Systemic sclerosis an clinical approach.pptx

Systemic sclerosis case

1. SYSTEMIC SCLEROSIS A CLINICAL APPROACH PRESENTED BY RAM PRASAD M S M 5 UNIT FINAL YEAR MBBS

2. Case scenario 35 yr old female presented with a 6-month history of gradual dyspnoea and a persistent dry cough . dyspnoea gradual, progressed from mmrc grade 1 to 3 with ratio FEV1/FVC > 0.8, DECREASED DLCO + dry cough. Skin becoming increasingly tight over her fingers and hands and spreading up her arms and legs with a feeling wearing a tight-fitting glove or sock . Raynaud’s Phenomenon: experiencing episodes of color change in her fingers ( white to blue to red ) in response to cold temperature Clinical Examination: Skin Examination: significant skin thickening in the distal extremities and extending up the arms and leg + decreased mobility . Respiratory Examination: B/l crackles (rales).

3. CASE scenario continued…, Probable diagnosis : SYSTEMIC SCLEROSIS WITH ILD Orphan disease Diverse presentation + Variable natural history TGF BETA LOW ESR Limited inflammatory presentation ( arthritis, myositis INVESTIGATIONS X ray : ground glass appearance HRCT : ground-glass opacities reticular patterns interlobular septal thickening ANTI TOPO ISOMERASE +VE

4. ETIOPATHOGENESIS AND ASSOCIATED CLINICAL FEATURE Aetiology 1.Unknown 2.HLA mutation 3.Parvo B 19 Endothelial cell injury Endothelial dysfunction Decreased NO Vasoconstriction Vascular obliteration TISSUE HYPOXIA

5. FIBROSIS AND ITS ASSOCIATED CLINICAL FEATURE SKIN : Increased thickening Extreme dryness of skin Loss of body oil Hair loss sever LUNGS : INTERSTITIAL LUNG DISEASE HEART : RESTRICTIVE CARDIOMYOPATHY EPITHELIAL CELLS ENDOTHELIAL CELLS FIBROSIS ( Fibrosis Proliferation ) Tissue Hypoxia ( TGF BETA ) Manifestations due to fibrosis

6. Secondary RAYNAUDS PHENOMENON Cold / Stress / Vibration Episodic Vasoconstriction Pallor , Cyanosis , Redness on rewarming : Extremity ulcer Acral osteolysis ( diffuse type ) (B) Gangrenous ulcers Critical limb ischemia RENAL involvement Thrombotic microangiopathy Symptoms associated

7. SYSTEMIC SCLEROSIS Diabetes Contrast induced (GADALONIUM) Contaminated L Tryptophan Diffuse systemic sclerosis Limited systemic sclerosis SCLERODERMA FACIES : SCLERODERMA VS SYSTEMIC SCLEROSIS SCLERODERMA MIMICS Types of SYSTEMIC SCLEROSIS

8. Limited systemic sclerosis Onset : Indolent CREST ANA + ve : Centromere Pattern Complication : Pulmonary Hypertension C alcinosis cutis R ayunaud’s E sophagitis S clerodactyly T elangiectasia

9. Diffuse systemic sclerosis Clinical features + investigation : case scenario Management : DOC : BOSENTON , NIFEDIPINE Disease Drug ILD steroids/ cyclophosphamide / MMF Renal crisis ACE inhibitors Arthritis steroids + MTX

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