Systemic sclerosis refractory disease: from the skin to the heart.
Autoimmun Rev
; 10(11): 693-701, 2011 Sep.
Article
em En
| MEDLINE
| ID: mdl-21575745
ABSTRACT
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.
Texto completo:
1
Bases de dados:
MEDLINE
Assunto principal:
Escleroderma Sistêmico
/
Transplante de Células-Tronco
/
Fatores Imunológicos
/
Antifibrinolíticos
Tipo de estudo:
Guideline
Limite:
Humans
Idioma:
En
Revista:
Autoimmun Rev
Assunto da revista:
ALERGIA E IMUNOLOGIA
Ano de publicação:
2011
Tipo de documento:
Article
País de afiliação:
Portugal