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PAH IN SYSTEMIC SCLEROSIs

Systemic sclerosis (SSc) is an autoimmune connective tissue disease affecting multiple organs. PAH is a well-known cardiopulmonary complication of SSc.1 The estimated prevalence of SSc-PAH in Australia is 11.8%, making it the second most common type of PAH after the idiopathic form.1,2

“Patients with systemic sclerosis can deteriotate very quickly between annual visits, even if their initial presentation is relatively mild.”

– Dr Hanish Bagga, Consultant Rheumatologist, NSW

SSc screening programs can reduce mortality5

Screening in SSc populations enables earlier diagnosis of PAH, facilitating earlier intervention and improving long-term survival.5 Annual cardiopulmonary screening is recommended in all patients with SSc, in order to identify patients who should have a right heart catheterisation to diagnose potential PAH.2

References

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This content is intended for Australian healthcare professionals. For more information on pulmonary hypertension, please contact your healthcare professional.
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World Health Organization functional class (WHO-FC) for patients with PH3,5

This system grades PH severity according to the patient’s functional status, by linking symptoms with activity limitations. WHO-FC remains a powerful predictor of outcomes in patients with PAH.

WHO - FC Description
I
Patients with PH in whom there is no limitation of usual physical activity; ordinary physical activity does not cause increased dyspnoea, fatigue, chest pain, or presyncope.
II
Patients with PH who have mild limitation of physical activity. There is no discomfort at rest, but normal physical activity causes increased dyspnoea, fatigue, chest pain, or presyncope.
III
Patients with PH who have a marked limitation of physical activity. There is no discomfort at rest, but less than ordinary activity causes increased dyspnoea, fatigue, chest pain, or presyncope.
IV
Patients with PH who are unable to perform any physical activity at rest and who may have signs of right ventricular failure. Dyspnoea and/or fatigue may be present at rest, and symptoms are increased by almost any physical activity.
Adapted from McGoon M, et al. 2004.5
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This website is intended for Australian healthcare professionals only