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SCLERODERMA
Systemic sclerosis Also called scleroderma Multisystem disease of unknown etiology Pathogenesis include immune system
activation , endothelial activation , and fibroblast activation
this results in small blood vessels damage and tissue fibrosis.
Epidemiology Rare disease incidence in the US 19/million/year
and prevalence of 19-75/100,000 Rare in children Peak between age 35-65 More women affected Family h/o other auto immune diseases Ethnic background influence survival and
disease manifestation.
Clinical features Raynaud’s Typical skin changes Esophageal and small bowel dysfunction Interstitial lung disease Pulmonary hypertension Renal crises
What is Raynaud’s Phenomenon Reversible skin color changes:White to blue to
red Due to vasospasm Induced by cold of emotion
Raynaud’s :Pallor phase
Raynaud’s:cyanotic phase
Causes of Raynaud’s Occlusive arterial disease Rheumatic
diseases:Scleroderma,CREST,MCTD,SLE,RA, Myositis
Repetitive vascular injury Hyperviscosity :Polycythemia,Cryoglobulinemia Thoracic outlet syndrome
Typical skin changes Tight thick skin,peaked nosePursed mouth
Salt and pepper pigmentation
Microstomia
Digital ulcers
Esophageal dysmotility:heart burn,and reflux symptoms Dilated esophagus onEsophagogram
CREST Calcinosis Raynaud’s Esophageal dysmotility Sclerodactyly Telangiectasias
Calcinosis
Sclerodactyly Skin changes,and flexion contactures
Telangiectasias Dilated capillaries seenover the face
Renal crises and hypertension in scleroderma Major complication Early in disease first few years Acute onset hypertension High Renin Renal impairment Microangiopathic hemolytic anemia,Thrombocytopenia Renal failure can be reversible if BP treated Drug of choice Angiotensin converting enzyme inhibitor
Shortness of breath in Scleroderma DD,1-Interstitial lung disease:CXR,PFT,HRCT chest2-Pulmonary hypertension:Echocardiogram,right
sided cardiac catheterization3-Cardiomyopathy,Heart failure,4-Pericardial effusion5-Renal crises
Interstitial Lung disease Major cause of morbidly
Diagnosis Clinical features ANA positive 90% Anti topoisomerase 1 antibody positive(scl-70)
30% in diffuse Anticentromere antibody positive in CREST and
limited scleroderma
Treatment Skin :no effective treatment,60% improve with
time Treat hypertension early and aggressive to
prevent renal damage and other hypertension complications
Calcium channel blockers may help Raynaud’s Treat ILD and pulmonary hypertension Avoid steroids(may provoke renal complications)