scholarly journals Lung Transplantation in a Patient with COPA Syndrome

2020 ◽  
Vol 2020 ◽  
pp. 1-2 ◽  
Author(s):  
Jorge M. Mallea ◽  
Anna Kornafeld ◽  
Andras Khoor ◽  
David B. Erasmus
Keyword(s):  
Pulmonary Function ◽  
Lung Transplantation ◽  
Survival Benefit ◽  
Lung Transplant ◽  
Autoimmune Disorder ◽  
Disease Recurrence ◽  
Antibody Mediated Rejection ◽  
Bilateral Lung ◽  
Copa Syndrome

COPA syndrome is a newly discovered, rare genetic autoimmune disorder, which can affect the lungs, joints, and kidneys. It is difficult to recognize, and the survival benefit of lung transplantation for these patients is not yet known. We present a case of a 24-year-old woman who received bilateral lung transplant for COPA syndrome. At 15 months posttransplant, her pulmonary function is stable with no episodes of acute cellular- or antibody-mediated rejection and no evidence of disease recurrence.

scholarly journals Outcome of Extracorporeal Photopheresis as an Add-On Therapy for Antibody-Mediated Rejection in Lung Transplant Recipients

2020 ◽  
Vol 47 (3) ◽  
pp. 205-213
Author(s):  
Alberto Benazzo ◽  
Nina Worel ◽  
Stefan Schwarz ◽  
Ulrike Just ◽  
Anna Nechay ◽  
...  
Keyword(s):  
Lung Transplantation ◽  
Lung Transplant ◽  
De Novo ◽  
Hla Class I ◽  
Class Ii ◽  
Class I ◽  
Extracorporeal Photopheresis ◽  
Transplant Recipients ◽  
Antibody Mediated Rejection ◽  
Lung Transplant Recipients

Introduction: The diagnosis and treatment of antibody-mediated rejection (AMR) after lung transplantation has recently gained recognition within the transplant community. Extracorporeal photopheresis (ECP), currently used to treat chronic lung allograft dysfunction, modulates various pathways of the immune system known to be involved in AMR. We hypothesize that adding ECP to established AMR treatments could prevent the rebound of donor-specific antibodies (DSA). Objectives: This study aimed to analyze the role of ECP as an add-on therapy to prevent the rebound of DSA. Methods: Lung transplant recipients who received ECP as an add-on therapy for pulmonary AMR between January 2010 and January 2019 were included in this single-center retrospective analysis. Baseline demographics of the patients, as well as their immunological characteristics and long-term transplant outcomes, were analyzed. Results: A total of 41 patients developed clinical AMR during the study period. Sixteen patients received ECP as an add-on therapy after first-line AMR treatment. Among the 16 patients, 2 (13%) had pretransplant DSA, both against human leukocyte antigen (HLA) class I (B38, B13, and C06). Fifteen patients (94%) developed de novo DSA (dnDSA), i.e., 10 (63%) against class I and 14 (88%) against class II. The median time to dnDSA after lung transplantation was 361 days (range 25–2,548). According to the most recent International Society of Heart and Lung Transplantation (ISHLT) consensus report, 2 (13%) patients had definite clinical AMR, 6 (38%) had probable AMR, and 7 (44%) had possible AMR. The median mean fluorescence intensity (MFI) of dnDSA at the time of clinical diagnosis was 4,220 (range 1,319–10,552) for anti-HLA class I and 10,953 (range 1,969–27,501) for anti-HLA class II antibodies. ECP was performed for a median of 14 cycles (range 1–64). MFI values of dnDSA against HLA classes I and II were significantly reduced over the treatment period (for anti-class I: 752; range 70–2,066; for anti-class II: 5,612; range 1,689–21,858). The 1-year survival rate was 55%. No adverse events related to ECP were reported in any of the patients. Conclusions: ECP is associated with a reduction of dnDSA in lung transplant recipients affected by AMR. Prospective studies are warranted to confirm the beneficial effects of ECP in the setting of AMR.

scholarly journals Implantable System for Treprostinil and Lung Transplantation: Case Series from Delivery for Pulmonary Arterial Hypertension Study

