[Pulmonary arterial hypertension in systemic sclerosis: definition of a screening algorithm for early detection (the ItinérAIR-Sclérodermie Study)].
OBJECTIVE
Pulmonary arterial hypertension (PAH) is a severe complication of scleroderma. Its prevalence varies from 5% to 35% in the literature. A systematic yearly screening is recommended for early detection and management of PAH, but no precise algorithm is yet available.


METHODS
From literature analysis as well as evaluation of medical needs and practices, a multidisciplinary board of experts proposed an algorithm for the screening of PAH in scleroderma.


RESULTS
This algorithm is based on a precise Doppler echocardiography methodology for the purpose of screening scleroderma patients for PAH. Patients are considered as being at high or low risk of PAH depending on the maximal tricuspid regurgitation velocity. High-risk patients undergo right heart catheterization for confirmation of the diagnosis of PAH. A French multicenter transversal observational study ("ItinérAIR Sclérodermie") will be conducted in 21 hospital centers in France and involved 100 investigators organized as multidisciplinary networks.


CONCLUSIONS
Final results will provide confirmation that the screening algorithm is applicable in a real world setting, as well as a better knowledge of the prevalence of PAH in the various sub-groups of scleroderma patients, of the risk profile for PAH and of the value of DLCO as a predictive factor for PAH, and will support elaboration of precise screening guidelines.
Hachulla
E
E
Service de médecine interne, CHRU Claude-Huriez, place de Verdun, 59037 Lille cedex, France. ehachulla@chru-lille.fr
Gressin
V
V
Guillevin
L
L
de Groote
P
P
Cabane
J
J
Carpentier
P
P
Francès
C
C
Kahan
A
A
Humbert
M
M
D000465:Algorithms; D006328:Cardiac Catheterization; D015150:Echocardiography, Doppler; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000209:etiology*; D008403:Mass Screening / Q000379:methods / Q000592:standards*; D017410:Practice Guidelines as Topic; D012307:Risk Factors; D012595:Scleroderma, Systemic / Q000150:complications*