raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:275786

Drug- or toxin-induced pulmonary arterial hypertension

Synonyms: Drug- or toxin-induced PAH

Type
Clinical group
Group
Group of disorders
Inheritance
Multigenic/multifactorial
Age of onset
Adolescent, Adult, Childhood, Elderly
Status
active

Drug- or toxin-induced pulmonary arterial hypertension (PAH) is a form of pulmonary arterial hypertension (PAH) secondary to the exposition to drugs. Drug- or toxin-induced PAH is characterized by elevated pulmonary arterial resistance leading to right heart failure. Drug or toxin induced PAH is progressive and potentially fatal.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enDrug- or toxin-induced pulmonary arterial hypertensionDrug- or toxin-induced PAH
frHypertension artérielle pulmonaire médicamenteuse ou toxiqueHTAP médicamenteuse ou toxique
dePulmonale arterielle Hypertonie, Medikamenten- oder Toxin-induziertePAH, Medikamenten- oder Toxin-induzierte
esHipertensión arterial pulmonar inducida por fármacos o toxinasHAP inducida por drogas o toxinas
itIpertensione arteriosa polmonare indotta dalle tossine o dai farmaciPAH indotta dalle tossine o dai farmaci
nlMedicatie- of toxinegeïnduceerde pulmonale arteriële hypertensieMedicatie- of toxinegeïnduceerde PAH
ptHipertensão arterial pulmonar induzida por fármacos ou toxinas
plTętnicze nadciśnienie płucne indukowane lekiem lub toksynąPAH inukowane lekiem lub toksyną
csPlicní arteriální hypertenze indukovaná léky nebo toxinemLéky nebo toxinem indukovaná PAH
ukЛегенева артеріальна гіпертензія, спричинена лікарськими засобами або токсинамиЛАГ, спричинена лікарськими засобами або токсинами
zh药物或毒素引起的肺动脉高压药物或毒物引起的PAH
trİlaç veya toksin kaynaklı pulmoner arteriyel hipertansiyonİlaç veya toksin kaynaklı PAH

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
MONDO0017149Evalidated
MONDO17149Evalidated
UMLSC0340544Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

No children.

4

Genes

1 direct
GeneAssociationStatusHGNCEnsemblOMIMLocusNameSource of validation
BMPR2Major susceptibility factor inAssessedHGNC:1078ENSG000002042176007992q33.1-q33.2bone morphogenetic protein receptor type 222523367[PMID]
5

Prevalence and incidence

1 record
TypeClassValueAreaQualificationValidationSource
Point prevalence1-9 / 1 000 000—FranceClass onlyValidated16456139[PMID]_ORPHANET

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

6

HPO phenotypes

Orphanet has no HPO annotation for this code in this release.

7

Sources for this card