Charles Lane

Disease Recurrence and Acute Cellular Rejection Episodes During the First Year After Lung Transplantation Among Patients With Sarcoidosis.

Introduction Sarcoidosis is reported to recur after lung transplantation (LT). We sought to determine the frequency of recurrent disease after LT and predictors of recurrence. We also evaluated the incidence and severity of acute cellular rejection (ACR) episodes among these patients. Methods The database of LT patients at Cleveland Clinic was interrogated for sarcoidosis patients who underwent LT between May 1993 and 2011. Charts were reviewed for demographics, type of transplant, posttransplant biopsy findings, and outcomes. Results Data were available for 30 patients (mean age, 50 ± 9.3 years; range, 30-65 years; M-to-F ratio, 17:13; single-to-double-to-heart lung ratio, 5:24:1). Recurrence of sarcoidosis was noted among 7 patients (pathological recurrence in all and radiological findings suggesting recurrence in 1 patient) with no impact on overall outcomes. Presence of granulomas on explanted lungs was the only predictor of recurrence (85.7% vs 30.4%, odds ratio, 13.7; 1.4-136.2; P = 0.02). Overall burden of ACR episodes on all bronchoscopies was significantly lower in patients with disease recurrence (7.6 % vs 21.3% of biopsies, P = 0.038). Among patients with recurrent disease, ACR did not develop once disease recurrence had been seen on transbronchial biopsy. Conclusions A significant proportion of sarcoidosis patients have disease recurrence after LT and presence of active granulomas on explant is associated with subsequent recurrence. There may be an association of recurrence with lower frequency of ACR episodes. There does not appear to be any impact of sarcoidosis recurrence on 1-, 3-, or 5-year survivals.

Characteristics and Outcomes of Patients With Lung Transplantation Requiring Admission to the Medical ICU

BACKGROUND There are few data on characteristics and outcomes among patients with lung transplantation (LT) requiring admission to the medical ICU (MICU) beyond the perioperative period. METHODS We interrogated the registry database of all admissions to the MICU at Cleveland Clinic (a 53-bed closed unit) to identify patients with history of LT done > 30 days ago (n = 101; mean age, 55.4 ± 12.6 years; 53 men, 48 women). We collected data regarding demographics, history of bronchiolitis obliterans syndrome, preadmission FEV1, clinical and laboratory variables at admission, MICU course, length of stay, hospital survival, and 6-month survival. RESULTS The most common indication for MICU admission was acute respiratory failure (n = 51, 50.5%). Infections were most frequently responsible for respiratory failure, whereas acute rejection (cellular or humoral) was less likely (16%). Nearly one-fourth of the patients required hemodialysis (24.1%), and more than one-half required invasive mechanical ventilation (53.5%). Despite excellent hospital survival (88 of 101), 6-month survival was modest (56.4%). APACHE (Acute Physiology and Chronic Health Evaluation) III score at admission and single LT were independent predictors of hospital survival but did not predict outcome at 6 months. Functional status at discharge was the only independent predictor of 6-month survival (adjusted OR, 5.1; 95% CI, 1.1-22.7; P = .035). CONCLUSIONS Acute rejection is an infrequent cause of decompensation among patients with LT requiring MICU admission. For patients admitted to the MICU, 6-month survival is modest. Functional status at the time of discharge is an independent predictor of survival at 6 months.

Acute ischemic optic neuropathy with extended prone position ventilation in a lung transplant recipient.

Prone position ventilation (PPV) improves mortality in severe acute respiratory distress syndrome (ARDS), but outcomes following its use in lung transplant recipients are not known. We report the case of a 42-year-old Caucasian man who presented with severe ARDS from Bordetella pertussis, 5 years after bilateral sequential lung transplant for cystic fibrosis. He was managed with PPV for 22 days and had a prolonged ICU stay complicated by hypoxic ischemic optic neuropathy leading to blindness. Since his discharge from the ICU 6 months ago, his FEV1has recovered to 47% predicted compared to his pre-ICU peak FEV1of 85% predicted, suggesting recovery of lung function. This is the first report of optic nerve damage and vision loss in patients undergoing PPV. Our report also suggests that, in appropriately selected lung transplant recipients, severe hypoxemia could potentially be managed with prone ventilation.

Post-transplant lymphoproliferative disorder of the bladder in a lung transplant recipient

Abstract Post-transplant lymphoproliferative disorder (PTLD) occurs in ~5% of solid organ and hematopoietic stem cell transplant recipients. We report a unique presentation of PTLD in the bladder of a lung transplant recipient. Our patient was a 62-year-old female who received a bilateral lung transplant for chronic obstructive pulmonary disease. She presented with fever, left-sided flank pain and foul-smelling urine consistent with urosepsis. An abdominal and pelvic computerized tomography revealed an irregular and nodular bladder wall thickening suspicious for urothelial neoplasm. Cystoscopy revealed multiple bladder masses and biopsy demonstrated non-Hodgkin lymphoma consistent with PTLD. She was treated with a reduction in immunosuppression followed by chemotherapy and achieved remission. PTLD in the lung transplant recipients has been described in the gut, respiratory tract, skin, liver and kidney but not in the bladder. This case highlights the need for maintaining a high clinical vigilance even when transplant recipients present with seemingly benign clinical complaints.

