Pulmonary veno-occlusive disease is highly prevalent in scleroderma patients undergoing lung transplantation

Pulmonary complications including pulmonary hypertension (PH) and interstitial lung disease (ILD) are leading causes of mortality in systemic sclerosis/scleroderma (SSc) [1]. PH associated with SSc (SSc-PH) has been attributed to World Health Organization (WHO) group 1 disease, pulmonary arterial hypertension (SSc-PAH); WHO group 2 due to left ventricular systolic or diastolic dysfunction (SSc-PVH); or WHO group 3 related to interstitial lung disease (SSc-PH-ILD) [2, 3]. SSc-PH is found in 10–12% of SSc patients [4]. Of the 71 out of 237 patients in the PHAROS (Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma) cohort who had PH, 69% had SSc-PAH, 10% had SSc-PVH and 21% had SSc-PH-ILD [1]. SSc-PAH has poor prognosis and less favourable response to pulmonary vasodilator therapy compared to idiopathic pulmonary arterial hypertension (IPAH) [5, 6]. SSc-PH-ILD is a strong predictor of death compared to SSc-PAH or ILD alone [7].
There is an unexpectedly high incidence of PVOD in patients with SSc-PH-ILD. Presence of PVOD may be an unrecognised contributor to the dismal prognosis of these patients. Early transplant referral should be considered for those with SSc-PH-ILD. http://ow.ly/vPvc30neJZV