Pulmonary arterial hypertension associated

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Pulmonary 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

HLA-BMajor susceptibility factor

Cross-references

MONDO 17151UMLS C3697982

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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