Case report
Acute Constrictive Pericarditis After Lung Transplantation for Lymphangioleiomyomatosis

https://doi.org/10.1016/j.healun.2008.10.009Get rights and content

Lymphangioleiomyomatosis (LAM) is a rare, cystic, progressive lung disease with many extrapulmonary manifestations, which may complicate allograft function after transplantation. We present a LAM patient with new dyspnea and declining spirometry without rejection, infection or recurrence one year after bilateral lung transplantation. Investigation revealed acute constrictive pericarditis, which has not been reported previously in a lung transplant patient with LAM. This represents a novel complication likely due to progression of extrapulmonary LAM and should be considered in LAM transplant patients with dyspnea.

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Case Report

A 32-year-old woman with LAM, who underwent bilateral lung transplantation 1 year earlier, presented with declining spirometry, worsening dyspnea and new-onset orthopnea. She was diagnosed with acute constrictive pericarditis and her symptoms responded to pericardiectomy.

After experiencing progressive dyspnea for 5 years, the patient was diagnosed with LAM on the basis of characteristic diffuse cystic lesions throughout her lungs on computed tomography (CT) and retroperitoneal lymphadenopathy.

Discussion

The concern for rejection in lung transplant recipients with unexplained progressive dyspnea and spirometry decline is appropriate and is quite high. In our patient, because this occurred in the setting of a decreased immunosuppressive regimen 1 year after lung transplant and there were no other obvious explanations for the respiratory decline, the decrease in FEV1 raised concern for BOS. Furthermore, with the discontinuation of mycophenolate mofetil, she was at high risk for acute cellular

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