INTRODUCTION:Lymphangioleiomyomatosis (LAM) is a rare and progressive lung disease complicated by the accumulation of chyle in the chest and abdomen. Currently, only lung transplantation is a viable therapeutic option for advanced stages of LAM. However, complications of persistent and progressive extra-pulmonary disease and recurrence of LAM in the transplanted lung may complicate transplantation. As unexplained decline in respiratory status raises the concern for rejection post lung transplantation, awareness of complications associated with underlying LAM is essential in treating episodes of dyspnea in lung transplant recipients with LAM.
CASE PRESENTATION:A 32 year old woman with LAM, one year status post bilateral lung transplantation, Cytomegalovirus (CMV) status donor positive-recipient negative, presented with shortness of breath while undergoing treatment with foscarnet for ganciclovir resistant CMV retinitis and viremia. To optimize response, her corticosteroids had been decreased to prednisone 5mg daily and mycophenolate and tacrolimus levels lowered. Ten months post transplantation, she presented with dyspnea and cough with a new pulmonary infiltrate and decline in pulmonary functional status. Lung rejection, infection, CMV pneumonitis, and recurrence of LAM were ruled out by multiple lung specimens retrieved by bronchoscopy as well as surgical lung biopsy. During the interim, she had also developed chylothorax that required percutaneous drainage and diet changes with subsequent improvement. High resolution computed tomography images of the chest had not demonstrated worsening pleural effusion, nor pericardial effusion or recurrence of LAM in the lungs. Right and left heart function and pericardium assessed by an echocardiogram were normal during this episode. Twelve months post lung transplantation, she manifested another episode of dyspnea which progressed rapidly over a few days. Since she had a recent decrease in immunosuppression and known CMV disease, the differential diagnosis included an acute lung rejection and CMV pneumonitis. A history of orthopnea, paroxysmal nocturnal dyspnea and jugular venous distension by examination prompted further diagnostic and therapeutic interventions despite having had a normal echocardiogram 10 days prior. A repeat echocardiogram demonstrated a new finding of constrictive pericarditis. A right and left heart catheterization confirmed compromised hemodynamic measurements. She was subjected to an open pericardiectomy that resulted in rapid improvement in hemodynamic measurements and her dyspnea. Special stains and cultures of the pericardial biopsy were all negative for infection including CMV by cultures and by polymerase chain reaction (PCR). Her CMV retinitis responded to maintenance foscarnet and lowered immunosuppressive regimen. She improved without requiring augmented antirejection medications.
DISCUSSIONS:While chylous pericardial effusion is a known complication of progressive LAM, acute constrictive pericarditis in lung transplant recipients with LAM has not been previously reported. Since our patient was at high risk for complications associated with CMV, constrictive pericarditis due to CMV was a possible explanation. As she had been responding to foscarnet and lowered immunosuppressive regimen, other possible etiologies had to be entertained. Since she had a normal echocardiogram done within 10 days of her presentation as well as no evidence for recurrence of LAM in the transplanted lungs, nor had substantial reaccumulation of chylothorax, the possibility of an acute rejection was a concern in this patient on lowered immunosuppressive regimen. However, the elicited history prompted the consideration of cardiac etiology for this episode of dyspnea.
CONCLUSION:This case illustrates that acute constrictive chylous pericarditis secondary to underlying LAM must be included in the midst of differential diagnosis of infection and rejection in the lung transplant recipient with LAM. Extra-pulmonary complications of LAM may continue to progress despite ‘successful’ lung transplantation for LAM. Awareness of this possibility will prompt aggressive and appropriate diagnostic and therapeutic interventions.
DISCLOSURE:Martha Billings, No Financial Disclosure Information; No Product/Research Disclosure Information