Outcomes in systemic sclerosis-related lung disease after lung transplantation
Open accessAbstract
BACKGROUND: Lung disease is the leading cause of death in systemic sclerosis (SSc). The diagnosis of SSc-related lung disease (SSc-LD) is often a contraindication to lung transplantation (LT) due to concerns that extrapulmonary involvement will yield worse outcomes. We sought to evaluate posttransplantation outcomes in persons with SSc-LD with esophageal involvement compared with persons with nonconnective tissue disease-related interstitial lung disease (nCTD-ILD). METHODS: From 1998 to 2012, persons undergoing LT for SSc-LD were age and gender matched in a 2:1 fashion to controls undergoing LT for nCTD-ILD. Esophageal function was assessed by pH testing and manometry. We defined esophageal dysfunction as the presence of a DeMeester score >14 or dysmotility more severe than "mild nonspecific disorder". The primary outcome was posttransplantation survival. Secondary outcomes included freedom from bronchiolitis obliterans syndrome (fBOS) and rates of acute rejection. Survival and fBOS were estimated with Kaplan-Meier methods. Acute rejection was compared with Student's t test. RESULTS: Survival was similar in 23 persons with SSc-LD and 46 controls who underwent LT (P = 0.47). For the SSc-LD group, 1- and 5-year survival was 83% and 76% compared with 91% and 64% in the nCTD-ILD group, respectively. There were no differences in fBOS (P = 0.83). Rates of acute rejection were less in SSc-ILD (P = 0.05). Esophageal dysfunction was not associated with worse outcomes (P>0.55). CONCLUSIONS: Persons with SSc-LD appear to have similar survival and fBOS as persons transplanted for nCTD-ILD. The risk of acute rejection after transplantation may be reduced in persons with SSc-LD. Esophageal involvement does not appear to impact outcomes.
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Studies of scleroderma recipients undergoing lung transplant at centers with expertise have demonstrated that esophageal dysfunction does not appear to impact outcomes.
“studies of scleroderma recipients, undergoing lung transplant at centers with expertise with this patient population, have demonstrated that esophageal dysfunction does not appear to impact outcomes.(36, 37)”
From Consensus document for the selection of lung transplant candidates: An update from the International Society for Heart and Lung Transplantation. (2021) · cites this paper as [36]
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“36,37 Hematologic abnormalities : Thrombocytopenia, leukopenia, or anemia may contribute to peri-operative complications and may limit use of optimal maintenance immunosuppression and necessary antimicrobial prophylaxis following lung transplant.”
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- Authors
- Sottile, P. D., Iturbe, D., Katsumoto, T. R., Connolly, M. K., Collard, H. R., Leard, L. A., Hays, S., Golden, J. A., Hoopes, C., Kukreja, J., & Singer, J. P.
- Affiliations
- University of California, San Francisco (US)
- University of Kentucky (US)
- Corresponding author
- Singer, Jonathan P.
- ORCID
- Venue
- Transplantation
- Published
- 2013-04-15
- Volume
- 95
- Issue
- 7
- Pages
- 975-980
- ISSN
- 0041-1337 (ISSN-L)
- PMC
- PMC3616420
- OpenAlex
- W1965553464
- Semantic Scholar
- 0ab77302ae4fe884509e613cec8661232b721366
- Cited by
- 85
- References
- 25
- Citations by year
- 2026: 4 · 2025: 2 · 2024: 4 · 2023: 8 · 2022: 2 · 2021: 9 · 2020: 8 · 2019: 10 · 2018: 9 · 2017: 6 · 2016: 9 · 2015: 6 · 2014: 6 · 2013: 2
- FWCI
- 7.55
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Transplantation (publishedVersion)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- PubMed Central (acceptedVersion)
- eScholarship (California Digital Library) (submittedVersion)
- Funders
- National Heart, Lung, and Blood Institute (T35HL007690, F32HL107003, K23HL111115)
- Topics
- Health Sciences › Medicine › Surgery › Transplantation: Methods and Outcomes
- Health Sciences › Medicine › Pathology and Forensic Medicine › Systemic Sclerosis and Related Diseases
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Pulmonary Hypertension Research and Treatments
- Keywords
- systemic sclerosis, lung transplantation, interstitial lung disease, esophageal involvement, esophageal dysfunction, bronchiolitis obliterans syndrome, acute rejection, post-transplant survival, extrapulmonary involvement
- MeSH terms
- Acute Disease, Adult, Aged, Bronchiolitis Obliterans, Female, Graft Rejection, Humans, Male, Middle Aged, Retrospective Studies, Risk Factors, San Francisco, Scleroderma, Systemic, Time Factors, Esophageal Motility Disorders, Multivariate Analysis, Chi-Square Distribution, Logistic Models, Lung Transplantation, Treatment Outcome, Lung Diseases, Interstitial, Risk Assessment, Kaplan-Meier Estimate
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