[Treatment of systemic sclerosis-associated interstitial lung disease].
Therapie der systemischen Sklerose-assoziierten interstitiellen Lungenerkrankung.
Connective tissue disease
Interstitial lung disease
Organ involvement
Systemic sclerosis
Therapy
Journal
Zeitschrift fur Rheumatologie
ISSN: 1435-1250
Titre abrégé: Z Rheumatol
Pays: Germany
ID NLM: 0414162
Informations de publication
Date de publication:
Apr 2020
Apr 2020
Historique:
pubmed:
23
11
2019
medline:
12
9
2020
entrez:
23
11
2019
Statut:
ppublish
Résumé
Systemic sclerosis (SSc) is a fibrosing autoimmune disease of the connective tissue. In addition to skin fibrosis, pulmonary involvement and interstitial lung disease (ILD) in particular are the most common and severe manifestations of SSc. The disease is associated with a substantial risk of morbidity and mortality, especially in progressive ILD. In the last 5 years new treatment concepts for SSc-ILD have been investigated in numerous clinical studies. This review is based on a literature search in PubMed, focusing on the most relevant papers published up to the end of 2018 with the keywords "SSc" and "treatment". The treatment of SSc-ILD has changed over the last few years due to the results of many clinical studies. The updated guidelines of the European League Against Rheumatism (EULAR) recommend the use of cyclophosphamide or hematopoietic stem cell transplantation. Data for a positive influence on SSc-ILD are also available for mycophenolate, tocilizumab and anabasum. Because of the pathophysiological similarities to idiopathic pulmonary fibrosis, the use of the antifibrotic agents nintedanib and pirfenidone is currently being investigated in randomized, multicenter clinical trials and could be a novel and promising therapeutic strategy. Current drug studies may provide innovative therapeutic perspectives for SSc-ILD and could significantly improve the prognosis of affected patients in the future.
Sections du résumé
BACKGROUND
BACKGROUND
Systemic sclerosis (SSc) is a fibrosing autoimmune disease of the connective tissue. In addition to skin fibrosis, pulmonary involvement and interstitial lung disease (ILD) in particular are the most common and severe manifestations of SSc. The disease is associated with a substantial risk of morbidity and mortality, especially in progressive ILD. In the last 5 years new treatment concepts for SSc-ILD have been investigated in numerous clinical studies.
MATERIAL AND METHODS
METHODS
This review is based on a literature search in PubMed, focusing on the most relevant papers published up to the end of 2018 with the keywords "SSc" and "treatment".
RESULTS
RESULTS
The treatment of SSc-ILD has changed over the last few years due to the results of many clinical studies. The updated guidelines of the European League Against Rheumatism (EULAR) recommend the use of cyclophosphamide or hematopoietic stem cell transplantation. Data for a positive influence on SSc-ILD are also available for mycophenolate, tocilizumab and anabasum. Because of the pathophysiological similarities to idiopathic pulmonary fibrosis, the use of the antifibrotic agents nintedanib and pirfenidone is currently being investigated in randomized, multicenter clinical trials and could be a novel and promising therapeutic strategy.
CONCLUSION
CONCLUSIONS
Current drug studies may provide innovative therapeutic perspectives for SSc-ILD and could significantly improve the prognosis of affected patients in the future.
Identifiants
pubmed: 31754786
doi: 10.1007/s00393-019-00724-y
pii: 10.1007/s00393-019-00724-y
doi:
Substances chimiques
Immunosuppressive Agents
0
Cyclophosphamide
8N3DW7272P
Types de publication
Journal Article
Review
Langues
ger
Sous-ensembles de citation
IM
Pagination
294-303Références
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