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Pulmonary Fibrosis Treatments: Lung Transplants

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
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Landscape by Janet Paulmenn, ISN ArtistLung Transplants, in most cases, are a treatment of last resort. This is because lung transplants are a very invasive procedure and they require a lung donor.

This procedure is also extremely expensive compared to other treatments. However, lung transplants have been proven to be effective for patient with scleroderma related pulmonary fibrosis.

Lung Transplant Discussions on Sclero Forums conversations including transplant testing and evaluation. ISN Sclero Forums.

Factors associated with esophageal motility improvement after bilateral lung transplant (LTx) in patients with an aperistaltic esophagus. Patients with esophageal aperistalsis and obstructive lung disease or pulmonary arterial hypertension, but not patients with restrictive lung disease and scleroderma, are likely to have improved esophageal peristalsis post-LTx. PubMed, J Thorac Cardiovasc Surg, 2021 Jan 9;S0022-5223(21)00027-1. (Also see Dysmotility Syndrome)

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 for Pulmonary Hypertension)

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 for Pulmonary Hypertension)

Usefulness of pH monitoring in predicting the survival status of patients with scleroderma awaiting lung transplantation. Esophageal pH monitoring could identify those for whom laparoscopic antireflux surgery should be performed quicker to prevent GERD and its detrimental effects in patients awaiting lung transplantation. Journal of Surgical Research. (Also see Reflux, Heartburn, GERD)

United Network for Organ Sharing. Resources for all aspects of organ transplantation. UNOS.

Lung Transplant. Lung transplant can benefit patients with advanced lung disease for which medical therapies are not effective. These lung diseases include pulmonary fibrosis, connective tissue disease, emphysema, pulmonary hypertension, sarcoidosis, lymphangioleiomyomatosis (LAM), pneumoconiosis, bronchoalveolar carcinoma (BAC), bronchiectasis, and cystic fibrosis (CF). Duke Medicine.

NHS Blood and Transplant. Resources for all types of transplants in the United Kingdom (UK). NHS.

Media Stories

Breakthrough shows a discarded diabetes drug could prevent life–threatening complications after heart transplant surgery. A discarded diabetes drug could be the key to beating organ transplant rejection, a landmark study has found. Mail Online.

Go to Pulmonary Hypertension

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