SYATEMIC SCLEROSIS (SCLERODERMA/SSc)
SSc is categorized into diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc (lcSSc) subsets on the basis of the extent and distribution of skin involvement. LcSSc is commonly associated with the CREST syndrome.
Skin involvement is a nearly universal feature of SSc, and is characterized by variable thickening and hardening of the skin. The fingers, hands, and face are generally the earliest areas of the body involved. Edematous swelling and erythema of the skin generally precedes skin induration. Other skin manifestations can occur. Malaise, fatigue, arthralgias and myalgias are common.
The most obvious clinical manifestation of vascular dysfunction
of SSc is Raynaud phenomenon, which is due to arterial vasoconstriction
in the digits. Characteristic sequential color changes in the digits of pallor ("white"), acrocyanosis ("blue"), and reperfusion hyperemia ("red") are precipitated by cold, stress, or even change in temperatures.
and subsequent chronic damage underlies other serious complications of SSc, including pulmonary artery
hypertension, scleroderma renal crisis
and gastric antral vascular ectasia, and also contributes to the pathogenesis of cardiac and gastrointestinal complications.
Gastrointestinal involvement is present in most patients and can involve any part of the gastrointestinal tract. Symptoms are present in more than half of patients and most commonly result from chronic gastroesophageal reflux, with subsequent chronic esophagitis and stricture formation, Barrett's esophagus, and abnormal motility.
Pulmonary involvement is seen in more than 70 percent of patients. The principal pulmonary manifestations are interstitial lung disease and pulmonary vascular disease
. The latter leads to pulmonary arterial hypertension and is more common in patients with limited cutaneous disease.
Clinical renal disease is observed in up to half of patients. Findings may include some degree of proteinuria, a mild elevation in the plasma creatinine concentration, and/or hypertension. Pathologic vascular changes are present in the kidneys in 60 to 80 percent of patients with dcSSc. Scleroderma renal crisis occurs in up to 10 to 15 percent of patients, generally among those with dcSSc.
Cardiac disease in SSc may be primary or secondary, and is associated with a poor prognosis. Musculoskeletal disease
, neuromuscular and genitourinary involvement may also occur, and the risk of malignancy may be increased