A 21-year-old woman comes to the office for evaluation of progressively increasing dyspnea and nonproductive cough for the past 2 weeks. Four months ago, the patient underwent bilateral lung transplantation for bronchiectasis due to underlying cystic fibrosis. Other medical conditions include pancreatic insufficiency and osteoporosis. Current medications are pancreatic enzymes, multivitamins, tacrolimus, mycophenolate mofetil, and prednisone. Temperature is 38 C (100.4 F), blood pressure is 110/78 mm Hg, pulse is 96/min, and respirations are 18/min. Pulse oximetry shows 98% with the patient using 2 liters/min of supplemental oxygen. Examination shows bilateral lung crackles. Chest x-ray reveals perihilar opacities, interstitial edema, and small bilateral pleural effusions. What is the most appropriate next step in management?

Question

A 21-year-old woman comes to the office for evaluation of progressively increasing dyspnea and nonproductive cough for the past 2 weeks.  Four months ago, the patient underwent bilateral lung transplantation for bronchiectasis due to underlying cystic fibrosis.  Other medical conditions include pancreatic insufficiency and osteoporosis.  Current medications are pancreatic enzymes, multivitamins, tacrolimus, mycophenolate mofetil, and prednisone.  Temperature is 38 C (100.4 F), blood pressure is 110/78 mm Hg, pulse is 96/min, and respirations are 18/min.  Pulse oximetry shows 98% with the patient using 2 liters/min of supplemental oxygen.  Examination shows bilateral lung crackles.  Chest x-ray reveals perihilar opacities, interstitial edema, and small bilateral pleural effusions.  What is the most appropriate next step in management?

Quizplus · Accepted Answer

11ec25c8_d949_ec1d_bc81_45245acc4e3c_MD0003_00 Maintaining an appropriate balance of chronic daily immunosuppression (eg, tacrolimus, mycophenolate, prednisone) in organ transplant recipients can be a challenge. Too little immunosuppression puts the patient at risk for transplant rejection, whereas too much immunosuppression creates susceptibility to infection. This balance can be especially challenging in lung transplant recipients because the transplanted organ is in direct contact with the outside environment, increasing the susceptibility to infection. This recent lung transplant recipient's presentation with dyspnea, nonproductive cough, low-grade fever, hypoxemia, and chest x-ray showing perihilar opacities with interstitial edema and small pleural effusions is consistent with acute lung transplant rejection (ALTR), which is a cell-mediated response that most commonly occurs within 6 months of transplant. ALTR is usually effectively treated with high-dose glucocorticoids (eg, pulse dose prednisone); however, such treatment could markedly worsen an acute infection and should not be initiated until infection has been ruled out (Choice D). Lung transplant recipients are at risk for both opportunistic (eg, cytomegalovirus, invasive aspergillosis, pneumocystis pneumonia) and nonopportunistic (eg, influenza, adenovirus) infections that can have similar clinical presentation to ALTR. Therefore, the diagnostic workup of ALTR should include bronchoalveolar lavage and lung biopsy. Results help both evaluate for infection and confirm acute rejection with consistent biopsy findings (eg, submucosal lymphocytic infiltrate, perivascular inflammation). (Choice B) CT angiogram of the chest is used to evaluate for acute pulmonary embolism, which can cause shortness of breath and effusions but is unlikely to explain progressive symptoms over 2 weeks and interstitial edema on chest x-ray. (Choice C) Furosemide and daily weight measurement is appropriate management for an acute heart failure exacerbation. An acute heart failure exacerbation can cause similar chest x-ray findings to ALTR, but it does not typically cause low-grade fever and is less likely in this young patient with no history of heart failure. (Choice E) Calcineurin inhibitors (eg, tacrolimus) are typically continued during diagnostic evaluation of ALTR as stopping them may worsen acute rejection. Even if infection is diagnosed, calcineurin inhibitors are often continued to lower the risk of concomitant acute rejection developing. Educational objective: Acute lung transplant rejection (ALTR) typically occurs within 6 months of transplant and may present with progressive dyspnea and cough accompanied by low-grade fever, hypoxemia, and chest x-ray revealing perihilar opacities and interstitial edema. Because pulmonary infection can have a similar clinical presentation and the treatment for ALTR (ie, high-dose glucocorticoids) could markedly worsen an infection, bronchoalveolar lavage and lung biopsy should be performed in the diagnostic workup of ALTR. References: Infections after lung transplantation. (http://www.ncbi.nlm.nih.gov/pubmed/30069386)

A 21-Year-Old Woman Comes to the Office for Evaluation of Progressively