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Long-term survival of lung transplantation for interstitial lung disease associated with connective tissue diseases: a study of 26 cases from a referral centre
D. Prieto-Peña1, A. Martínez-Meñaca2, M. Calderón-Goercke3, V.M. Mora-Cuesta4, S. Fernández-Rozas5, D. Iturbe-Fernández6, J.J. Gómez-Román7, J.M. Cifrián-Martínez8, S. Castañeda9, J.L. Hernández10, M.A. González-Gay11, R. Blanco12
- Epidemiology, Genetics and Atherosclerosis Research Group on Systemic Inflammatory Diseases, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain.
- Pneumology Division, Hospital Universitario Marqués de Valdecilla, Molecular Imaging Group IDIVAL, Santander, Spain.
- Epidemiology, Genetics and Atherosclerosis Research Group on Systemic Inflammatory Diseases, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain.
- Pneumology Division, Hospital Universitario Marqués de Valdecilla, Molecular Imaging Group IDIVAL, Santander, Spain.
- Pneumology Division, Hospital Universitario Marqués de Valdecilla, Molecular Imaging Group IDIVAL, Santander, Spain.
- Pneumology Division, Hospital Universitario Marqués de Valdecilla, Molecular Imaging Group IDIVAL, Santander, Spain.
- Pathology Division, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain.
- Pneumology Division, Hospital Universitario Marqués de Valdecilla, Molecular Imaging Group IDIVAL, Santander, Spain.
- Rheumatology Department, Hospital de la Princesa, IIS-Princesa, Cátedra UAM-Roche (EPID-Future), Universidad Autónoma de Madrid, Madrid, Spain.
- Epidemiology, Genetics and Atherosclerosis Research Group on Systemic Inflammatory Diseases, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain.
- Epidemiology, Genetics and Atherosclerosis Research Group on Systemic Inflammatory Diseases, Rheumatology Division, Hospital Universitario Marqués deValdecilla, IDIVAL, Santander; University of Cantabria, School of Medicine, Santander, Spain; and Cardiovascular Pathophysiology and Genomics Research Unit, School of Physiology, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa. miguelaggay@hotmail.com
- Epidemiology, Genetics and Atherosclerosis Research Group on Systemic Inflammatory Diseases, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain. rblanco@humv.es
CER12418
2020 Vol.38, N°4
PI 0615, PF 0620
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PMID: 31694743 [PubMed]
Received: 12/05/2019
Accepted : 22/07/2019
In Press: 17/10/2019
Published: 28/07/2020
Abstract
OBJECTIVES:
Interstitial lung disease (ILD) is a leading cause of mortality in patients with connective tissue diseases (CTD). Lung transplantation has become a viable option for patients with end-stage CTD-ILD. However, patients with CTD are often considered suboptimal candidates for lung transplantation because of concerns of worse outcomes. We assessed post-transplant survival of patients with CTD-ILD compared to patients with idiopathic pulmonary fibrosis (IPF).
METHODS:
Medical records of patients who underwent lung transplantation for CTD-ILD at a single referral centre for lung transplantation in Northern Spain between 1998 and 2018 were reviewed. This cohort was compared with patients with IPF (group-matched for age ±3.3 years, transplant year and use of basiliximab induction previous to transplant). Cumulative survival rates after transplantation were estimated by the Kaplan-Meier method and compared between groups using the log-rank test.
RESULTS:
We studied 26 patients with CTD-ILD and 26 patients with IPF. The underlying diseases of CTD-ILD patients were rheumatoid arthritis (n=9), scleroderma (n=6), Sjögren’s syndrome (n=4), ANCA-associated vasculitis (n=3), anti-synthetase syndrome (n=2), and dermatomyositis, systemic lupus erythematosus (1 each). Baseline characteristics were similar in both groups. CTD-ILD patients experienced acute graft rejection less commonly than those with IPF (32.0% vs. 62.5%; p=0.032). However, a non-statistically significant increased frequency of chronic graft rejection was observed in CTD-ILD patients (20.0% vs. 8.3%; p=0.417). In this regard, the 5-year cumulative survival rates after transplantation was reduced in CTD-ILD (42.4% vs. 65.8%) but the difference did not achieve statistical significance (p=0.075).
CONCLUSIONS:
Long-term post-transplant survival in Northern Spanish patients with CTD-ILD is reduced compared with IPF.