Pulmonary hemodynamics and transplant-free survival in sarcoidosis-associated pulmonary hypertension: Results from an international registry.

Pulmonary hypertension (PH) is a risk factor for mortality in patients with sarcoidosis. Severe PH in chronic lung disease has previously been defined as mean pulmonary arterial pressure (mPAP) ≥ 35 mmHg or mPAP 25 ≥ mmHg with cardiac index (CI) ≤ 2 L/min/m. However, there is no clear definition denoting severity of sarcoidosis-associated PH (SAPH).

Predictors of lung transplant waitlist mortality for sarcoidosis.

Rationale: Unlike in other chronic lung diseases, criteria for lung transplant referral in sarcoidosis is not well-established. Waitlist mortality may offer clues in identifying clinical factors that warrant early referral. We aim to identify predictors for transplant waitlist mortality to improve referral criteria for patients with sarcoidosis.

The six-minute walk test in sarcoidosis associated pulmonary hypertension: Results from an international registry.

Introduction: Sarcoidosis associated pulmonary hypertension (SAPH) is a leading contributor to sarcoidosis-related mortality. The 6-min walk test (6MWT) is widely used in assessment of cardiorespiratory conditions. A reduced 6-min walk distance (6MWD) has been associated with increased mortality in SAPH.

Effect of antifibrotic therapy in patients with idiopathic pulmonary fibrosis undergoing lung transplant in the peri and post-operative period.

Background: Idiopathic pulmonary fibrosis (IPF) is a rapidly progressive interstitial lung disease with high mortality. Lung transplant remains a cornerstone of treatment despite the advent of antifibrotic therapy. In light of the increasing number of patients on antifibrotic therapy prior to lung transplantation it is paramount to demonstrate these medications do not augment postoperative complications following transplant.

Clinical predictors and explant lung pathology of acute exacerbation of idiopathic pulmonary fibrosis.

Background: Idiopathic pulmonary fibrosis (IPF) is characterised by constant threat of acute exacerbation of IPF (AE-IPF). It would be significant to identify risk factors of AE-IPF. We sought to determine the prognostic value of lung transplantation candidacy testing for AE-IPF and describe explant pathology of recipients with and without AE-IPF before lung transplantation.

Pulmonary vasculopathy in explanted lungs from patients with interstitial lung disease undergoing lung transplantation.

Background: Pulmonary hypertension (PH) causes increased morbidity and mortality in patients with interstitial lung diseases (ILD). Classification schemes, while well-characterised for the vasculopathy of idiopathic PH, have been applied, unchallenged, to ILD-related PH. We evaluated pulmonary arterial histopathology in explanted human lung tissue from patients who were transplanted for advanced fibrotic ILD.

COVID-19 in lung transplant recipients.

Solid organ transplant recipients are considered at high risk for COVID-19 infection due to chronic immune suppression; little data currently exists on the manifestations and outcomes of COVID-19 infection in lung transplant recipients. Here we report 8 cases of COVID-19 identified in patients with a history of lung transplant. We describe the clinical course of disease as well as preexisting characteristics of these patients.

Physiological predictors of survival in patients with sarcoidosis-associated pulmonary hypertension: results from an international registry.

Introduction: Sarcoidosis-associated pulmonary hypertension (SAPH) is associated with reduced survival in single-centre studies. The international Registry for SAPH (ReSAPH) with long-term follow-up was established to enrich our knowledge of this complication of sarcoidosis. This analysis aims to elucidate factors associated with reduced transplant-free survival in SAPH patients.

Effect of Acute Exacerbation of Idiopathic Pulmonary Fibrosis on Lung Transplantation Outcome.

Background: Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) has an expected median survival of 3 months. Lung transplantation is a potentially lifesaving therapy for AE-IPF. However, the current knowledge of transplantation outcomes during AE-IPF is limited to a few small retrospective studies, reporting only 1-year post-transplantation survival.

