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.

Lung Transplant Outcomes in Systemic Sclerosis (SSc) with Significant Esophageal Dysfunction: A Comprehensive Single Center Experience. Relative to other lung transplant indications, our SSc group experienced comparable survival, despite the high prevalence of severe esophageal dysfunction. PubMed, Ann Am Thorac Soc, 04/14/2016.

Survival of adults with systemic sclerosis following lung transplantation: A nationwide cohort study. A diagnosis of SSc may confer an increased risk of death 1 year following lung transplantation compared to a diagnosis of ILD, but this risk is similar to that of PAH, a widely accepted indication for lung transplantation. PubMed, Arthritis Rheumatol, 01/07/2015. (Also see What is Scleroderma?)

Improved transplant-free survival in patients with systemic sclerosis-associated pulmonary hypertension and interstitial lung disease (SSc-PH-ILD). Survival of patients with SSc-PH-ILD has modestly improved, and may partly be due to aggressive PAH-targeted therapy. PubMed, Arthritis Rheumatol, 2014 Apr 11. (Also see 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, 17 March 2014. (Also see Reflux, Heartburn, GERD)

Lung transplantation in patients with scleroderma. Lung transplantation represents a viable therapeutic option to consider for patients with end-stage lung disease due to systemic sclerosis (SSc) who have limited extrapulmonary manifestations. PubMed, Curr Opin Rheumatol, 2013 Nov;25(6):714-8.

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

Patients denied transplants as donor organs are discarded. Transplant candidates are dying even while viable organs are being thrown out, because government regulations have forced centers to focus on overall post-transplant survival rates instead of the well-being of individual patients. The Bulletin, 01/03/2014.

5 Discoveries That Will Change The Future of Organ Transplants. There are 75,660 active patients waiting for a transplant in the U.S., according to the the Organ Procurement and Transplantation Network. Some will never make it to the top of the list. Time Healthland, 06/06/2013.

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