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We present a case of cardiac sarcoidosis with persistent, focal fluorodeoxyglucose uptake at the left ventricular apical aneurysm concerning for ongoing active inflammatory injury, prompting aggressive immunosuppressive therapy. This case highlights the importance of understanding the various clinical entities that may resemble disease activity on fluorodeoxyglucose positron emission tomography/computed tomography imaging. (Level of Difficulty: Intermediate.).

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Tens of thousands of patients with advanced lung diseases may be eligible to be considered as potential candidates for lung transplant around the world each year. The timing of referral, evaluation, determination of candidacy, and listing of candidates continues to pose challenges and even ethical dilemmas. To address these challenges, the International Society for Heart and Lung Transplantation appointed an international group of members to review the literature, to consider recent advances in the management of advanced lung diseases, and to update prior consensus documents on the selection of lung transplant candidates. The purpose of this updated consensus document is to assist providers throughout the world who are caring for patients with pulmonary disease to identify potential candidates for lung transplant, to optimize the timing of the referral of these patients to lung transplant centers, and to provide transplant centers with a framework for evaluating and selecting candidates. In addition to addressing general considerations and providing disease specific recommendations for referral and listing, this updated consensus document includes an ethical framework, a recognition of the variability in acceptance of risk between transplant centers, and establishes a system to account for how a combination of risk factors may be taken into consideration in candidate selection for lung transplantation.

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Before coronavirus disease 2019 (COVID-19), telehealth evaluation and management (E/M) services were not widely used in the United States and often were restricted to rural areas or locations with poor access to care. Most Medicare beneficiaries could not receive telehealth services in their homes. In response to the COVID-19 pandemic, Medicare, Medicaid, and commercial insurers relaxed restrictions on both coverage and reimbursement of telehealth services. These changes, together with the need for social distancing, transformed the delivery of outpatient E/M services through an increase in telehealth use. In some cases, the transition from in-person outpatient care to telehealth occurred overnight. Billing and claim submission for telehealth services is complicated; has changed over the course of the pandemic; and varies with each insurance carrier, making telehealth adoption burdensome. Despite these challenges, telehealth is beneficial for health-care providers and patients. Without additional legislation at the federal and state levels, it is likely that telehealth use will continue to decline after the COVID-19 public health emergency.

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INRODUCTION: The mechanistic target of rapamycin inhibitors (mTORi) sirolimus and everolimus stabilize lung function in patients with pulmonary lymphangioleiomyomatosis (LAM) but do not induce remission. Pre-clinical studies suggest that simvastatin in combination with sirolimus induces LAM cell death. The objective of this study was to assess the safety of simvastatin with either sirolimus or everolimus in LAM patients. METHODS: This was a phase II single arm trial evaluating the safety of escalating daily simvastatin (20-40 mg) in LAM patients already treated with sirolimus or everolimus. Adverse events and changes in lipid panel profile, pulmonary function tests, and VEGF-D were assessed. RESULTS: Ten LAM patients on a stable dose of mTORi for >3 months were treated with 20 mg simvastatin for two months followed by 40 mg for two months. The most common adverse events were peripheral edema (30%), cough (30%), and diarrhea (30%). No patients withdrew or had a reduction in simvastatin dose because of adverse events. Two patients required sirolumus dose reduction for supratherapeutic trough levels following simvastatin initiation. Total cholesterol and low density lipoproteins declined over the study period (-46.0 mg/dL±20.8, p = 0.008; -41.9 mg/dL±22.0, p = 0.01, respectively). There was also a decline in FEV1 (-82.0 mL±86.4, p = 0.02) but no significant change in FVC, DLCO, or VEGF-D. CONCLUSIONS: The combination of simvastatin with mTORi in LAM patients is safe and well-tolerated from an adverse events perspective. The addition of simvastatin, however, was associated with decline in FEV1 and the efficacy of this combination should be explored in larger trials.

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CASE PRESENTATION: A 31-year-old white man presented for evaluation of productive cough and dyspnea on exertion of 2 months' duration. Associated symptoms included wheezing, chest tightness, and postnasal drip. He was recently treated for pneumonia with a 7-day course of levofloxacin because of an abnormal chest radiograph demonstrating bilateral infiltrates, but his symptoms failed to improve. He had a medical history of mild intermittent asthma and was on no active treatment. He was a nonsmoker. He was born in the United States. He denied any recent travel within or outside the United States, sick contacts, or illicit drug use. He did not have any pets and denied exposure to mold, hot tubs, a down comforter, or pillows. He worked in a vocational program and his hobbies included refinishing wood floors.

