Characteristics of Interstitial Fibrosis and Inflammatory Cell Infiltration in Right Ventricles of Systemic Sclerosis-Associated Pulmonary Arterial Hypertension

Table 2

Characteristics of SScPAH patients.

	Antibody- profile	Cause of death	SSc disease duration (yr)^§	Survival after PAH diagnosis (yr)	Medication at time of death
1 LcSSc^∗	Anticentromere	RV failure	4	0,5	prostacyclin
2 LcSSc	Anticentromere	RV failure	12	0,75	prostacyclin
3 LcSSc	Anticentromere	RV failure	1	0,08	prostacyclin
4 LcSSc	Anticentromere	Iatrogenic abdominal bleeding due to ascites punction^	1	3	ERA, PDE-5 inhibitor
5 LcSSc	ANA	Post- LTX	13	0,42	Prostacyclin

Abbreviations: ANA: antinucleolar antibody; ERA: endothelin receptor antagonist; LcSSc: Limited cutaneous SSc; LTX: lung transplantation; PDE-5: phosphodiesterase 5; RV: right ventricle; SScPAH: Systemic sclerosis-associated pulmonary arterial hypertension. ∗According to [13]. §Since first non-Raynaud symptom, at time of diagnosis of pulmonary arterial hypertension. ^Ascites caused by RV failure.