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SYSTEMIC SCLEROSIS (FEATURES (microvascular abnormalities, internal organ…
SYSTEMIC SCLEROSIS
FEATURES
microvascular abnormalities
internal organ fibrosis
scleroderma (skin fibrosis)
Skin disease is limited or diffuse.
Limited involves
hands
feet
face
(formally crest syndrome
calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia.
It is associated with
anticentromere antibodies
in 70–80%.
Pulmonary hypertension is often present subclinically, and can become rapidly life-threatening, so should be looked for (℞:
sildenafil, bosentan
).
Diffuse involves:
Antitopoisomerase-1 (scl-70) antibodies in 40%
anti-rna polymerase in 20%
Prognosis is often poor
Control bp meticulously.
Perform annual echocardiogram and spirometry.
Both limited and diffuse have the potential for organ fibrosis: lung, cardiac, gi, and renal but this occurs later in limited sub-set.
Severe cases have a 40–50% mortality at 5 years.
90% are ana positive
30–40% have anticentromere antibodies