WHAT IS PH? : EPIDEMIOLOGY

DEFINITION AND CLASSIFICATION / PATHOPHYSIOLOGY / EPIDEMIOLOGY / CLINICAL PRESENTATION / MANAGEMENT / NATURAL HISTORY

Epidemiology data on PAH have varied between publications and also depend on the aetiology of the condition. The most recent data have been published by the French National Reference Centre (1). The incidence in the French population was 2.4 per million per year and the prevalence was 15 per million which represent the lower limit of the true figures. The table below summarises the distribution of PAH cases according to aetiology in France.

Form of PAH	% of PAH Cases	Female : Male	Mean Age at Diagnosis (yrs)
IPAH	40.5	1.6 : 1	52
FPAH	2.5	2.2 : 1	37
Connective tissue disease	18.2	3.9 : 1	56
Congenital heart disease	4.1	2.0 : 1	39
Portal hypertension	14.9	1: 1.5	52
Anorexigens	3.3	15 : 1	57
HIV	9.9	1 : 1.2	42

The proportion of patients with the condition affected by PAH varies widely and is summarized in the table below.

Condition	% with PAH
Systemic sclerosis	12
Congenital heart disease	15-29
Portal hypertension	2
HIV	0.5

(1) Humbert M et al. Pulmonary arterial hypertension in France. Results from a national registry. AJRCCM 2006; 173: 1023-30.