2021 ◽  
pp. 204589402199929
Author(s):  
Jeremy Feldman ◽  
Mardi Gomberg-Maitland ◽  
Shelly M Shapiro ◽  
Amy Lautenbach ◽  
Marty Morris ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lung Transplantation ◽  
Lung Transplant ◽  
Case Series ◽  
Patient Characteristics ◽  
Effective Strategies ◽  
Transplant Program ◽  
Pulmonary Arterial ◽  
Bilateral Lung

Background The implanted system for treprostinil (IST) has been described in previous publications. There is no information published about how to handle this system around lung or heart lung transplantation. We present the experience from the DelIVery for Pulmonary Arterial Hypertension (PAH) study. Methods Of the 60 subjects enrolled in the DelIVery study, seven subjects from five pulmonary arterial hypertension (PAH) centers had been listed for bilateral lung or heart and lung transplant, and were included in this analysis. All subjects were participating in the previously described DelIVery for PAH study. 1,2 Results Seven subjects with implanted pumps have been listed for lung or heart-lung transplant. Six subjects underwent lung or heart lung transplantation and one remains on the transplant list. Three different methods of patient management for transplant were used. In three subjects the implanted system was filled with saline prior to transplantation and treprostinil was infused via an external system. Three subjects had their drug-filled implanted pump and catheter system explanted at the time of transplant. One patient had the drug-filled implanted system removed prior to being listed for transplantation. Four subjects were hospitalized while waiting for transplantation. Conclusion The eight year experience from the DelIVery for PAH study confirms that the IST is not a barrier to safe lung or heart lung transplantation. The experience described here provides three effective strategies for managing the implanted system around lung or heart lung transplantation. The optimal strategy will depend on patient characteristics and lung transplant program preferences and wait list times.

scholarly journals Single-Lung Transplantation in the Setting of Aborted Bilateral Lung Transplantation

2011 ◽  
Vol 2011 ◽  
pp. 1-4
Author(s):  
Varun Puri ◽  
Tracey Guthrie ◽  
Masina Scavuzzo ◽  
Daniel Kreisel ◽  
Alexander S. Krupnick ◽  
...  
Keyword(s):  
Lung Transplantation ◽  
Lung Transplant ◽  
Hemodynamic Instability ◽  
Graft Dysfunction ◽  
Single Lung Transplantation ◽  
Bilateral Lung Transplantation ◽  
Single Lung Transplant ◽  
Lung Transplants ◽  
Bilateral Lung ◽  
The One

Background. The outcome of patients undergoing a single-lung transplant in the setting of an aborted bilateral lung transplant is unclear.Methods. A retrospective review of single lung transplants at an institutional program.Results. Of the 543 lung transplants performed over the last 10 years, 31 (5.7%) were single-lung transplants. Nineteen of 31 (61%) were planned single-lung transplants, while 12/31 (39%) were intraoperatively aborted, double lung transplants converted to single-lung transplants. The aborted and planned groups were similar in age, lung allocation score and NYHA status. The reasons for aborted double lung transplantation were cardiac/hemodynamic instability 4/12 (33%), difficult pneumonectomy 3/12 (25%), size mismatch 4/12(33%), and technical issues 1/12 (8%). The aborted group had higher CPB utilization (5/12 versus 1/19, ), similar ischemic times (260 versus 234 min) and similar incidence of grade 3 primary graft dysfunction (6/12 versus 3/19, ). ECMO was required for graft dysfunction in 2 patients in the aborted group. The one and two-year survival was 84% and 79% in the planned group and 62% and 52% in the aborted group, respectively.Conclusions. Patients undergoing single-lung transplantation in the setting of an aborted bilateral lung transplant may be at a higher risk of worse outcomes.

scholarly journals Lung Transplantation and the Era of the Sensitized Patient

2021 ◽  
Vol 12 ◽  
Author(s):  
Katherine A. Young ◽  
Hakim A. Ali ◽  
Kristi J. Beermann ◽  
John M. Reynolds ◽  
Laurie D. Snyder
Keyword(s):  
Risk Factor ◽  
Lung Transplantation ◽  
Lung Transplant ◽  
Management Strategies ◽  
Long Term Outcomes ◽  
Hla Antibodies ◽  
Antibody Mediated Rejection ◽  
The Past ◽  
Transplant Candidates