Clinical features of sarcoidosis associated pulmonary hypertension: Results of a multi-national registry.

Background: Pulmonary hypertension (PH) is a significant cause of morbidity and mortality in sarcoidosis. We established a multi-national registry of sarcoidosis associated PH (SAPH) patients.

Methods: Sarcoidosis patients with PH confirmed by right heart catheterization (RHC) were studied.

A Telemedicine-Based Intervention Reduces the Frequency and Severity of COPD Exacerbation Symptoms: A Randomized, Controlled Trial.

Background: Patients with chronic obstructive pulmonary disease (COPD) may not recognize worsening symptoms that require intensification of therapy. They may also be reluctant to contact a healthcare provider for minor worsening of symptoms. A telemedicine application for daily symptom reporting may reduce these barriers and improve patient outcomes.

Bosentan for sarcoidosis-associated pulmonary hypertension: a double-blind placebo controlled randomized trial.

Background: Sarcoidosis-associated pulmonary hypertension (SAPH) is a common problem in patients with persistent dyspneic sarcoidosis. The objective of this study was to determine the effect of bosentan therapy on pulmonary arterial hemodynamics in patients with SAPH.

Methods: This 16-week study was a double-blind, placebo-controlled trial of either bosentan or placebo in patients with SAPH confirmed by right-sided heart catheterization.

Sarcoidosis-associated pulmonary hypertension.

Purpose Of Review: Pulmonary hypertension is a serious complication of sarcoidosis. This review discusses clinical characteristics of patients with sarcoid-associated pulmonary hypertension (SAPH) and pitfalls in the diagnosis, and highlights potential therapies.

Recent Findings: SAPH is common in patients with advanced disease, but it can occur in patients with minimal disease burden.

Medical pneumoplasty, surgical resection, or lung transplant.

Over the last decade, advances in bronchoscopic and surgical techniques have expanded our treatment armamentarium for patients with severe emphysema who previously would have received a pessimistic outlook from their physician. Advances in our understanding of the different COPD phenotypes and its natural history has refined our selection process as to which group of emphysema patients will derive maximum benefit from LVR, bullectomy, or lung transplantation. Because emphysema is a progressive disease, initial treatment with bronchoscopic or surgical LVR or bullectomy does not preclude lung transplantation in the future.

Full face mask for noninvasive positive-pressure ventilation in patients with acute respiratory failure.

Background: Noninvasive positive-pressure ventilation (NPPV) is commonly used to improve ventilation and oxygenation in patients with acute respiratory failure (ARF). Mask leak and intolerance due to facial discomfort or claustrophobia often occur with NPPV and are frequently cited reasons for treatment failure.

Methods: Retrospective review of patient records from a tertiary-care referral hospital.

Treatment of Guillain-Barré syndrome induced by cyclosporine in a lung transplant patient.

We report the case of a 58-year-old man with severe chronic obstructive pulmonary disease who developed acute quadriparesis during the post-operative period following bilateral lung transplantation after receiving cyclosporine for immunosuppression. Electromyography with nerve conduction study and cerebrospinal fluid analysis supported a diagnosis of Guillain-Barré Syndrome, which improved upon the discontinuation of cyclosporine, replacement with tacrolimus, and initiation of plasmapheresis. We propose the discontinuation of cyclosporine and initiation of plasmapheresis as a treatment for cyclosporine-associated Guillain-Barré syndrome.

Lung volume reduction surgery as a bridge to lung transplantation.

Lung volume reduction surgery (LVRS) improves lung function, exercise capacity, and quality of life in patients with advanced emphysema. In some patients with emphysema who are candidates for lung transplantation, LVRS is an alternative treatment option to lung transplantation, or may be used as a bridge to lung transplantation. Generally accepted criteria for LVRS include severe non-reversible airflow obstruction due to emphysema associated with significant evidence of lung hyperinflation and air trapping.