Lege Artis Medicinae

[Up-to-date management of systemic sclerosis]

SZŰCS Gabriella

SEPTEMBER 21, 2009

Lege Artis Medicinae - 2009;19(08-09)

[Systemic sclerosis is a chronic autoimmune disease characterized by three major features: widespread fibrosis in the skin and internal organs, a non-inflammatory small vessel obliterative vasculopathy and immunological activation with disease-specific autoantibodies. It is necessary to take a systematic approach to the diagnosis and evaluation of each case in order to provide appropriate treatment. Disease-modifying approaches can be classified according to the underlying pathogenic process. Thus vascular therapies include agents used for Raynaud’s phenomenon, critical digital ischaemia and organ-based vascular complications such as scleroderma renal crisis and pulmonary hypertension. Immunosuppressive drugs are used in lung involvement or rapid skin progression. The results of different anti-fibrotic therapies are controversial. Finally in managing organ-based manifestations and complications a multidisciplinary approach to the therapy is useful with patient education as an integral component of successful management.]

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