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
Adapted from Hao Y, et al. 2016.4
GI, gastrointestinal; ILD, interstitial lung disease.

About 1 in 10 patients with SSc will develop PAH during their lifetime.3

PAH is a leading cause of death in SSc, and mortality in these patients remains unacceptably high.4 Patients with SSc-PAH have poorer outcomes and almost 3 times higher mortality compared to patients with PAH from other causes.1

Early diagnosis of SSc-PAH is difficult, with patients usually presenting later, with severe functional and haemodynamic compromise.5 Therefore, close vigilance and regular screening are an essential cornerstone in the management of patients with SSc.1,5

"PAH remains one of the major causes of Scleroderma related death. It occurs in 10% of Scleroderma patients. Regular screening for PAH even in those without specific symptoms of PAH results in earlier detection and treatment of PAH. This leads to better outcomes both in terms of quality of life and survival for our patients."

– Dr Wendy Stephens, Consultant Rheumatologist, VIC

“Patients with systemic sclerosis can deteriorate 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

Adapted from Morrisroe K, et al. 2017.2
Survival in incident SSc-PAH patients detected in routine clinical practice compared with those detected as part of a screening program5
The Australian Scleroderma Interest Group (ASIG) has proposed the below algorithm for screening SSc patients for PAH.6 It utilises the biomarker NT-proBNP, which is an indicator of ventricular wall stress and is measured by a simple blood test.6 More information about the investigations needed to help identify PAH can be found here.
ASIG proposed SSc-PAH screening algorithm6
Adapted from Quinlivan A, et al. 2015.6
References
  1. Galiè N, et al. Eur Heart J 2016; 37:67–119.
  2. Morrisroe K, et al. Arthritis Res Ther 2017; 19:42.
  3. Thakkar V, et al. Intern Med J 2015; 45:248–54.
  4. Hao Y, et al. Arthritis Rheum 2016; 69 (5): 1067-1077
  5. Humbert M, et al. Arthritis Rheum 2011; 63:3522–30.
  6. Quinlivan A, et al. Intern Med J 2015; 45:1134–40.
Footnotes
ASIG=Australian Scleroderma Interest Group; DLCO=diffusing capacity of the lung for carbon monoxide; FVC=forced vital capacity; NT-proBNP=N-terminal pro b-type natriuretic peptide; PAH=pulmonary arterial hypertension; PFTs=pulmonary function tests; SSc=systemic sclerosis; TTE=transthoracic echocardiography.

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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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