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8 Cards in this Set

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Definition of systemic sclerosis

(aka scleroderma)


An autoimmune disorder of connective tissue that is characterised by vasomotor disturbances and fibrosis & atrophy of the skin, subcutaneous tissue, muscles and internal organs

Epidemiology of systemic sclerosis

- 3x more common in females


- commonly present between the ages of 30-50yrs

2

Types of systemic sclerosis

- limited: , distal to elbow/knee, plus face, 70%


- diffuse: diffuse skin and organ involvement, 30%

2

Features of limited systemic sclerosis

Includes CREST syndrome


- calcinosis


- Raynaud's


- esophageal & gut dysmotility: GORD


- sclerodactyly: lost mobility


- telangiectasia




- skin involvement (confined to face, hands & feet): telangiectasia, microstomia (small mouth), beak-shaped nose


- pumonary HTN: in 15%

7

Features of diffuse systemic sclerosis

- GIT: GORD, aspiration, dysphagia, anal incontinence


- lung: fibrosis, pulmonary HTN


- cardiac: arrhythmias, conduction defects


- renal: acute hypertensive crisis (most common cause of death)

4

Blood tests in systemic sclerosis

- FBC: anaemia


- U&Es: renal impairment


- anti-centromere: more common in limited


- anti-Scl-70 (aka topoisomerase): diffuse


- anti-RNA polymerase 1, 2, 3: diffuse

5

Investigations in systemic sclerosis

- CXR: cardiomegaly, bibasal fibrosis


- barium swallow: impaired oesophageal motility


- high resolution CT


- echo (+ECG): pulmonary HTN

4

Management of systemic sclerosis by system

- contractures: exercise & skin lubricants


- Raynaud's: hand warmers, Ca channel blockers, ACE inhibitors, IV prostacyclin


- renal: BP control, ARBs


- oesophageal: PPIs, prokinetics (metoclopramide)


- pulmonary HTN: sildenafil, bosentan