Long term outcomes in lung transplant are limited by the development of chronic lung allograft dysfunction (CLAD). Within the past several decades, antibody-mediated rejection (AMR) has been recognized as a risk factor for CLAD. The presence of HLA antibodies in lung transplant candidates, “sensitized patients” may predispose patients to AMR, CLAD, and higher mortality after transplant. This review will discuss issues surrounding the sensitized patient, including mechanisms of sensitization, implications within lung transplant, and management strategies.

scholarly journals Refractory chylous effusions in lymphangioleiomyomatosis patient post lung transplant

2020 ◽  
Vol 8 ◽  
pp. 2050313X2092133
Author(s):  
Samuel Jacob ◽  
Mojahid Ali ◽  
Magdy M El-Sayed Ahmed ◽  
Maxim Itkin ◽  
Tathagat Narula ◽  
...  
Keyword(s):  
Lung Transplantation ◽  
Young Women ◽  
Surgical Procedures ◽  
Lung Transplant ◽  
Chylous Ascites ◽  
Unknown Etiology ◽  
Bilateral Lung Transplantation ◽  
Systemic Disorder ◽  
Bilateral Lung

Lymphangioleiomyomatosis is a rare systemic disorder of unknown etiology that affects young women almost exclusively. Chylous effusions are known to be associated with lymphangioleiomyomatosis and may be difficult to treat. We present the case of a 37-year-old female who received bilateral lung transplantation for lymphangioleiomyomatosis complicated by refractory chylothorax and chylous ascites, ultimately controlled through repeated, open surgical procedures and percutaneous lymphatic embolization interventions. The combined surgical and interventional radiological approach, while not novel in their own right, suggests that a multi-modal interventional approach may be required in refractory cases.

Antibody-Mediated Rejection Management Following Lung Transplantation

2021 ◽  
pp. 106002802110124
Author(s):  
Kristen Neuhaus ◽  
Benjamin Hohlfelder ◽  
Jessica Bollinger ◽  
Marcus Haug ◽  
Heather Torbic
Keyword(s):  
Lung Transplantation ◽  
Lung Transplant ◽  
Management Strategies ◽  
Organ Transplant ◽  
Outcome Analysis ◽  
Solid Organ ◽  
Transplant Recipients ◽  
Antibody Mediated Rejection ◽  
Patient Admissions ◽  
Lung Transplant Recipients

Background Although antibody-mediated rejection (AMR) is described in other solid organ transplant populations, the literature describing the management following lung transplantation is limited. Objective The purpose of this study is to evaluate the management strategies of AMR in lung transplant recipients. Methods This single-center, retrospective study described the management of AMR in adult lung transplant recipients who received treatment with rabbit antithymocyte globulin, bortezomib, rituximab, intravenous immune globulin (IVIG), and/or plasmapheresis between September 2015 and June 2019. Results A total of 270 medication orders for 55 patient admissions were included in the primary outcome analysis. The most commonly used regimen consisted of IVIG, plasmapheresis, and rituximab (49.1%; n = 27), followed by IVIG and plasmapheresis alone (27.3%, n = 15). A total of 51 patients (93%) received plasmapheresis as part of their AMR treatment, with a median of 4 [3, 5] sessions per encounter; 86% of patients with positive donor-specific antibodies (DSAs) had a reduction in DSAs following AMR treatment. Overall, 23.5% of patients had noted allograft failure or need for retransplantation. A total of 10 patients died during the AMR treatment hospital admission, and an additional 11 patients died within 1 year of the initial encounter. Conclusion and Relevance This represents the largest report describing management strategies of AMR in lung transplant recipients. Although practice varied, the most commonly used regimen consisted of plasmapheresis, IVIG, and rituximab.

scholarly journals Suspected pleuroparenchymal fibroelastosis relapse after lung transplantation: a case report and literature review