Everolimus induced pneumonitis in a lung transplant recipient

Abstract Everolimus is a mechanistic target of rapamycin inhibitor used for the treatment of various cancers and prevention of allograft rejection in solid organ transplantation. We present a case of a lung transplant recipient on everolimus who was admitted with generalized weakness, hypoxia and new onset bilateral pulmonary infiltrates on imaging. Extensive workup revealed no infectious etiology and high levels of serum everolimus levels. Her condition deteriorated over the hospital course with symptoms and signs of systemic everolimus toxicity. She was treated with high-dose steroids with significant improvement. Follow-up imaging showed resolution of infiltrates. Everolimus induced pneumonitis is seldom reported in the lung transplant literature. It is important to recognize early signs of toxicity to intervene and preserve the lung allograft.

Non-invasive positive pressure ventilation in lung transplant recipients with acute respiratory failure: Beyond the perioperative period

Purpose: The purpose of this study is to evaluate outcomes in MICU lung transplant recipients with acute respiratory failure treated with non‐invasive positive pressure ventilation (NPPV) and identify factors associated with NPPV failure (need for intubation). Methods: Retrospective chart review of all lung transplant recipients who were admitted with acute respiratory failure to the MICU from January 2009–August 2016 was completed. Logistic regression analysis was performed to determine which factors were independently associated with NPPV failure. Results: Of 156 patients included in the study, 125 (80.1%) were tried on NPPV. Sixty‐eight (54.4%) were managed successfully with NPPV with a hospital survival rate of 94.1%. Subjects who failed NPPV had higher hospital mortality, similar to those intubated from the outset (15 [48.3%]; 22 [38.6%], p = .37). In multivariate analyses, APACHE III scores >78 (9.717 [3.346, 28.22]) and PaO2/FiO2 ≤ 151 (4.54 [1.72, 11.99]) were associated with greater likelihood of NPPV failure. There was no difference in NPPV failure based on the presence of BOS. In patients with high severity of illness, there was no difference in mortality between initial IMV and NPPV failure when stratified on the basis of hypoxemia (PaO2/FiO2 > 151, p‐value 0.34; PaO2/FiO2 ≤ 151, p‐value 0.99). Conclusions: NPPV is a viable option for lung transplant recipients with acute respiratory failure. Extreme caution should be exercised when used in patients with high severity of illness (APACHE III >78) and/or severe hypoxemia (PaO2/FiO2 ≤ 151). HighlightsLung transplant recipients with acute respiratory failure are at high risk of death.NPPV is a viable option for lung transplant recipients with acute respiratory failure.NPPV should be used sparingly in lung transplant recipients on vasopressors, with high severity of illness (APACHE III score > 78) and/or severe hypoxemia (PaO2/FiO2 ≤ 151).

Pulmonary Vein Stenosis Following Single-Lung Transplantation Successfully Treated with Intravascular Ultrasound-Guided Angioplasty and Stent Placement

Patient: Female, 60 Final Diagnosis: Pulmonary vein stenosis following single lung transplant Symptoms: Dyspnea on exertion and dry cough Medication: — Clinical Procedure: Balloon angioplasty and stenting of the left common pulmonary vein Specialty: Cardiology Objective: Unusual clinical course Background: Pulmonary vein stenosis (PVS) is a rare event following lung transplantation, but is a serious complication that requires prompt diagnosis and treatment. Case Report: We describe the case of a 60-year-old woman who underwent single-lung transplantation for idiopathic pulmonary fibrosis (IPF). One year following her single-lung transplant, she was admitted to the hospital for hypoxemic respiratory failure. The results of quantitative lung perfusion scintigraphy (LPS) raised the possibility of a diagnosis of PVS. Computed tomography angiography (CT angiography) of the chest identified more than 50% stenosis of the left common pulmonary vein at the anastomotic site with the left atrium. She was successfully treated with angioplasty and stent placement guided by intravascular ultrasonography. Post dilatation of the PVS, the pulmonary venous pressure gradient decreased from 12–16 mm Hg to 3–4 mm Hg. At three-month follow-up, the patient reported resolution of her shortness of breath. In support of this case report, we review the management of four previously reported cases from the literature of lung transplantation associated with PVS. Conclusions: PVS should be considered in the differential diagnosis of lung transplant patients who present with worsening dyspnea. Quantitative LPS and CT angiography are important in the diagnosis of PVS. Successful management of PVS, with salvage of the transplanted lung and the prevention of further surgical interventions, may be achieved with intravascular ultrasound-guided angioplasty and stent placement.