SESSION TITLE: Diffuse Lung Disease Cases
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Monday, October 26, 2015 at 03:15 PM - 04:15 PM
INTRODUCTION: Pulmonary alveolar proteinosis occurs due to the accumulation of lipoproteinaceous surfactant like materials. This condition is extremely rare in lung transplant recipients. We here present a case of left single lung transplant with right sided native lung with fibrosis due to IPF, diagnosed with PAP occurring in lung allograft 1 year after transplant. He was successfully treated with whole lung lavage of the left lung with the support of ECMO.
CASE PRESENTATION: 75 yr old man with history of left single lung transplantation done 1 year ago for IPF. His other comorbidities included hypertension, dyslipidemia, CKD stage III, diabetes mellitus and GERD. His immunosuppressive regimen included tacrolimus, prednisone and mycophenolate mofetil. He presented with worsening dyspnea and hypoxemia with exertion. CT chest showed ground glass opacities in the transplanted lung with areas of "crazy paving pattern". Patient underwent bronchoscopy, bronchoalveolar lavage and transbronchial lung biopsy done on left side and was diagnosed with PAP. Staining for PJP was negative. As the patient had left single lung transplant and his right sided native lung had advanced fibrosis, he underwent whole lung lavage with ECMO support. After the lavage, his left lung was connected to mechanical ventilator and ECMO was decanulated. He was later started on GM-CSF; Sargramostim. His symptoms improved back to baseline prior to discharge which was within 72 hours of the procedure.
DISCUSSION: Pulmonary alveolar proteinosis occurs due to accumulation on lipoproteinaceous material in the alveoli leading to radiologic abnormality as well as hypoxemia. The occurrence of PAP in lung transplant patients is extremely rare. There are reports of organ transplant recipients on sirolimus developing PAP, but our patient was not on siroliums.
CONCLUSIONS: PAP should be considered as a differential diagnosis in transplant patients presenting with hypoxemia and abnormal chest imaging such as ground glass opacity. Such patients needs to be followed closely for recurrence after the initial treatment. Secondary causes for PAP also needs to be looked into in these patients.
Reference #1: Recurrent alveolar proteinosis following double lung transplantation. Parker et. Al; Chest. 1997 May;111(5):1457-8
Reference #2: Whole-lung lavage for pulmonary alveolar proteinosis; Michaud G et. Al; Chest. 2009 Dec;136(6):1678-81
Reference #3: Pulmonary alveolar proteinosis in a kidney transplant: a rare complication of sirolimus; Kadikoy H et al Nephrol Dial Transplant. 2010 Aug;25(8):2795-8
DISCLOSURE: The following authors have nothing to disclose: Joseph Thachuthara-George, Sinha Neeraj, Brian Buckner, Babith Mankidy, Scott Scheinin, Thomas Kaleekal
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