2019 ◽  
Vol 5 (4) ◽  
pp. 20190040
Author(s):  
Edoardo Rasciti ◽  
Alessandra Cancellieri ◽  
Micaela Romagnoli ◽  
Andrea Dell'Amore ◽  
Maurizio Zompatori
Keyword(s):  
Lung Transplantation ◽  
Lung Transplant ◽  
Medical Literature ◽  
Therapeutic Option ◽  
Histopathological Analysis ◽  
Visceral Pleura ◽  
Pleuroparenchymal Fibroelastosis ◽  
Bilateral Lung Transplantation ◽  
Respiratory State ◽  
Bilateral Lung

Pleuroparenchymal fibroelastosis (PPFE) is a very rare lung disease characterized by dense fibrous thickening of the visceral pleura and intraalveolar fibrosis containing prominent elastosis, with typical upper lobe predominance. PPFE usually shows progressive restrictive ventilatory impairment refractory to medical treatment; bilateral lung transplantation currently remains the only available therapeutic option. In this report, we describe a case of suspected PPFE relapse after lung transplantation that, to our knowledge, has never been described in the medical literature. A 48-year-old male with idiopathic pleuroparenchymal fibroelastosis underwent a bilateral lung transplant in our department. 8 months later, he presented with progressively worsening clinical condition, his respiratory state gradually deteriorated. High-resolution CT again showed bilateral diffused parenchymal consolidations, with prevalence in the upper lobes and subpleural regions. A PPFE relapse was therefore suspected, so he was listed for lung retransplantation, which was performed ten months after the first transplant. Histopathological analysis of the second explanted lung again confirmed the diagnosis of PPFE. The case highlights the possibility of PPFE relapse after lung transplantation, that may add to the increasing evidence of an underlying auto-immune mechanism contributing to its pathogenesis.

scholarly journals Canadian Respirologists’ Experience with Lymphangioleiomyomatosis

2002 ◽  
Vol 9 (6) ◽  
pp. 413-416
Author(s):  
Stacey M Pollock-BarZiv ◽  
Marsha M Cohen ◽  
Heather Maclean ◽  
Gregory P Downey
Keyword(s):  
Literature Review ◽  
Lung Transplantation ◽  
Pulmonary Disease ◽  
Research Funding ◽  
Lung Transplant ◽  
Case Reports ◽  
Wait List ◽  
Patient Support ◽  
Bilateral Lung ◽  
Research Service

BACKGROUND: Lymphangioleiomyomatosis (LAM) is a rare pulmonary disease occurring primarily in women. A literature review of LAM in Canada found sporadic mention of LAM in case reports or within lung transplant studies. The LAM Foundation, a patient support and research funding organization, lists 23 Canadian patients in its database. The present study was designed to assess the scope of LAM across Canada and to identify potential patients for further evaluation. OBJECTIVES: To ascertain Canadian respirologists’ experience with patients with LAM (current and historical), lung transplantation (single or bilateral) and deaths due to LAM, and awareness of the LAM Foundation.METHODS: Four hundred twelve brief surveys were sent anonymously to members of the Canadian Lung Association (inserted in their newsletters) to ascertain the experience of Canadian respirologists with LAM.RESULTS: One hundred twelve surveys were returned (27%). Fifty-one respondents had 'ever' been involved in the care of at least one patient with LAM; eight had cared for three or more patients. At the time of the study, 26 respondents were following a total of 46 patients with LAM; 22 of the 51 respirologists (43%) who had ever cared for a patient with LAM reported the death of a patient. Thirty-three patients had been put on wait lists for transplantation; six died while on the wait list. Nineteen patients underwent lung transplantation - six single-lung recipients and 13 bilateral lung recipients. Of the 51 respirologists who had ever cared for a patient with LAM, only 30 (61%) were aware of the LAM Foundation’s services. Of the 112 respondents, only 47 (43%) were aware of the LAM Foundation.CONCLUSIONS: This study identified a moderate level of awareness of a significant existing patient support and research service (the LAM Foundation). There were many patients with LAM who were unknown to the LAM Foundation and could benefit from its resources. Results suggest that there may be more patients with LAM in Canada than are reported in the existing literature.

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