SESSION TITLE: Lung Pathology Case Report Posters
SESSION TYPE: Affiliate Case Report Poster
PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM
INTRODUCTION: Chronic Lung Allograft Dysfunction (CLAD) is a major cause of late mortality in lung transplant patients. The hallmark of chronic rejection is fall in FEV1 with an obstructive ventilatory defect, or bronchiolitis obliterans syndrome. However, a restrictive phenotype of CLAD has been recognized which occurs in approximately 30% of CLAD patients.
CASE PRESENTATION: 23 year old woman with cystic fibrosis underwent double lung transplant with post-operative hospital course complicated by pneumonia and intracranial hemorrhage requiring hemicraniectomy. Following this she had hypoxemic and hypercarbic respiratory failure. Transbronchial biopsy two months after transplant showed diffuse alveolar damage with eosinophils. She had positive donor specific antibodies and so was treated for acute antibody mediated rejection. Open lung biopsy performed due to worsening hypoxemia showed acute fibrinoid organizing pneumonia. She was diagnosed with CLAD and trial of pirfenidone given. Her condition continued to worsen and she was made comfort care.
DISCUSSION: Survival after lung transplantation is significantly shorter than survival after transplant of other solid organs. Chronic rejection limits the 5-year survival to approximately 55%. Acute fibrinoid organizing pneumonia is diagnosed on transbronchial biopsies, characterized by patent bronchioles with peribronchial and alveolar fibrin deposition with little or no inflammation. They present with non-obstructive pulmonary function defect and bilateral infiltrates. Patients deteriorate quickly and pulmonary function testing is not possible and in them biopsy may suggest rCLAD. Considering how sick our patient was, preventing PFT measurements, we believe the initial transbronchial biopsy showing diffuse alveolar damage may be the start of rCLAD. What also needs to be looked at, is whether the intracranial hemorrhage triggered her chronic rejection.
CONCLUSIONS: The importance of recognizing AFOP is due to the significantly worse survival among patients with rCLAD when compared to recipients with BOS. Treatment is limited and case reports demonstrate the potential of perfinidone slowing the evolution of rCLAD, but definitive studies are required to diagnose and treat this particular condition.
Reference #1: Verleden SE, Ruttens D. et al; Restrictive chronic lung allograft dysfunction: Where are we now? J Heart Lung Transplant 2015(article in press)
Reference #2: Verleden GM, Raghu G et al. A new classification system for chronic lung allograft dysfunction. J Heart Lung Transplant, 2014; 33: 127-33
DISCLOSURE: The following authors have nothing to disclose: Muhammad Rashad, Kriti Lonial, Linh Truong, Jaymica Patel, Mark Valdez, Sivagini Ganesh
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