SYSTEMIC SCLEROSIS

FEATURES

microvascular abnormalities

internal organ fibrosis

scleroderma (skin fibrosis)

Severe cases have a 40–50% mortality at 5 years.

90% are ana positive

30–40% have anticentromere antibodies

Skin disease is limited or diffuse.

Limited involves

Diffuse involves:

hands

feet

face

(formally crest syndrome

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).

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.

calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia.