Therapeutic Challenges And Advances In The Management Of Systemic Sclerosis-Related Pulmonary Arterial Hypertension (SSc-PAH)

Figures & data

Table 1a Echocardiographic Probability Of Pulmonary Hypertension (PH) In Symptomatic Patients With A Suspicion Of Pulmonary Hypertension.

Peak Tricuspid Regurgitation Velocity (m/s)	Presence Of Other Echo “PH Signs”^a	Echocardiographic Probability Of PH
≤2.8 or not measurable	No	Low
≤2.8 or not measurable	Yes	Intermediate
2.9–3.4	No
2.9–3.4	Yes	High
> 3.4	Not required

Notes: ^aSee Table 1b. Reproduced with permission of the © 2019 European Society of Cardiology & European Respiratory Society. European Respiratory Journal 46 (4) 903-975; DOI: 10.1183/13993003.01032-2015 Published 30 September 2015.³⁰

Table 1b Echocardiographic Signs Used To Assess The Probability Of Pulmonary Hypertension In Addition To The TR Velocity Measurement As Detailed In Table 1a.

A: The Ventricles	B: Pulmonary Artery	C: Inferior Vena Cava (IVC) And Right Atrium
Right ventricle/left ventricle basal diameter ratio > 1.0	Right ventricular outflow Doppler acceleration time < 105 msec and/or mid systolic notching	IVC diameter >21 mm with decreased inspiratory collapse (<50% with a sniff or <20% with quiet inspiration)
Flattening of the interventricular septum (left ventricular eccentricity index^a > 1.1 in systole and/or diastole)	Early diastolic pulmonary regurgitation velocity > 2.2m/sec	Right atrial area (end-systole) > 18 cm^2
	Pulmonary artery diameter > 25mm

Notes: ^aLeft ventricular eccentricity index: ratio of the antero-inferior and septal-posterolateral cavity dimensions at the mid-ventricular level. At least two echocardiographic signs, from two different categories (A/B/C) listed above, should be present to alter the level of echocardiographic probability of pulmonary hypertension as noted in Table 1a. Reproduced with permission of the © 2019 European Society of Cardiology & European Respiratory Society. European Respiratory Journal 46 (4) 903-975; DOI: 10.1183/13993003.01032-2015 Published 30 September 2015.³⁰

Table 2 FDA-Approved Therapies For Pulmonary Arterial Hypertension

Drug Class / MechanismOf ActionRoute Of Administration	PDE5 Inhibitors	sGC Stimulator	Endothelin Receptor Antagonists	Prostacyclin Analogs
Oral	Sildenafil Tadalafil	Riociguat	Bosentan Ambrisentan Macitentan	Treprostinil Selexipag
Inhaled	n/a	n/a	n/a	Treprostinil
Subcutaneous	n/a	n/a	n/a	Treprostinil
Intravenous	Sildenafil	n/a	n/a	Epoprostenol Treprostinil

Abbreviations: PDE5, phosphodiesterase type 5; sGC, soluble guanylate cyclase.

Figure 1 The DETECT algorithm to screen for pulmonary arterial hypertension in the systemic sclerosis patient.

Note: Adapted by permission from BMJ Publishing Group Limited. [Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Coghlan JG, Denton CP, Grunig E, et al. Ann Rheum Dis. 73(7):1340–1349, copyright 2014].²⁸

Figure 2 The ASIG (Australian Scleroderma Interest Group) algorithm to screen for pulmonary arterial hypertension in a patient with systemic sclerosis

Note: Data from Thakkar et al.⁵²

Galie N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: the joint task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J. 2016;37(1):67–119. doi:10.1093/eurheartj/ehv31726320113

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Coghlan JG, Denton CP, Grunig E, et al. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Ann Rheum Dis. 2014;73(7):1340–1349. doi:10.1136/annrheumdis-2013-20330123687283

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Thakkar V, Stevens WM, Prior D, et al. N-terminal pro-brain natriuretic peptide in a novel screening algorithm for pulmonary arterial hypertension in systemic sclerosis: a case-control study. Arthritis Res Ther. 2012;14(3):R143. doi:10.1186/ar387622691291

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