Pulmonary Hypertension in Scleroderma
Pulmonary Hypertension in Scleroderma
Pulmonary arterial hypertension is a frequent complication of SSc, with a prevalence of 8–12%. A recent meta-analysis of more than 3500 SSc patients showed that PAH prevalence in SSc, based on RHC, is slightly less than 10%. In a recent large, multicenter cohort study of SSc patients, using a revised screening algorithm for PAH diagnosis based on dyspnea and Doppler echocardiographic evaluation of tricuspid regurgitant jet (TRJ) for referral of patients to RHC, 384 SSc patients without severe respiratory disease or severe left heart disease at baseline for a mean of 41.03 ± 5.66 months (median 40.92 months), an overall incidence of pulmonary hypertension of 1.37 cases per 100 patient-years [95% confidence interval (CI) 0.74–2.00] was observed. Among the 18 patients diagnosed with pulmonary hypertension, cardiopulmonary hemodynamics were consistent with PAH in eight (incidence: 0.61 cases per 100 patient-years), postcapillary pulmonary hypertension in eight (incidence: 0.61 cases per 100 patient-years), and pulmonary hypertension due to pulmonary fibrosis in two (incidence: 0.15 cases per 100 patient-years). In all cases, RHC was necessary for diagnosis of PAH, as well as excluding a diagnosis of postcapillary pulmonary hypertension.
Pulmonary arterial hypertension is generally considered a late complication of limited cutaneous SSc. Previous studies report the duration between diagnosis of SSc and onset of PAH, ranging from 9.08 ± 6.6 to 14 ± 5 years. In a recent study, mean PAH diagnosis occurred 6.3 ± 6.6 years after the first non-Raynaud's phenomenon symptom of SSc. In fact, PAH can occur at any time following the diagnosis of SSc, in about half of cases within the first 5 years following the first non-Raynaud's symptom. Patients with early-onset PAH tended to be older at the time of SSc diagnosis, have more severe pulmonary vascular disease, and a lower cardiac index and higher PVR than patients with late-onset PAH. Since the mean age at the time of PAH diagnosis between two groups was similar, these results suggest that patients who develop SSc later in life may experience a more rapid and severe development of pulmonary vascular manifestations that results in an earlier onset of PAH. The reasons why patients with late-onset PAH and more severe hemodynamic parameters exhibit the same survival rate as patients with early-onset PAH require further investigation.
Patients with limited cutaneous SSc are generally considered at greater risk of PAH than patients with diffuse cutaneous SSc. However, diagnosis of PAH is often not based on RHC and, therefore, false-positive diagnosis of PAH or existence of postcapillary pulmonary hypertension cannot be excluded in a significant proportion of cases. The frequency of diffuse cutaneous SSc observed in a recent study (22% of 78 patients with PAH associated with SSc) confirms the frequency of 25% reported among 148 patients with PAH in the Royal Free Hospital population. Therefore, PAH should not be considered a specific vascular complication of limited cutaneous SSc alone.
Epidemiology
Pulmonary arterial hypertension is a frequent complication of SSc, with a prevalence of 8–12%. A recent meta-analysis of more than 3500 SSc patients showed that PAH prevalence in SSc, based on RHC, is slightly less than 10%. In a recent large, multicenter cohort study of SSc patients, using a revised screening algorithm for PAH diagnosis based on dyspnea and Doppler echocardiographic evaluation of tricuspid regurgitant jet (TRJ) for referral of patients to RHC, 384 SSc patients without severe respiratory disease or severe left heart disease at baseline for a mean of 41.03 ± 5.66 months (median 40.92 months), an overall incidence of pulmonary hypertension of 1.37 cases per 100 patient-years [95% confidence interval (CI) 0.74–2.00] was observed. Among the 18 patients diagnosed with pulmonary hypertension, cardiopulmonary hemodynamics were consistent with PAH in eight (incidence: 0.61 cases per 100 patient-years), postcapillary pulmonary hypertension in eight (incidence: 0.61 cases per 100 patient-years), and pulmonary hypertension due to pulmonary fibrosis in two (incidence: 0.15 cases per 100 patient-years). In all cases, RHC was necessary for diagnosis of PAH, as well as excluding a diagnosis of postcapillary pulmonary hypertension.
Pulmonary arterial hypertension is generally considered a late complication of limited cutaneous SSc. Previous studies report the duration between diagnosis of SSc and onset of PAH, ranging from 9.08 ± 6.6 to 14 ± 5 years. In a recent study, mean PAH diagnosis occurred 6.3 ± 6.6 years after the first non-Raynaud's phenomenon symptom of SSc. In fact, PAH can occur at any time following the diagnosis of SSc, in about half of cases within the first 5 years following the first non-Raynaud's symptom. Patients with early-onset PAH tended to be older at the time of SSc diagnosis, have more severe pulmonary vascular disease, and a lower cardiac index and higher PVR than patients with late-onset PAH. Since the mean age at the time of PAH diagnosis between two groups was similar, these results suggest that patients who develop SSc later in life may experience a more rapid and severe development of pulmonary vascular manifestations that results in an earlier onset of PAH. The reasons why patients with late-onset PAH and more severe hemodynamic parameters exhibit the same survival rate as patients with early-onset PAH require further investigation.
Patients with limited cutaneous SSc are generally considered at greater risk of PAH than patients with diffuse cutaneous SSc. However, diagnosis of PAH is often not based on RHC and, therefore, false-positive diagnosis of PAH or existence of postcapillary pulmonary hypertension cannot be excluded in a significant proportion of cases. The frequency of diffuse cutaneous SSc observed in a recent study (22% of 78 patients with PAH associated with SSc) confirms the frequency of 25% reported among 148 patients with PAH in the Royal Free Hospital population. Therefore, PAH should not be considered a specific vascular complication of limited cutaneous SSc alone.
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