A
  1. Early examination - often normal
    Later examination
    - reduced chest wall expansion
    - loss of lumbar lordosis and increased thoracic kyphosis
  2. 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
4
Q

Examining a pt with Ankylosing Spondylitis

  1. Early examination vs later on
  2. What test might you perform and what is normal?
A
  1. Early examination - often normal
    Later examination
    - reduced chest wall expansion
    - loss of lumbar lordosis and increased thoracic kyphosis
  2. 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.
5
Q

What management for a pt with Ankylosing Spondylitis?

A

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
6
Q

Patients with which _S_____ and __S_____ can develop myositis ?

A

Patients with which __Scleroderma__ and __SLE__ can develop myositis ?

7
Q

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

A
  • 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.
8
Q

What are the two subtypes of Systemic sclerosis (SSc) aka scleroderma?

A

Limited SSc, diffuse SSc

9
Q

What other name is given to limited SSc?

A

CREST syndrome

10
Q

What does CREST in CREST syndrome (i.e. limited SSc) stand for?

A
Calcinosis Cutis 
Raynaud's phenomenon 
Eoesophageal dysmotility 
Sclerodactyly 
Telangiectasia
11
Q

What is the usual pattern of symptoms in Limited SSc/ crest syndrome?

A

Have Raynaud’s symptoms first, then get scleroderma (thickening of the skin). After about 10 years of these symptoms, get Pulmonary Arterial HTN.

12
Q

How is Diffuse scleroderma/SSc characterised?

A

Sudden onset of skin involvement. Proximal to the elbows and the knees (- so above elbow/nearer shoulder and above knee - so nearer thigh. )

13
Q

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?

A

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

14
Q

How is systemic sclerosis managed?

A

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

15
Q

Compare and contrast limited (CREST) and diffuse scleroderma

A

Limited - gradual onset. Diffuse - sudden onset

Limited - lower mortality. Diffuse - higher mortality

16
Q

What antibodies is most specific for limited cutaneous systemic sclerosis?

A

Anti-centromere antibodies

Although ANA is positive in 90% of patients with systemic sclerosis, anti-centromere antibodies are the most specific test for limited cutaneous systemic sclerosis

17
Q

What antibodies are associated to diffuse sclerosis?

A

anti scl-70 antibodies

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