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Start free with EleplanPulmonary arterial hypertension associated with connective tissue disease
ORPHA:275798Clinical group
Also called PAH associated with connective tissue disease
What it is
A form of pulmonary arterial hypertension (PAH) characterized by an elevated pulmonary arterial resistance leading to right heart failure observed as a complication of a connective tissue disease.
Key facts
- Prevalence
- 1-9 / 1 000 000 (Europe)
- Age of onset
- All ages
- Inheritance
- Not applicable
- Classified as
- Clinical group
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Gene
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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