Table 3.

Treatment/Outcomes of Subjects with SSc-ILD and PH

Treatment/Outcome	SSc-ILD and PH (N=29)
History of Treatment with PAH Targeted Therapies, no (%)	24 (82.8)
Initial Treatment with PAH Targeted Therapies, no (%)
None	5 (17.2)
PDE5I	19 (65.5)
PDE5I and ERA	4 (13.8)
Intravenous Prostacyclin	1 (3.4)
Most Recent PAH Targeted Therapy Regimen, no (%)
None	6 (20.7)
PDE5I	13 (44.8)
ERA	1 (3.4)
PDE5I and ERA	3 (10.3)
PDE5I and Inhaled Prostacyclin	1 (3.4)
ERA and IV Prostacyclin	1 (3.4)
PDE5I, ERA and Inhaled Prostacyclin	2 (6.9)
PDE5I, ERA, and IV Prostacyclin	1 (3.4)
PDE5I, ERA and Clinical Trial	1 (3.4)
Use of Single Agent PAH Targeted Therapy, no (%)	11 (37.9)
Use of Dual Agent PAH Targeted Therapy, no (%)	9 (31)
Use of Triple Agent PAH Targeted Therapy, no (%)	4 (13.8)
Requirement of Prostacyclin During PH Therapy, no (%)	6 (20.7)
History of ILD Treatment, no (%)	24 (82.8)
History of ILD Treatment with Mycophenolate Mofetil, no (%)	21 (72.4)
History of ILD Treatment with Pulse IV Cyclophosphamide, no (%)	8 (27.6)
Most Recent ILD Treatment, no (%)
None	9 (31)
Mycophenolate Mofetil	15 (51.7)
Rituximab	2 (6.9)
Tocilizumab and Mycophenolate Mofetil	1 (3.4)
Pirfenidone and Mycophenolate Mofetil	1 (3.4)
Cyclophosphamide	1 (3.4)
History of Supplemental Oxygen Use, no (%)	9 (31)
History of Transplant,* no (%)	1 (3.4)
Alive or Deceased, no (%)
Alive	27 (93.1)
Deceased	2 (6.9)
WHO Functional Class (Prior to PH Diagnosis), no (%)
Class I	0 (0)
Class II	12 (41.4)
Class III	16 (55.2)
Class IV	1 (3.4)
WHO Functional Class (Most Recent), no (%)
Class I	4 (13.8)
Class II	8 (27.6)
Class III	17 (58.6)
Class IV	0 (0)

^*Autologous HSCT

SSc – systemic sclerosis; ILD – interstitial lung disease; SSc-ILD – systemic sclerosis associated interstitial lung disease; PH – pulmonary hypertension; PAH – pulmonary arterial hypertension; PDE5i – phosphodiesterase-5 inhibitor; ERA – endothelin-receptor antagonist; HSCT – hematopoietic stem-cell transplant; WHO – World Health Organization; IV-intravenous.