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Pulmonary arterial hypertension associated with connective tissue disease

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Disease definition

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.

ORPHA:275798

Classification level: Group of disorders

Synonym(s):
  • PAH associated with connective tissue disease

Prevalence: 1-9 / 1 000 000

Inheritance: Not applicable

Age of onset: All ages

UMLS: C3697982

A summary on this disease is available in Español, Deutsch, Nederlands
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

General public
Article for general public
Français (2022) - ELF
Polski (2022) - ELF
Deutsch (2022) - ELF
Українська (2021.pdf) - ELF
Ελληνικά (2022) - ELF
English (2023) - ELF
Español (2022) - ELF
Svenska (2022) - Socialstyrelsen
Русский (2022) - ELF
Italiano (2022) - ELF
Guidelines
Emergency guidelines
Français (2020.pdf) - Orphanet Urgences
Clinical practice guidelines
Français (2020) - PNDS Logo FSMR
Deutsch (2020) - AWMF
English (2023) - Eur J Heart Fail Logo ERN
English (2022) - Eur Heart J Logo ERN
English (2011) - Int J Cardiol Logo ERN
English (2016) - Eur Heart J Logo ERN
Disease review articles
Review article
English (2013) - Orphanet J Rare Dis
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Patient-Centered Outcome Measures (PCOMs)
Access questionnaires assessing quality of life in this disease (English)
Additional information

Further information on this disease

Patient-centred resources for this disease

Research activities on this disease

Newborn screening

The documents contained in this website are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.