SESSION TITLE: ILD Global Case Reports
SESSION TYPE: Global Case Report
PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM
INTRODUCTION: LAM is a rare disease affecting almost exclusively young women. It might involve multiple organs but the lungs are the most commonly affected. Histologically, it is characterised by the presence of typical LAM cells near the cystic lesions in the lungs and along small airways, blood vessels and lymphatics. Patients would have deterioration in lung function and end up in respiratory failure with lung transplantation being the only realistic treatment option. We report here a case of LAM who has undergone single lung transplant and has survived 16 years despite the complications of recurrence of LAM and hyperinflation of native lung.
CASE PRESENTATION: A female Chinese patient suffering from LAM with advanced respiratory failure underwent single ( left ) lung transplant in 1998 at age of 38. Her immunosuppressive therapy after transplant comprised of cyclosporin, azathioprine and prednisolone. Her peak FEV1 post transplant was 1.41L. Since early post transplant stage, there has been hyperinflation of native right lung shifting the mediastinum and compressing on the left lung allograft. She remained largely stable in condition but the lung function showed steady decline over the subsequent years with the decrease of FEV1 at a rate of about 54ml per year. Serial CXRs showed progressive worsening in hyperinflation of the right lung. CT thorax 7 years post-transplant started to unveil small cystic changes in the left lung suggestive of recurrence of LAM in the allograft. Her FEV1 deteriroated down to 0.92L in 2008 ( 10 years post transplant ) when she developed an episode of bleeding angiomyolipoma of right kidney requiring embolisation. As a result of that and with the chronic cyclosporin use, the renal function showed significant deterioration. Cyclosporin was switched to sirolimus as renal-sparing immunosuppression and as a pharmacological treatment of LAM affecting kidneys and lungs. Renal angiomyolipoma remained static in size with no further haemorrage. The deterioration in her lung condition was also stabilised. Serial CXRs and CT thorax showed no further worsening of the native lung hyperinflation but LAM disease in the allograft appeared progressive. The FEV1 decline over the past 6 years after sirolimus treatment was about 18ml per year, compared with 54 ml/year before. She is now 16 years post single lung transplant and remained stable in clinical condition. She is fully ambulatory on level ground and her exercise tolerance was 3 flight of stairs. Her latest FEV1 was 0.82L ( 33% predicted ).
DISCUSSION: Although lung transplant is a well accepted treatment option for patients with LAM, it has been reported that the disease process could recur in the lung allograft. With better understanding of the genetic and molecular pathogenesis, pharmacological treatment is now available. Given its inhibitory effects of mTOR-mediated proliferation of LAM cells, sirolimus has been tried and shown to have the potential of improving lung functions of patients with LAM. Although lung transplant saved the life of our patient with end-stage LAM, the disease process was ongoing after transplant as evidenced by the progressive hyperinflation of the native lung, radiological recurrence of LAM in the lung allograft and the angiomyolipoma in the kidney with bleeding complication. However, the lung condition seemed to be stabilised after the episode of bleeding renal angiomyolipoma and the subsequent treatment with sirolimus. Although the change in compliance of the left lung allograft affected by LAM might account for the less compression by the native right lung, the stabilisation is more likely to be attributed to the therapeutic effect of sirolimus both as immunosuppressant and pharmacological treatment for LAM .
CONCLUSIONS: We have presented a case of LAM with single lung transplant complicated with native lung hyperinflation and recurrence in the allograft, and condition stabilised with the use of mTOR inhibitors. We suggest the early consideration of mTOR inhibitors for post-lung transplant patients with underlying diagnosis of LAM especially when there is evidence of disease activity.
Reference #1: McCormack et al. Efficacy and safety of sirolimus in lymphangioleiomyomatosis. N Engl J Med. 2011; 364: 1595-606.
Reference #2: Benden et al. Lung transplantation for lymphangioleiomyomatosis: the European experience. J Heart Lung Transplant. 2009; 28:1-7.
DISCLOSURE: The following authors have nothing to disclose: Chi Fong Wong, See Wan Yan
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