Bilateral lung transplant for a connective tissue disorder: esophageal motility and 3-year survival

2021 ◽  
Author(s):  
Máté Csucska ◽  
Deepika Razia ◽  
Takahiro Masuda ◽  
Ashraf Omar ◽  
Luca Giulini ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Esophageal Motility ◽  
Lung Transplant ◽  
Connective Tissue Disorder ◽  
Bilateral Lung

O15 ESOPHAGEAL MOTILITY OF LUNG TRANSPLANT RECIPIENTS DIAGNOSED WITH SYSTEMIC CONNECTIVE TISSUE DISORDER: A SINGLE-CENTER EXPERIENCE

2019 ◽  
Vol 32 (Supplement_2) ◽  
Author(s):  
Mate Csucska ◽  
Sumeet K Mittal ◽  
Takahiro Masuda ◽  
Michael A Smith ◽  
Rajat Walia ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Esophageal Motility ◽  
Lung Transplant ◽  
Connective Tissue Disorder ◽  
Transplant Recipients ◽  
Ineffective Esophageal Motility ◽  
Esophageal Body ◽  
Body Motility ◽  
Lung Transplant Recipients ◽  
Esophageal Body Motility

Abstract Aim To examine the esophageal motility of lung transplant recipients diagnosed with systemic connective tissue disorder, and to evaluate the changes Backgrounds The decision to offer lung transplantation to patients diagnosed with a systemic connective tissue disorder (CTD) is frequently complicated by associated esophageal dysmotility. Absent or ineffective esophageal motility and associated gastroesophageal reflux disease are serious risk factors of posttransplant bronchiolitis obliterans syndrome and subsequent allograft dysfunction. The aim of our study was to evaluate esophageal motility of lung transplant recipients with systemic CTDs, focusing on 1-year survival. Methods After IRB approval of this retrospective cohort study, we reviewed patients who underwent lung transplant at our institution between January 2012 and December 2017. Patients with systemic CTDs such as scleroderma, rheumatoid arthritis, systemic lupus erythematosus, polymyositis, dermatomyositis, mixed connective tissue disorder, and Sjögren syndrome were included. Endoscopy studies, high-resolution manometry (HRM), and ambulatory pH studies were re-evaluated. Results A total of 545 patients underwent lung transplant during study period; of these, 34 met study criteria. Detailed demographic characteristics of the study cohort are shown in Table 1. The overall 1-year survival was 85.3%. Of the 34 patients diagnosed with a systemic CTD (mean age: 59.24 ± 10.44 years; 24/34 women [70.6%]), 27 had both pre- and posttransplant esophageal function testing data available. 7 patients were diagnosed with complete aperistalsis, 5 patients had ineffective esophageal motility, and the remaining 15 patients had normal or hypercontractile esophageal motility (Figure 1). Pathologic distal acid exposure in each of these groups was 57.1%, 40.0%, and 26.7%, respectively. Seven patients with aperistalsis (5 with scleroderma, 1 with polymyositis, and 1 with dermatomyositis) showed no improvement in esophageal body motility, regardless of underlying CTD. 1-year survival in these patients was 55.5%. Five patients with ineffective esophageal motility (3 with rheumatoid arthritis, 1 with scleroderma, 1 with mixed connective tissue disorder) showed significant peristaltic improvement (mean positive change in distal contractile integral: 1288 ± 391.3 mmHg*cm*s and in the number of normal swallows 6.4 ± 1.4 out of 10). All 5 patients with ineffective esophageal motility were alive 1 year posttransplant. Conclusion In our experience, preexisting absent esophageal body motility (i.e., peristalsis) is unlikely to improve after transplantation in patients with systemic CTDs. This cohort of patients appeared to have a significantly decreased 1-year survival. In contrast, ineffective esophageal body motility improved significantly after transplant and was associated with excellent 1-year survival. Detailed esophageal evaluation is necessary in patients with a systemic connective tissue disorder to minimize the risk of future allograft injury.

Long-Term Outcome after Aortic Valve-Sparing Root Surgery in Patients with Connective Tissue Disorder

2020 ◽  
Author(s):  
J. Petersen ◽  
D. Gaekel ◽  
E. Girdauskas ◽  
H. Reichenspurner ◽  
C. Detter
Keyword(s):  
Aortic Valve ◽  
Connective Tissue ◽  
Connective Tissue Disorder ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Valve Sparing

scholarly journals Disseminated Mycobacterium Fortuitum Infection in Bilateral Lung Transplant Recipient

2021 ◽  
Vol 40 (4) ◽  
pp. S514
Author(s):  
C. Nichols ◽  
M. Howsare ◽  
D.R. Nunley ◽  
S. Sarwar ◽  
M. Sobhanie
Keyword(s):  
Transplant Recipient ◽  
Lung Transplant ◽  
Lung Transplant Recipient ◽  
Mycobacterium Fortuitum ◽  
Bilateral Lung

scholarly journals Elevated Liver Function in a Bilateral Lung Transplant Recipient with Scleroderma: Is the Medication to Blame?

2021 ◽  
Vol 40 (4) ◽  
pp. S511
Author(s):  
M.T. Olson ◽  
A. Omar ◽  
S. McCartney ◽  
H. Mohamed ◽  
B. Buddhdev ◽  
...  
Keyword(s):  
Liver Function ◽  
Transplant Recipient ◽  
Lung Transplant ◽  
Lung Transplant Recipient ◽  
Bilateral Lung

scholarly journals Atypical presentation of rheumatoid arthritis: a case of massive bilateral pleural effusions in a patient with Caplan syndrome

2019 ◽  
Vol 12 (10) ◽  
pp. e231899
Author(s):  
Yinglun Wu ◽  
Philip C Dittmar
Keyword(s):  
Rheumatoid Arthritis ◽  
Atrial Fibrillation ◽  
Pleural Effusion ◽  
Connective Tissue ◽  
Minimal Change Disease ◽  
Connective Tissue Disorder ◽  
Minimal Change ◽  
Atypical Presentation ◽  
Pleural Effusions ◽  
Recent Treatment

Rheumatoid arthritis (RA) is a common connective tissue disorder affecting the synovial joints. In patients with RA, involvement of the lungs occurs in 30%–40% of cases while pleural effusions occur in only 3%–5%. However, the majority of RA-associated pleural effusions are small, unilateral and asymptomatic. We present a case of massive bilateral pleural effusions in a patient with established rheumatoid pneumoconiosis (Caplan syndrome). Interestingly, the pleural effusion occurred following recent treatment for minimal change disease and atrial fibrillation.

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