Pulmonary Hypertension in Scleroderma
Pulmonary Hypertension in Scleroderma
Pulmonary arterial hypertension is a progressive disease with an extremely poor prognosis. PAH associated with SSc (PAH–SSc) is particularly aggressive, accounting for 30% of deaths among SSc patients and, if left untreated, PAH–SSc is associated with a median survival time of 1 year following diagnosis. Recent advances in diagnostic techniques and the emergence of evidence-based therapies for PAH facilitate earlier detection of the disease and, thus, earlier initiation of effective treatment. Early detection of PAH and prompt initiation of effective therapy are considered essential components of disease management, as in limited study patients diagnosed earlier in the course of the disease appear to have a more pronounced benefit from therapy.
Furthermore, the prevalence of RHC-proven PAH in large SSc patient cohorts is about 10%; this recognized predisposition of SSc patients to develop PAH as a complication means that with regular screening, early diagnosis of PAH is a realistic possibility in this patient population. Without screening, data from the French national registry have shown that the majority of patients are diagnosed in New York Heart Association/WHO functional class III or IV and, as such, are already severely comprised. Diagnosis of PAH in functional class I or II is challenging and, even when patients begin to deteriorate, symptoms are nonspecific and patients can be reluctant to present to their physician. Therefore, despite the potential for earlier diagnosis, in reality PAH–SSc is still frequently identified late, with more than two-thirds of patients in functional class III or IV at the time of diagnosis.
Screening: Why is Pulmonary Arterial Hypertension Screening Needed in Scleroderma Patients?
Pulmonary arterial hypertension is a progressive disease with an extremely poor prognosis. PAH associated with SSc (PAH–SSc) is particularly aggressive, accounting for 30% of deaths among SSc patients and, if left untreated, PAH–SSc is associated with a median survival time of 1 year following diagnosis. Recent advances in diagnostic techniques and the emergence of evidence-based therapies for PAH facilitate earlier detection of the disease and, thus, earlier initiation of effective treatment. Early detection of PAH and prompt initiation of effective therapy are considered essential components of disease management, as in limited study patients diagnosed earlier in the course of the disease appear to have a more pronounced benefit from therapy.
Furthermore, the prevalence of RHC-proven PAH in large SSc patient cohorts is about 10%; this recognized predisposition of SSc patients to develop PAH as a complication means that with regular screening, early diagnosis of PAH is a realistic possibility in this patient population. Without screening, data from the French national registry have shown that the majority of patients are diagnosed in New York Heart Association/WHO functional class III or IV and, as such, are already severely comprised. Diagnosis of PAH in functional class I or II is challenging and, even when patients begin to deteriorate, symptoms are nonspecific and patients can be reluctant to present to their physician. Therefore, despite the potential for earlier diagnosis, in reality PAH–SSc is still frequently identified late, with more than two-thirds of patients in functional class III or IV at the time of diagnosis.
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