Interstitial Lung Disease Associated with Systemic Sclerosis.
Semin Respir Crit Care Med
; 45(3): 342-364, 2024 Jun.
Article
in En
| MEDLINE
| ID: mdl-38714203
ABSTRACT
Systemic sclerosis (SSc) is a rare autoimmune disease characterized by a tripod combining vasculopathy, fibrosis, and immune-mediated inflammatory processes. The prevalence of interstitial lung disease (ILD) in SSc varies according to the methods used to detect it, ranging from 25 to 95%. The fibrotic and vascular pulmonary manifestations of SSc, particularly ILD, are the main causes of morbidity and mortality, contributing to 35% of deaths. Although early trials were conducted with cyclophosphamide, more recent randomized controlled trials have been performed to assess the efficacy and tolerability of several medications, mostly mycophenolate, rituximab, tocilizumab, and nintedanib. Although many uncertainties remain, expert consensus is emerging to optimize the therapeutic management and to provide clinicians with evidence-based clinical practice guidelines for patients with SSc-ILD. This article provides an overview, in the light of the latest advances, of the available evidence for the diagnosis and management of SSc-ILD.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Scleroderma, Systemic
/
Lung Diseases, Interstitial
/
Immunosuppressive Agents
Limits:
Humans
Language:
En
Journal:
Semin Respir Crit Care Med
Journal subject:
TERAPIA INTENSIVA
Year:
2024
Type:
Article
Affiliation country:
France