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.