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Approach_to_Systemic_Sclerosis_50_Slides.pptx
- 1. Approach to SystemicSclerosis Clinical evaluation, diagnosis, and management
- 2. Learning Objectives • Understandthe spectrum of systemic sclerosis • Develop a structured diagnostic approach • Recognize organ involvement early • Plan organ-based management
- 3. What is SystemicSclerosis? • Chronic autoimmune connective tissue disease • Characterized by vasculopathy, fibrosis, autoimmunity • Skin and internal organ involvement
- 4. Terminology • Scleroderma =hard skin • Systemic sclerosis = multisystem disease • Localized vs systemic forms
- 5. Epidemiology • Female predominance(4–5:1) • Onset: 30–50 years • More severe disease in males
- 6. Etiopathogenesis Overview • Endothelialinjury • Immune activation • Fibroblast dysfunction and fibrosis
- 7. Genetic and EnvironmentalFactors • HLA associations • Silica, organic solvents • Smoking as a risk factor
- 8. Disease Classification • Limitedcutaneous systemic sclerosis • Diffuse cutaneous systemic sclerosis • Sine scleroderma
- 9. Limited Cutaneous SSc •Skin involvement distal to elbows/knees • CREST features • Late pulmonary hypertension
- 10. Diffuse Cutaneous SSc •Proximal skin involvement • Early visceral disease • Higher renal crisis risk
- 11. Sine Scleroderma • Noskin thickening • Internal organ involvement present • Diagnosed by antibodies and organ disease
- 12. Clinical Approach: Overview •Detailed history • Focused physical examination • Targeted investigations • Organ-specific assessment
- 13. Key History Points •Raynaud phenomenon • Skin tightness or swelling • Dyspnea, dysphagia, chest pain
- 14. Raynaud Phenomenon • Oftenfirst manifestation • Triphasic color change • Indicator of vasculopathy
- 15. Skin Symptoms • Puffyfingers • Skin tightening • Reduced mobility
- 16. Systemic Symptoms • Fatigue •Weight loss • Myalgia and arthralgia
- 17. Physical Examination: Skin •Skin thickness scoring • Sclerodactyly • Digital ulcers
- 18. Modified Rodnan SkinScore • 17 body areas assessed • Score 0–3 per area • Used for severity and follow-up
- 19. Face and OralFindings • Mask-like facies • Microstomia • Telangiectasia
- 20. Nailfold Capillaroscopy • Dilatedcapillaries • Capillary dropout • Helps early diagnosis
- 21. Musculoskeletal Assessment • Jointcontractures • Tendon friction rubs • Inflammatory arthritis
- 22. Gastrointestinal Involvement • Esophagealdysmotility • GERD • Malabsorption
- 23. Pulmonary Involvement • Interstitiallung disease • Pulmonary arterial hypertension • Leading cause of mortality
- 24. Cardiac Involvement • Arrhythmias •Myocardial fibrosis • Pericardial effusion
- 25. Renal Involvement • Sclerodermarenal crisis • Acute hypertension • Rapid renal failure
- 26. Approach to Investigations •Autoantibody testing • Imaging studies • Functional assessments
- 27. Autoantibody Profile • ANApositive in most patients • Anti-centromere • Anti–Scl-70 • Anti–RNA polymerase III
- 28. Significance of Autoantibodies •Disease subtype prediction • Organ involvement risk • Prognostic implications
- 29. Baseline Laboratory Tests •CBC, ESR, CRP • Renal function tests • Urinalysis
- 30. Pulmonary Evaluation • HRCTchest • Pulmonary function tests • DLCO measurement
- 31. Cardiac Evaluation • ECG •Echocardiography • Cardiac MRI if needed
- 32. Renal Monitoring • Bloodpressure monitoring • Serum creatinine • Urine protein
- 33. Differential Diagnosis • Mixedconnective tissue disease • Dermatomyositis • Eosinophilic fasciitis
- 34. When to SuspectEarly SSc • Raynaud with positive ANA • Abnormal nailfold capillaries • Puffy fingers
- 35. Principles of Management •No curative therapy • Early detection of organ disease • Symptom and organ-based treatment
- 36. Management of Raynaud Phenomenon •Avoid cold exposure • Calcium channel blockers • PDE-5 inhibitors
- 37. Skin Disease Management •Methotrexate • Mycophenolate mofetil • Physical therapy
- 38. ILD Management • Mycophenolatemofetil • Cyclophosphamide • Antifibrotic agents
- 39. PAH Management • Endothelinreceptor antagonists • PDE-5 inhibitors • Prostacyclin analogues
- 40. Renal Crisis Management •Immediate ACE inhibitors • Avoid high-dose steroids • Close BP monitoring
- 41. Multidisciplinary Care • Rheumatology •Pulmonology • Cardiology and nephrology
- 42. Monitoring and Follow-Up •Regular organ screening • Assess treatment response • Early detection of complications
- 43. Prognostic Factors • Extentof skin disease • Lung and heart involvement • Autoantibody profile
- 44. Key Take-Home Messages •Early recognition is crucial • Systematic organ-based approach needed • Regular monitoring improves outcomes