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8 Cards in this Set
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- 3rd side (hint)
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 |
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Epidemiology of systemic sclerosis |
- 3x more common in females - commonly present between the ages of 30-50yrs |
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Types of systemic sclerosis |
- limited: , distal to elbow/knee, plus face, 70% - diffuse: diffuse skin and organ involvement, 30% |
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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% |
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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) |
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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 |
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Investigations in systemic sclerosis |
- CXR: cardiomegaly, bibasal fibrosis - barium swallow: impaired oesophageal motility - high resolution CT - echo (+ECG): pulmonary HTN |
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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 |
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