Abstract

Scleroderma is a rare persistent hardening and contraction of the body’s connective tissue, which often affect any organ, including the skin, heart, kidney, lung or oesophageal. In skin, it shows thickened and waxy, mauve at first but ivory-coloured later.
Systemic scleroderma is a systemic fibrotic disorder. In the skin, there is early CD4+ T-cell infiltration and massive normal type Ι collagen deposition by dermal fibroblasts likely induced by transforming growth factor β (TGFβ). Arterial endothelial cell damage with myointimal cell proliferation (onion skinning) occurs, resulting in narrowing of the vascular lumen. Ischemic damage and fibrosis can occur in visceral organs as a result of this vasculopathy.
In limited systemic scleroderma, fibrotic skin disease is limited to the hands and forearms, fet, neck, and face. Pulmonary hypertension can occur. Patients with limited scleroderma have a high incidence of anticentromere antibodies. In systemic scleroderma, fibrotic skin involves the fingers, hands, arms,legs, and typically the trunk and face. Pulmonary (interstitial lung disease), renal, gastrointestinal, and cardiac involvement can occur. Patients with systemic scleroderma are more likely to have antibodies to topoisomerase І.
The lower oesophageal involvement that occur in patients with systemic scleroderma may lead to severe oesophageal reflux, dysphagia and ultimately oesophageal strictures may develop. Involvement of the small intestine may lead to low of motility with malabsorption secondary to bacterial overgrowth. Other complications of gastrointestinal involvement with systemic scleroderma include watermelon stomach (gastric antral vascular ectasia) and pnewmatosis cystoids intestinalis.
The hearts of patients with systemic scleroderma may be affected by patchy fibrosis, which can cause conduction disturbances and arrhythmias. Pericarditis and congestive heart failure can also occur. In the event of renal involvement, patients can have hypertension with mild proteinuria that sometimes leads to scleroderma renal crisis (accelerated hypersenison and rapid loss of kidney function progesssing to renal failure). Most patients who develop scleroderma renal crisis have diffuse cataneous involmement. Microangiopathic haemolytic anemia and thrombocytopenia can be present in the setting of renal crisis.