Advances in Clinical and Experimental Medicine

Title abbreviation: Adv Clin Exp Med
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ISSN 1899–5276 (print)
ISSN 2451-2680 (online)
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Advances in Clinical and Experimental Medicine

2008, vol. 17, nr 4, July-August, p. 441–446

Publication type: review article

Language: English

Immunological Aspects of Systemic Sclerosis and New Strategies in Therapy

Immunologiczne aspekty twardziny układowej i nowe możliwości terapeutyczne

Ewa Morgiel1,, Joanna Krywejko1,, Piotr Wiland2,

1 Department of Rheumatology and Internal Diseases Silesian Piasts University of Medicine in Wrocław, Poland

2 Department of Balneology Silesian Piasts University of Medicine in Wrocław, Poland

Abstract

Systemic sclerosis is a chronic autoimmune disease characterized by multiorgan symptoms such as skin thickening, pulmonary fibrosis, pulmonary arterial hypertension, renal crisis, and gastrointestinal complications. There are two major subtypes of systemic sclerosis: limited and diffuse. The pathogenic process includes inflammatory cell activation, vascular damage, and fibroblast activation. Recent research has focused on understanding its pathological pathways. A new strategy in therapy is developing substances which interfere with the main pathogenic pathways and which will be able to improve prognostics in systemic sclerosis. The efficacy of current therapies is restricted.

Streszczenie

Twardzina układowa jest autoimmunologiczną, przewlekłą chorobą, która charakteryzuje się zajęciem narządów wewnętrznych. Poważne następstwa choroby to włóknienie skóry i płuc, nadciśnienie płucne, kryza nerkowa oraz zaburzenia układu pokarmowego. Istnieją dwie postacie kliniczne choroby: twardzina układowa ograniczona i uogólniona. Na patogenezę choroby składają sie trzy główne procesy: aktywacja immunologiczna, uszkodzenie naczyń oraz aktywacja fibroblastów. Ostatnie badania koncentrują się na zrozumieniu szlaków patogenetycznych twardziny układowej. Nowymi kierunkami w leczeniu tej choroby wydają się substancje, które zablokują specyficzny szlak patologiczny choroby, co poprawi rokowanie. Skuteczność obecnie stosowanych leków jest niestety ograniczona.

Key words

systemic sclerosis, vascular damage, cyclophosphamide, fibrosis, pathogenesis

Słowa kluczowe

twardzina układowa, uszkodzenie naczyń, cyklofosfamid, zwłóknienie, patogeneza

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