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BACKGROUND: Recommendations regarding key aspects related to the diagnosis and pharmacological treatment of lymphangioleiomyomatosis (LAM) were recently published. We now provide additional recommendations regarding four specific questions related to the diagnosis of LAM and management of pneumothoraces in patients with LAM. METHODS: Systematic reviews were performed and then discussed by a multidisciplinary panel. For each intervention, the panel considered its confidence in the estimated effects, the balance of desirable (i.e., benefits) and undesirable (i.e., harms and burdens) consequences, patient values and preferences, cost, and feasibility. Evidence-based recommendations were then formulated, written, and graded using the GRADE (Grading of Recommendations, Assessment, Development, and Evaluation) approach. RESULTS: For women who have cystic changes on high-resolution computed tomography of the chest characteristic of LAM, but who have no additional confirmatory features of LAM (i.e., clinical, radiologic, or serologic), the guideline panel made conditional recommendations against making a clinical diagnosis of LAM on the basis of the high-resolution computed tomography findings alone and for considering transbronchial lung biopsy as a diagnostic tool. The guideline panel also made conditional recommendations for offering pleurodesis after an initial pneumothorax rather than postponing the procedure until the first recurrence and against pleurodesis being used as a reason to exclude patients from lung transplantation. CONCLUSIONS: Evidence-based recommendations for the diagnosis and treatment of patients with LAM are provided. Frequent reassessment and updating will be needed.

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Despite significant advances in surgical techniques, perioperative care, and immunosuppressive therapy, solid organ transplantation still carries considerable risk of complications. Pulmonary complications, in particular, are a major cause of morbidity and mortality. Although infectious complications prevail, the lungs are also vulnerable to a variety of noninfectious complications related to the transplant surgery and adverse effects of the immunosuppressive regimen. This article focuses on noninfectious pulmonary complications associated with the 3 most commonly performed solid organ transplant procedures: liver, kidney, and heart.

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Venovenous extracorporeal membrane oxygenation (ECMO) has become a viable and increasingly utilized option for the treatment of refractory hypoxemia in severe acute respiratory distress syndrome (ARDS). However, options are limited for ARDS patients who fail to wean from ECMO. The high rates of infection, presence of extrapulmonary end organ damage, intensive care unit-acquired weakness, and high short-term mortality associated with ARDS are all significant hurdles that make lung transplantation a difficult prospect to consider. However, ECMO support has been used as a bridge to transplant in patients with other underlying chronic lung diseases. Our case illustrates the successful use of lung transplantation for a patient with no previous lung disease who developed refractory ARDS requiring protracted ECMO support. The use of ambulatory ECMO with early institution of physical therapy is an essential component in preparing such patients for successful transplantation.

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BACKGROUND: Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease that primarily affects women. The purpose of these guidelines is to provide recommendations for the diagnosis and treatment of LAM. METHODS: Systematic reviews were performed to summarize evidence pertinent to our questions. The evidence was summarized and discussed by a multidisciplinary panel. Evidence-based recommendations were then formulated, written, and graded using the Grading of Recommendations, Assessment, Development, and Evaluation approach. RESULTS: After considering the panel's confidence in the estimated effects, the balance of desirable (i.e., benefits) and undesirable (i.e., harms and burdens) consequences of treatment, patient values and preferences, cost, and feasibility, recommendations were formulated for or against specific interventions. These included recommendations for sirolimus treatment and vascular endothelial growth factor D testing and recommendations against doxycycline and hormonal therapy. CONCLUSIONS: Evidence-based recommendations for the diagnosis and treatment of patients with LAM are provided. Frequent reassessment and updating will be needed.

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Birt-Hogg-Dubé syndrome (BHD) is a rare autosomal dominant disorder caused by mutations in the Folliculin gene and is characterized by the formation of fibrofolliculomas, early onset renal cancers, pulmonary cysts, and spontaneous pneumothoraces. The exact pathogenesis of tumor and lung cyst formation in BHD remains unclear. There is great phenotypic variability in the clinical features of BHD, and patients can present with any combination of skin, pulmonary, or renal findings. More than 80% of adult patients with BHD have pulmonary cysts on high-resolution computed tomography scan of the chest.

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Lung transplantation is now an established treatment for a broad spectrum of end-stage pulmonary diseases. According to the International Society for Heart and Lung Transplantation Registry, more than 50,000 lung transplants have been performed worldwide, with nearly 11,000 lung transplant recipients alive in the United States. With the increasing application of lung transplantation, pulmonologists must be cognizant of common complications unique to the postlung transplant period and the associated radiologic findings. The aim of this review is to describe clinical manifestations and prototypical radiographic features of both common and rare complications encountered in lung transplant recipients.

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This document was developed through the collaborative efforts of the Society of Critical Care Medicine, the American College of Chest Physicians, and the Association of Organ Procurement Organizations. Under the auspices of these societies, a multidisciplinary, multi-institutional task force was convened, incorporating expertise in critical care medicine, organ donor management, and transplantation. Members of the task force were divided into 13 subcommittees, each focused on one of the following general or organ-specific areas: death determination using neurologic criteria, donation after circulatory death determination, authorization process, general contraindications to donation, hemodynamic management, endocrine dysfunction and hormone replacement therapy, pediatric donor management, cardiac donation, lung donation, liver donation, kidney donation, small bowel donation, and pancreas donation. Subcommittees were charged with generating a series of management-related questions related to their topic. For each question, subcommittees provided a summary of relevant literature and specific recommendations. The specific recommendations were approved by all members of the task force and then assembled into a complete document. Because the available literature was overwhelmingly comprised of observational studies and case series, representing low-quality evidence, a decision was made that the document would assume the form of a consensus statement rather than a formally graded guideline. The goal of this document is to provide critical care practitioners with essential information and practical recommendations related to management of the potential organ donor, based on the available literature and expert consensus.

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