Pulmonary Hypertension in Scleroderma
Pulmonary Hypertension in Scleroderma
The 1-year, 2-year and 3-year survival rates in a recent study were 86, 67 and 65%, respectively. Before specific PAH treatment era, the 3-year survival rate for patients with SSc–PAH was less than 35%. Recently, Williams et al. measured survival in two groups of matched patients with PAH associated with SSc: those treated with conventional medical therapy and prostanoids if needed (historical control group), and those treated with oral first-line treatment (current treatment era). Survival at 1 year was 68% in the historical control group, compared with 81% in the current treatment era group; the 2-year survival rates were 47 and 71%, respectively.
Mukerjee et al. reported that the prognosis of PAH associated with SSc is better whether mPAP is mildly elevated at diagnosis; in patients with mPAP less than 32 mmHg survival rates were 93 and 78% at 1 and 2 years, respectively. Another recent study observed a 2-year survival of 100% in patients with mPAP less than 32 mmHg at diagnosis. Although lead-time bias cannot be excluded, these observations suggest that early detection of PAH may improve prognosis. Earlier diagnosis of PAH is a sine qua non condition for earlier treatment; however, whether earlier diagnosis is associated with increased life expectancy in SSc patients is not yet known.
Figure 2 indicates signs and symptoms associated with prognosis and may help define the intensity of treatment that should be considered.
(Enlarge Image)
Figure 2.
Pulmonary arterial hypertension – pathogenesis occurs prior to clinical symptoms
These prognosis factors have, however, been extrapolated from patients with idiopathic PAH (IPAH) and may not be pertinent or obtainable in patients with SSc–PAH (i.e. 6-min walk test >500 m) because of associated comorbidities, muscular involvement, and lower limb ischemia (Fig. 3). Moreover, there are multiple causes of dyspnea in SSc that may mask the dyspnea associated with PAH. It appears therefore necessary to identify specific PAH–SSc goals. However, if a patient with SSc–PAH remains in functional class I/II after 4 months of treatment, prognosis is relatively good compared with patients who have deteriorated to functional class III/IV during this period (P = 0.007). A cardiac index greater than 2.71 l/min/m is also associated with a better prognosis than a cardiac index less than 2.71 l/min/m (P = 0.03). Therefore, it can be argued that surveillance RHC be performed 4 months after initiation of PAH-specific therapy; if dyspnea or functional class status is not improved to appropriate levels and/or if cardiac index remains below 2.71 l/min/m, more aggressive therapy should be considered.
(Enlarge Image)
Figure 3.
Prognosis factors according to recommendations
Prognosis
The 1-year, 2-year and 3-year survival rates in a recent study were 86, 67 and 65%, respectively. Before specific PAH treatment era, the 3-year survival rate for patients with SSc–PAH was less than 35%. Recently, Williams et al. measured survival in two groups of matched patients with PAH associated with SSc: those treated with conventional medical therapy and prostanoids if needed (historical control group), and those treated with oral first-line treatment (current treatment era). Survival at 1 year was 68% in the historical control group, compared with 81% in the current treatment era group; the 2-year survival rates were 47 and 71%, respectively.
Mukerjee et al. reported that the prognosis of PAH associated with SSc is better whether mPAP is mildly elevated at diagnosis; in patients with mPAP less than 32 mmHg survival rates were 93 and 78% at 1 and 2 years, respectively. Another recent study observed a 2-year survival of 100% in patients with mPAP less than 32 mmHg at diagnosis. Although lead-time bias cannot be excluded, these observations suggest that early detection of PAH may improve prognosis. Earlier diagnosis of PAH is a sine qua non condition for earlier treatment; however, whether earlier diagnosis is associated with increased life expectancy in SSc patients is not yet known.
Figure 2 indicates signs and symptoms associated with prognosis and may help define the intensity of treatment that should be considered.
(Enlarge Image)
Figure 2.
Pulmonary arterial hypertension – pathogenesis occurs prior to clinical symptoms
These prognosis factors have, however, been extrapolated from patients with idiopathic PAH (IPAH) and may not be pertinent or obtainable in patients with SSc–PAH (i.e. 6-min walk test >500 m) because of associated comorbidities, muscular involvement, and lower limb ischemia (Fig. 3). Moreover, there are multiple causes of dyspnea in SSc that may mask the dyspnea associated with PAH. It appears therefore necessary to identify specific PAH–SSc goals. However, if a patient with SSc–PAH remains in functional class I/II after 4 months of treatment, prognosis is relatively good compared with patients who have deteriorated to functional class III/IV during this period (P = 0.007). A cardiac index greater than 2.71 l/min/m is also associated with a better prognosis than a cardiac index less than 2.71 l/min/m (P = 0.03). Therefore, it can be argued that surveillance RHC be performed 4 months after initiation of PAH-specific therapy; if dyspnea or functional class status is not improved to appropriate levels and/or if cardiac index remains below 2.71 l/min/m, more aggressive therapy should be considered.
(Enlarge Image)
Figure 3.
Prognosis factors according to recommendations
