🎧 MSRA Podcast Deep Dive: Systemic Sclerosis (Scleroderma)
The rapid-fire essentials for your MSRA revision
🧠 Key Learning Points
Definition
• 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 descent
Pathophysiology
• Overactive fibroblasts → collagen → tissue fibrosis
• Microvascular damage: Raynaud’s, digital ulcers
• Autoantibodies: ANA, anti-centromere, anti-Scl-70, anti-RNA pol III
Symptoms & 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, dyspareunia
Diagnosis & 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 monitoring
Complications & 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/
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