Progressive+Systemic+Sclerosis+(PSS)

//Western Diagnosis//
Scleroderma Chronic disease characterized by diffuse fibrosis; degenerative changes by diffuse fibrosis; degenerative changes, and vascular abnormalities in the skin, articular structures, and internal organs- esp. esophagus, GI, lung, heart, kidney

Etiology
- Unknown - occurs 4x more often in women than men -Ages 20-50 -rare in children

Generalized cutaneous thickening with progressive and fatal visceral involvement 2. Minimal skin involvement with very gradual (decades) internal involvement CREST syndrome: -Calcinosis -Raynaud's phenomenon -Esophageal dysfunction -Sclerodactyly -Telangiectasia (spider veins)

Signs & Symptoms
1. Initial -Raynaud's -Swelling of ends of fingers -Skin thickening on fingers -Polyarthralgias -Heartburn, dysphagia -Dyspnea 2. Dermatological -sclerodactyly -Generalized induration (hardness) -Taut, shiny, hyperpigmented skin -masklike facies -Telangiectasia (spider veins) on fingers, chest, face, lips, tongue -subcutaneous calcifications - calcinosis: fingertips, bony prominences 3. Muscular -Friction rubs over joints, tendond, bursae -Flexion contractures of fingers, wrists, elbows -Trophic ulcers on fingertips 4. Gastrointestinal -Esophageal dysfunction: dysphagia, acid reflux, esophagitis -Barrett's metaplasia - SI hypomobility -Pneumatosis cystoides intestinalis: sacs in colon due to degeneration of muscular mucosa 5. Respiratory -Lung fibrosis with exertional dyspnea -pleurisy -pericarditis 6. CV -pulmonary hypertension -arrhythmias -ECG abnormal -cardiac failure 7. Renal -hyperplasia of smaller arteries in kidney - malignant hypertension -renal insufficiency

Lab Testing
1. RF in 1/3 of cases ANA positive in 90% of cases antitropoisomerase I levels

Prognosis
1. variable, unpredictable course -often slowly progressive 3. poor if Cardiovascular, pulmonary, renal signs early

Treatment
1. No drug has influenced natural history of PSS 2. meds for various signs