Pulmonary arterial hypertension associated with connective tissue diseases (CTD-PAH): Recent and advanced data.
Autoimmun Rev
; 23(4): 103506, 2024 Apr.
Article
in En
| MEDLINE
| ID: mdl-38135175
ABSTRACT
Pulmonary arterial hypertension (PAH), corresponding to group 1 of pulmonary hypertension classification, is a rare disease with a major prognostic impact on morbidity and mortality. PAH can be either primary in idiopathic and heritable forms or secondary to other conditions including connective tissue diseases (CTD-PAH). Within CTD-PAH, the leading cause of PAH is systemic sclerosis (SSc) in Western countries, whereas systemic lupus erythematosus (SLE) and mixed connective tissue disease (MCTD) are predominantly associated with PAH in Asia. Although many advances have been made during the last two decades regarding classification, definition early screening and risk stratification and therapeutic aspects with initial combination treatment, the specificities of CTD-PAH are not yet clear. In this manuscript, we review recent literature data regarding the updated definition and classification of PAH, pathogenesis, epidemiology, detection, prognosis and treatment of CTD-PAH.
Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Connective Tissue Diseases
/
Pulmonary Arterial Hypertension
Limits:
Humans
Language:
En
Journal:
Autoimmun Rev
Journal subject:
ALERGIA E IMUNOLOGIA
Year:
2024
Document type:
Article
Country of publication:
Países Bajos