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Colegio de Medicina Interna de México.

2006, Number 3

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Med Int Mex 2006; 22 (3)

Systemic sclerosis

Vera LOL

Language: Spanish
References: 99
Page: 231-245
PDF size: 373.82 Kb.


ABSTRACT

Systemic sclerosis or scleroderma is a connective tissue disease whose cause is unknown. It is characterized by excessive production of extracellular matrix (collagen) by fibroblasts in the skin and some internal organs, as well as by damage of endothelium of small vessels with subsequent intimal hyperplasia and tissue ischemia, together with activation of the immune system. The pathogenesis of systemic sclerosis begins with an environmental stimulus in a genetically susceptible individual; this stimulus activates the immune system, with delivery of immunologic mediators (cytokines) that produce endothelial damage, proliferation of fibroblasts and synthesis of collagen. Delivering endothelial cytokines produces more activation of the immune system. Systemic sclerosis is divided into: diffuse systemic sclerosis, limited systemic sclerosis, and scleroderma without scleroderma. Diffuse systemic sclerosis is characterized by generalized thickening, limited systemic sclerosis affects only face and distal extremities (arms, forearms), CREST syndrome is a form of limited systemic sclerosis; and in the scleroderma without scleroderma the skin is not affected, it only presents organs involvement. Systemic sclerosis is characterized by chronic multiorganic involvement. The earliest clinical and more frequent manifestations are Raynaud’s phenomenon, weakness, and musculoskeletal disorders. Skin is the target organ affected in the systemic sclerosis; the involvement of the skin has three forms: edematous form, hardening form and atrophic form. The gastrointestinal tract is one of the most affected organs and the esophagus is involved very frequently. Musculoskeletal manifestations are joint pain, flexion contractures, particularly of the digits by fibrosis of tendon sheaths. Lung affection is characterized by interstitial fibrosis, and pulmonary arterial hypertension; the heart is also affected, there are arrhythmias, diastolic dysfunction of left ventricle, cardiac failure, and others. Kidney is affected and there can be renal crisis. Criteria for classification are proximal scleroderma and two more of the following: sclerodactyly, digital pitting scars of fingertips or loss of substance of the distal finger pad, and bibasilar pulmonary fibrosis. In the treatment of systemic sclerosis there have been used several drugs, whose results have been discouraging. D-penicillamine as antibifrotic is effective in the treatment of the disease. However, there are some advances for the treatment of Raynaud’s phenomenon as calcium antagonists (nifedipine) and prostaglandin analogues (iloprost); prokinetics, proton pump inhibitors for gastrointestinal manifestations; methylprednisolone and cyclophosphamide pulses for pulmonary fibrosis, and cyclophosphamide pulses and endothelin-receptor antagonist, bosentan, in the pulmonary arterial hypertension.


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