Systemic sclerosis refractory disease: from the skin to the heart.

• Type: Article in International Scientific Journal
• Date: 2011

Authors

Isabel Almeida (Author) ICBAS; Lúcia Faria (Author) ICBAS; Pedro Vita (Author) ICBAS; Carlos Vasconcelos (Author) ICBAS

Journal

Title: Autoimmunity Reviews

Vol. 10 No. 11

Pages: 693-701

ISSN: 1568-9972

Publisher: Elsevier

Indexing

Pubmed / Medline

Scientific classification

FOS: Medical and Health sciences > Clinical medicine

Other information

DOI: 10.1016/j.autrev.2011.04.025

Abstract (EN): Systemic sclerosis or scleroderma (SSc) is an heterogeneous disease involving the connective tissue and the microvasculature with fibrosis and vascular occlusion. It is difficult to define refractory SSc once it is itself a paradigm of a refractory condition: there is no evidence of when to act to stop the progression to fibrosis and irreversible microvascular damage. There is no definition of refractory disease in SSc and to propose a definition we used mainly the Medsger severity index and the EULAR 2009 treatment recommendations from the skin to the heart through peripheral vascular, musculoskeletal, gastrointestinal, renal, pulmonary hypertension and interstitial lung disease. We used some clinical setting reflecting the different reasoning when there is probable refractory disease and finally we briefly pointed out some available treatment options to refractory disease. With this reflection, we would like to open paths to a broader discussion.

Language: Portuguese

Type (Professor's evaluation): Scientific