SYSTEMIC SCLEROSIS (Skin disease is limited or diffuse. (Limited involves,…
Skin disease is limited or diffuse.
Both limited and diffuse have the potential for organ fibrosis: lung, cardiac, gi, and renal but this occurs later in limited sub-set.
Perform annual echocardiogram and spirometry.
Control bp meticulously.
Prognosis is often poor
anti-rna polymerase in 20%
Antitopoisomerase-1 (scl-70) antibodies in 40%
Pulmonary hypertension is often present subclinically, and can become rapidly life-threatening, so should be looked for (℞:
It is associated with
(formally crest syndrome
calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia.
scleroderma (skin fibrosis)
internal organ fibrosis
30–40% have anticentromere antibodies
90% are ana positive
Severe cases have a 40–50% mortality at 5 years.