MSK: Systemic Sclerosis: Free MSRA Podcast

🎧 MSRA Podcast Deep Dive: Systemic Sclerosis (Scleroderma)The rapid-fire essentials for your MSRA revision🧠 Key Learning PointsDefinition• Systemic sclerosis (SSc) = chronic autoimmune connective tissue disease, excessive collagen causes fibrosis of skin & internal organs (lungs, GI, heart, kidneys, vessels).• 2 main types: - Limited cutaneous (lcSSc/CREST) - Diffuse cutaneous (dcSSc)Causes & Risk Factors• Unknown cause: Genetics (family/HLA), environment (silica, solvents), immune triggers• Female (4:1), viral triggers, some drugs/chemicals• Risk: Raynaud’s, family history, African descentPathophysiology• Overactive fibroblasts → collagen → tissue fibrosis• Microvascular damage: Raynaud’s, digital ulcers• Autoantibodies: ANA, anti-centromere, anti-Scl-70, anti-RNA pol IIISymptoms & Mnemonics• Raynaud’s phenomenon (early clue)• Skin: sclerodactyly, thickening, pigment changes, telangiectasia, calcinosis - CREST mnemonic for limited form:  - Calcinosis  - Raynaud’s  - Esophageal dysmotility  - Sclerodactyly  - Telangiectasia• MSK: joint pain, contractures• GI: reflux, dysphagia, bloating, malabsorption - Mnemonic: “Heartburn Over Gastric Reduced Constipation Obstruction Anorectal”• Lungs: fibrosis, pulmonary hypertension - Mnemonic: “Fibrosis, Hypertension, Dyspnoea, Cough, Crackles”• Heart: arrhythmia, myocarditis, pericarditis - Mnemonic: “Micro-accelerated MYO pericarditis arrhythmia endo PAH”• Kidneys: renal crisis (life-threatening hypertension/AKI) - Mnemonic: “Glomerulonephritis, Reduced function, Crisis, Hypertension, Failure”• GU: erectile dysfunction, dyspareuniaDiagnosis & Investigations• Autoantibodies: - ANA (90%) - Anti-centromere (limited/PAH) - Anti-Scl-70 (diffuse/lung, kidney risk) - Anti-RNA pol III (diffuse/renal crisis)• Bloods: FBC, U&E, CRP/ESR• Imaging: hand x-ray (calcinosis), HRCT chest (ILD), echo/ECG• Nailfold capillaroscopy (Raynaud’s, microvascular changes)• Criteria: 2013 ACR/EULAR classification (skin thickening proximal to MCPs is key)Management• MDT approach: Rheumatology, respiratory, cardiac, renal, physio/OT• General: patient education, skin care, BP checks, smoking cessation• Raynaud’s: keep warm, CCBs (nifedipine first-line)• GI: PPIs, prokinetics, antibiotics for SIBO• Immunosuppression: methotrexate, mycophenolate, cyclophosphamide (for progressive disease/ILD)• PAH: endothelin receptor antagonists, PDE5 inhibitors, prostacyclin analogues• Renal crisis: urgent ACE inhibitors• Other: treat digital ulcers, calcinosis, refer for organ-specific care• Pregnancy: pre-pregnancy counselling, avoid teratogens, careful monitoringComplications & Prognosis• Pulmonary fibrosis, PAH, renal crisis (major mortality risks)• GI dysmotility, severe Raynaud’s/ulcers, cardiac disease, depression, increased cancer risk• Prognosis: variable; 5-year survival ≈ 75%. Early diagnosis/intervention improves outcomes.Essential Revision Links📝 Revision Notes:https://www.passthemsra.com/topic/systemic-sclerosis-revision-notes/💬 Accordion Q&A:https://www.passthemsra.com/topic/systemic-sclerosis-accordion-qa-notes/🧠 Flashcards:https://www.passthemsra.com/topic/systemic-sclerosis-flashcards/🚀 Rapid Quiz:https://www.passthemsra.com/topic/systemic-sclerosis-rapid-quiz/🎓 Full Quiz:https://www.passthemsra.com/quizzes/systemic-sclerosis/🎓 Full Course:https://www.passthemsra.com/courses/msk-for-the-msra/#MSRA #SystemicSclerosis #Scleroderma #MSRARevision #Rheumatology #MSK #MSRAExam #Autoimmune