Lung Transplants for Pulmonary Hypertension

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer

Many centers do not approve heart or lung transplants for Scleroderma patients, however, a few of them do, so contact the Second Wind Lung Transplant Association for further information.

Pulmonary veno–occlusive disease (PVOD) is highly prevalent in scleroderma (SSc) patients undergoing lung transplantation. Presence of PVOD may be an unrecognised contributor to the dismal prognosis in patients with SSc –pulmonary hypertenson–interstitial lung disease. PubMed, ERJ Open Res, 2019 Feb 11;5(1). (Also see Lung Transplants)

Lung transplantation in scleroderma: recent advances and lessons. Both short–term and long–term outcomes following lung transplantation are comparable to counterparts with fibrotic lung disease or pulmonary arterial hypertension. PubMed, Curr Opin Rheumatol, 2018 Nov;30(6):562-569. (Also see Lung Transplants)

Lung transplantation in scleroderma compared with idiopathic pulmonary fibrosis (IPF) and idiopathic pulmonary arterial hypertension (IPAH). Patients with scleroderma who are recipients of lung transplantation experience similar rates of survival 2 years after the procedure compared with those with IPF or IPAH. Lung transplantation may represent a viable therapeutic option to consider for patients with end-stage lung disease due to scleroderma. PubMed, Arthritis Rheum.

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