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.