Baseline characteristics and follow-up in patients with normal haemodynamics versus borderline mean pulmonary arterial pressure in systemic sclerosis: results from the PHAROS registry.
BACKGROUND
Patients with normal (mean pulmonary arterial pressure (mPAP) ≤20 mm Hg) and borderline mean pulmonary pressures (21-24 mm Hg) are "at risk" of developing pulmonary hypertension (PH). The objectives of this analysis were to examine the baseline characteristics in systemic sclerosis (SSc) with normal and borderline mPAP and to explore long-term outcomes in SSc patients with borderline mPAP versus normal haemodynamics.


METHODS
PHAROS is a multicentre prospective longitudinal cohort of patients with SSc "at risk" or recently diagnosed with resting PH on right heart catheterisation (RHC). Baseline clinical characteristics, pulmonary function tests, high-resolution CT, 2-dimensional echocardiogram and RHC results were analysed in normal and borderline mPAP groups.


RESULTS
206 patients underwent RHC (results showed 35 normal, 28 borderline mPAP, 143 resting PH). There were no differences in the baseline demographics. Patients in the borderline mPAP group were more likely to have restrictive lung disease (67% vs 30%), fibrosis on high-resolution CT and a higher estimated right ventricular systolic pressure on echocardiogram (46.3 vs 36.2 mm Hg; p<0.05) than patients with normal haemodynamics. RHC revealed higher pulmonary vascular resistance and more elevated mPAP on exercise (≥30; 88% vs 56%) in the borderline mPAP group (p<0.05 for both). Patients were followed for a mean of 25.7 months and 24 patients had a repeat RHC during this period. During follow-up, 55% of the borderline mPAP group and 32% of the normal group developed resting PH (p=NS).


CONCLUSIONS
Patients with borderline mPAP have a greater prevalence of abnormal lung physiology, pulmonary fibrosis and the presence of exercise mPAP ≥30 mm Hg.
Department of Internal Medicine, University of California, Los Angeles, California, USA.
Sangmee
S
Bae
Rajeev
R
Saggar
Marcy B
MB
Bolster
Lorinda
L
Chung
Mary Ellen
ME
Csuka
Chris
C
Derk
Robyn
R
Domsic
Aryeh
A
Fischer
Tracy
T
Frech
Avram
A
Goldberg
Monique
M
Hinchcliff
Vivien
V
Hsu
Laura
L
Hummers
Elena
E
Schiopu
Maureen D
MD
Mayes
Vallerie
V
McLaughlin
Jerry
J
Molitor
Nausheen
N
Naz
Daniel E
DE
Furst
Paul
P
Maranian
Virginia
V
Steen
Dinesh
D
Khanna
D001794:Blood Pressure / Q000502:physiology*; D006328:Cardiac Catheterization; D005260:Female; D005500:Follow-Up Studies; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D011651:Pulmonary Artery / Q000503:physiopathology*; D011658:Pulmonary Fibrosis / Q000150:complications / Q000503:physiopathology; D012129:Respiratory Function Tests; D012595:Scleroderma, Systemic / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology*