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Updated: Nov 16, 2025

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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
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Systemic Sclerosis: Current State and Survival After Lung Transplantation
Artem Minalyan1, Lilit Gabrielyan2, Shristi Khanal1
1Internal Medicine, Abington Hospital-Jefferson Health, Abington, USA.
Cureus
|February 25, 2021
Summary
Systemic sclerosis (SSc) patients now face pulmonary issues like interstitial lung disease and pulmonary arterial hypertension as leading causes of death. This review examines lung transplantation outcomes for SSc patients with these severe lung conditions.
Area of Science:
- Immunology
- Pulmonology
- Transplantation Medicine
Background:
- Systemic sclerosis (SSc) is an autoimmune disease affecting skin and internal organs.
- Pulmonary manifestations, including interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH), are now the primary cause of mortality in SSc patients.
- Lung transplantation for SSc was historically limited due to concerns about extrapulmonary disease and survival rates.
Purpose of the Study:
- To review recent advancements in evaluating and managing pulmonary manifestations of SSc.
- To systematically assess survival data for SSc patients following lung transplantation.
Main Methods:
- Systematic literature review of studies on SSc pulmonary manifestations.
- Analysis of survival outcomes in SSc patients post-lung transplantation.
Main Results:
- Angiotensin-converting enzyme inhibitors (ACEIs) have reduced mortality from scleroderma renal crisis (SRC).
- Pulmonary disease (ILD and PAH) is the leading cause of death in SSc.
- Data on lung transplant survival in SSc patients is being systematically evaluated.
Conclusions:
- Pulmonary complications are the main threat to SSc patients.
- Lung transplantation is a viable option for select SSc patients.
- Further research is needed to optimize lung transplant outcomes in SSc.

