The three-year incidence of pulmonary arterial hypertension associated with systemic sclerosis in a multicenter nationwide longitudinal study in France.
OBJECTIVE
An algorithm for the detection of pulmonary arterial hypertension (PAH), based on the presence of dyspnea and the findings of Doppler echocardiographic evaluation of the velocity of tricuspid regurgitation (VTR) and right-sided heart catheterization (RHC), which was applied in a large multicenter systemic sclerosis (SSc) population, estimated the prevalence of PAH to be 7.85%. The aim of this observational study was to investigate the incidence of PAH and pulmonary hypertension (PH) during a 3-year followup of patients from the same cohort (the ItinérAIR-Sclérodermie Study).


METHODS
Patients with SSc and without evidence of PAH underwent evaluation for dyspnea and VTR at study entry and during subsequent visits. Patients in whom PAH was suspected because of a VTR of 2.8-3.0 meters/second and unexplained dyspnea or a VTR of >3.0 meters/second underwent RHC to confirm the diagnosis.


RESULTS
A total of 384 patients were followed up for a mean+/-SD of 41.03+/-5.66 months (median 40.92 months). At baseline, 86.7% of the patients were women, and the mean+/-SD age of the patients was 53.1+/-12.0 years. The mean+/-SD duration of SSc at study entry was 8.7+/-7.6 years. After RHC, PAH was diagnosed in 8 patients, postcapillary PH in 8 patients, and PH associated with severe pulmonary fibrosis in 2 patients. The incidence of PAH was estimated to be 0.61 cases per 100 patient-years. Two patients who exhibited a mean pulmonary artery pressure of 20-25 mm Hg at baseline subsequently developed PAH.


CONCLUSIONS
The estimated incidence of PAH among patients with SSc was 0.61 cases per 100 patient-years. The high incidence of postcapillary PH highlights the value of RHC in investigating suspected PAH.
Hachulla
Eric
E
Hôpital Claude Huriez, Department of Internal Medicine, National Reference Center for Scleroderma, and University of Lille, Lille, France. ehachulla@chru-lille.fr
de Groote
Pascal
P
Gressin
Virginie
V
Sibilia
Jean
J
Diot
Elisabeth
E
Carpentier
Patrick
P
Mouthon
Luc
L
Hatron
Pierre-Yves
PY
Jego
Patrick
P
Allanore
Yannick
Y
Tiev
Kiet Phong
KP
Agard
Christian
C
Cosnes
Anne
A
Cirstea
Daniela
D
Constans
Joël
J
Farge
Dominique
D
Viallard
Jean-François
JF
Harle
Jean-Robert
JR
Patat
Frédéric
F
Imbert
Bernard
B
Kahan
André
A
Cabane
Jean
J
Clerson
Pierre
P
Guillevin
Loïc
L
Humbert
Marc
M
D000328:Adult; D000368:Aged; D001794:Blood Pressure / Q000502:physiology; D006328:Cardiac Catheterization; D015331:Cohort Studies; D004417:Dyspnea / Q000175:diagnosis / Q000209:etiology; D015150:Echocardiography, Doppler; D005260:Female; D005602:France / Q000453:epidemiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000453:epidemiology* / Q000209:etiology*; D015994:Incidence; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D011651:Pulmonary Artery / Q000503:physiopathology; D012595:Scleroderma, Systemic / Q000150:complications*; D014262:Tricuspid Valve Insufficiency / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology