Pulmonary alveolar proteinosis after lung transplantation

Divithotawela, Chandima; Apte, Simon H.; Tan, Mingming; Silva, Tharushi Ayanthika de; Chambers, Daniel C.

doi:10.1002/rcr2.566

Cited by 7 publications

(4 citation statements)

References 7 publications

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“…To date, 14 cases of PAP after LTx, including the present two cases have been reported (Table 1 ). 2 , 3 , 4 , 5 , 6 , 7 , 8 The median age was 57.5 years (range, 4–69), and 71% were men. The patients received a single LTx (36%), bilateral LTx (57%), or heart‐lung transplantation (7%) for interstitial pneumonia (IP) (71%), chronic obstructive pulmonary disease (14%), or others (14%).…”

Section: Discussionmentioning

confidence: 99%

Pulmonary alveolar proteinosis after lung transplantation: Two case reports and literature review

Kawana

Miyoshi

Tanaka

et al. 2023

Respirology Case Reports

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Pulmonary alveolar proteinosis (PAP) affecting transplanted lungs is not well recognized. Herein, we report two cases of PAP after lung transplantation (LTx). The first case was a 4‐year‐old boy with hereditary pulmonary fibrosis who underwent bilateral LTx and presented with respiratory distress on postoperative day (POD) 23. He was initially treated for acute rejection, died due to infection on POD 248, and was diagnosed with PAP at autopsy. The second case involved a 52‐year‐old man with idiopathic pulmonary fibrosis who underwent bilateral LTx. On POD 99, chest computed tomography revealed ground‐glass opacities. Bronchoalveolar lavage and transbronchial biopsy led to a diagnosis of PAP. Follow‐up with immunosuppression tapering resulted in clinical and radiological improvement. PAP after lung transplantation mimics common acute rejection; however, is potentially transient or resolved with tapering immunosuppression, as observed in the second case. Transplant physicians should be aware of this rare complication to avoid misconducting immunosuppressive management.

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Section: Discussionmentioning

confidence: 99%

Pulmonary alveolar proteinosis after lung transplantation: Two case reports and literature review

Kawana

Miyoshi

Tanaka

et al. 2023

Respirology Case Reports

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“…These are the only reported cases of allo-HSCT in proven hereditary PAP, as far as we know. Of note, recurrence of autoimmune PAP and development of secondary PAP have also been described as a late complication of both allo-HSCT ( 29 ) or lung transplantation ( 30 ), emphasizing the need for post-transplant follow-up.…”

Section: Discussionmentioning

confidence: 99%

Allogeneic Hematopoietic Stem Cell Transplantation After Prior Lung Transplantation for Hereditary Pulmonary Alveolar Proteinosis: A Case Report

Beeckmans

Ambrocio²,

Bos³

et al. 2022

Front. Immunol.

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Pulmonary alveolar proteinosis (PAP) is a rare, diffuse lung disorder characterized by surfactant accumulation in the small airways due to defective clearance by alveolar macrophages, resulting in impaired gas exchange. Whole lung lavage is the current standard of care treatment for PAP. Lung transplantation is an accepted treatment option when whole lung lavage or other experimental treatment options are ineffective, or in case of extensive pulmonary fibrosis secondary to PAP. A disadvantage of lung transplantation is recurrence of PAP in the transplanted lungs, especially in hereditary PAP. The hereditary form of PAP is an ultra-rare condition caused by genetic mutations in genes encoding for the granulocyte macrophage-colony stimulating factor (GM-CSF) receptor, and intrinsically affects bone marrow derived-monocytes, which differentiate into macrophages in the lung. Consequently, these macrophages typically display disrupted GM-CSF receptor-signaling, causing defective surfactant clearance. Bone marrow/hematopoietic stem cell transplantation may potentially reverse the lung disease in hereditary PAP. In patients with hereditary PAP undergoing lung transplantation, post-lung transplant recurrence of PAP may theoretically be averted by subsequent hematopoietic stem cell transplantation, which results in a graft-versus-disease (PAP) effect, and thus could improve long-term outcome. We describe the successful long-term post-transplant outcome of a unique case of end-stage respiratory failure due to hereditary PAP-induced pulmonary fibrosis, successfully treated by bilateral lung transplantation and subsequent allogeneic hematopoietic stem cell transplantation. Our report supports treatment with serial lung and hematopoietic stem cell transplantation to improve quality of life and prolong survival, without PAP recurrence, in selected patients with end-stage hereditary PAP.

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“…Interestingly PAP can occur in lung allografts of patients with a different primary disease as well. There are case reports of PAP occurrence after LTx performed for IPF, Eisenmerger's syndrome and pulmonary hypertension [ 91 – 93 ]. One case with acute myeloid leukaemia appearing 5 years after the LTx developed PAP after receiving chemotherapy and had a bacterial pneumonia leading to death [ 94 ].…”

Section: Pulmonary Alveolar Proteinosismentioning

confidence: 99%

Recurrence of primary disease following lung transplantation

et al. 2022

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Lung transplant has become definitive treatment for patients with several end-stage lung diseases. Since the first attempted lung transplantation in 1963, survival has significantly improved due to advancement in immunosuppression, organ procurement, ex-vivo lung perfusion, surgical techniques, prevention of chronic lung allograft dysfunction, and bridging to transplant using extra-corporeal membrane oxygenation. Despite a steady increase in number of lung transplantations each year, there is still a huge gap between demand and supply of organs available, and work continues to select recipients with potential for best outcomes. According to review of the literature, there are some rare primary diseases that may recur following transplantation. As the number of lung transplants increase, we continue to identify disease processes at highest risk for recurrence, thus shaping our future approaches. While the aim of lung transplantation is improving survival and quality of life, choosing the best recipients is crucial due to shortage of donated organs. Here we discuss the common disease processes that recur and highlight its impact on overall outcome following lung transplantation.

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Pulmonary alveolar proteinosis after lung transplantation

Cited by 7 publications

References 7 publications

Pulmonary alveolar proteinosis after lung transplantation: Two case reports and literature review

Pulmonary alveolar proteinosis after lung transplantation: Two case reports and literature review

Allogeneic Hematopoietic Stem Cell Transplantation After Prior Lung Transplantation for Hereditary Pulmonary Alveolar Proteinosis: A Case Report

Recurrence of primary disease following lung transplantation

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