Pre PACES Podcast #70 Systemic sclerosis
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Sep 24, 2023 Dr John Pauling, consultant rheumatologist specializing in systemic sclerosis and Raynaud's, shares clinical insight. He defines systemic sclerosis and its triad. He walks through recognizing Raynaud's, skin changes from puffy fingers to sclerodactyly, and GI reflux clues. He covers organ screening, key antibodies and nailfold checks, investigation pathways, treatments from vasodilators to antifibrotics, and multidisciplinary care.
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Skin Extent Defines Subsets And Prognosis
- Extent of skin involvement determines subset and prognosis: limited cutaneous versus diffuse cutaneous disease.
- Limited affects fingers/hands/face (CREST features, anticentromere); diffuse involves limbs/trunk with higher fibrotic risk.
Systematic Review Focused On High Risk Organs
- Ask targeted system reviews: cardiac syncope/angina, respiratory exertional dyspnea/dry cough, GI bloating/diarrhea, and monitor kidney function and blood pressure.
- Pulmonary fibrosis and pulmonary arterial hypertension are major causes of mortality—screen proactively.
Detailed Hand Exam And Nailfold Assessment Are High Yield
- Examine hands for coolness, sclerodactyly, digital ulcers, pitting, calcinosis, telangiectasia and perform nailfold capillaroscopy.
- Demonstrate findings to the examiner (e.g., ask to inspect face/mouth opening) to score in PACES.