Rheumatology (Oxford, England) · 2010
Pulmonary arterial hypertension associated with systemic sclerosis in patients with functional class II dyspnoea: mild symptoms but severe outcome.
СтатьяМногоцентровое исследованиеПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод в очереди
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Hachulla Eric, Launay David, Yaici Azzedine, Berezne Alice, de Groote Pascal, Sitbon Olivier, Mouthon Luc, Guillevin Loïc, Hatron Pierre-Yves, Simonneau Gérald, Clerson Pierre, Humbert Marc, French PAH-SSc Network
Аннотация
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.
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.