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)