SESSION TITLE: Pulmonary Vascular Disease Case Report Posters
SESSION TYPE: Affiliate Case Report Poster
PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM
INTRODUCTION: Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary arterial hypertension (PAH) associated with poor prognosis and often refractory to PAH-targeted therapy. While it has been reported in association with hematopoietic stem cell transplantation (HSCT) and various connective tissue diseases, to our knowledge de novo PVOD has never been reported in association with orthotopic liver transplantation (OLT) or autoimmune hepatitis (AIH).
CASE PRESENTATION: We report a 49-year old female with history of cirrhosis from AIH who had undergone OLT seven years prior to this presentation. She presented with arthrlagias and one-week of progressive exertional dyspnea. Echocardiography and right heart catheterization were consistent with severe PAH with right ventricular dysfunction, new since OLT. Liver biopsy for concomitant elevated liver enzymes was consistent with immune-mediated hepatitis. She was started on diuretic therapy, escalating epoprostenol infusion, and inhaled nitric oxide. Immunosuppression was also intensified for possible AIH versus graft rejection. Despite therapy, she developed decompensated right ventricular failure, circulatory shock, and hypoxemic respiratory failure requiring mechanical ventilatory support. Within four weeks of presentation, she passed away. Autopsy confirmed PVOD. In retrospect, our patient's presentation, imaging findings, and response to therapy are classic for PVOD.
DISCUSSION: In transplantation, PVOD is a rare but established complication of HSCT. In contrast, PVOD has not been demonstrated as a complication of solid organ transplantation. While this case invokes a potential link between solid organ transplantation and development of PVOD, the possibility of active AIH confounds this relationship. PVOD may represent part of the spectrum of vasculopathy seen in PAH, and PAH has been reported in association with AIH. Conversely AIH may reflect part of an overlap syndrome with an autoimmune disease already associated with PVOD.
CONCLUSIONS: Despite no previously reported association between PVOD and AIH, the potential for overlap of various autoimmune diseases with AIH makes recurrent AIH a more compelling culprit than OLT in the development of PVOD in this patient.
Reference #1: Montani D et al. Pulmonary veno-occlusive disease. Eur Respir J. 2009;33:189-200
Reference #2: Montani D et al. Pulmonary veno-occlusive disease: clinical, functional, radiologic, and hemodynamic characteristics and outcome of 24 cases confirmed by histology. Medicine. 2008;87:220-33
DISCLOSURE: The following authors have nothing to disclose: Jimmy Johannes, Rajan Saggar
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