Pulmonary arterial hypertension associated with systemic sclerosis in patients with functional class II dyspnoea: mild symptoms but severe outcome.
OBJECTIVE
To describe the history of SSc-associated pulmonary arterial hypertension (SSc-PAH) in patients with New York Heart Association (NYHA) functional class (FC) II dyspnoea at diagnosis.


METHODS
Data at the time of diagnosis were collected and analysed retrospectively for 77 consecutive patients with SSc-PAH.


RESULTS
Twelve patients (15.6%) presented with PAH and NYHA FC II dyspnoea. After a mean follow-up of 44 months, only 4 out of the 12 PAH patients remained stable in FC II, while 8 showed worsening to FC III or IV. Three patients died during the observation period; two from PAH and one from rectal cancer. Survival in patients in FC II at diagnosis was 100, 91 and 80% at 1, 2 and 3 years, respectively.


CONCLUSIONS
A majority of patients with mildly symptomatic SSc-PAH in NYHA FC II at diagnosis have a severe disease with poor prognosis.
Department of Internal Medicine, National Reference Center for Scleroderma, Claude Huriez Hospital, University of Lille, France. ehachulla@chru-lille.fr
Eric
E
Hachulla
David
D
Launay
Azzedine
A
Yaici
Alice
A
Berezne
Pascal
P
de Groote
Olivier
O
Sitbon
Luc
L
Mouthon
Loïc
L
Guillevin
Pierre-Yves
PY
Hatron
Gérald
G
Simonneau
Pierre
P
Clerson
Marc
M
Humbert
D000328:Adult; D000368:Aged; D000704:Analysis of Variance; D004417:Dyspnea / Q000150:complications* / Q000503:physiopathology; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D012595:Scleroderma, Systemic / Q000150:complications* / Q000503:physiopathology; D012720:Severity of Illness Index; D013997:Time Factors