Impact of tacrolimus vs cyclosporine on chronic lung allograft dysfunction incidence and allograft survival in the International Society of Heart and Lung Transplantation registry.

Background: The ScanCLAD study reported a lower incidence of chronic lung allograft dysfunction (CLAD) with the use of once-daily tacrolimus vs twice-daily cyclosporine. Using the International Society for Heart and Lung Transplantation (ISHLT) Thoracic Organ Transplant (TTX) Registry data, we evaluated the hypothesis that tacrolimus is superior to cyclosporine in real-world clinical practice.

Methods: This study is a retrospective cohort study of adult lung transplant recipients in the ISHLT registry from January 1, 2000, to June 30, 2018, with known CLAD status.

Results from randomized trial of pirfenidone in patients with chronic rejection (STOP-CLAD study).

Background: Chronic lung allograft dysfunction (CLAD) is the leading long-term cause of poor outcomes after transplant and manifests by fibrotic remodeling of small airways and/or pleuroparenchymal fibroelastosis. This study evaluated the effect of pirfenidone on quantitative radiographic and pulmonary function assessment in patients with CLAD.

Methods: We performed a single-center, 6-month, randomized, placebo-controlled trial of pirfenidone in patients with CLAD.

Pamrevlumab for Idiopathic Pulmonary Fibrosis: The ZEPHYRUS-1 Randomized Clinical Trial.

Importance: Current treatments for idiopathic pulmonary fibrosis slow the rate of lung function decline, but may be associated with adverse events that affect medication adherence. In phase 2 trials, pamrevlumab (a fully human monoclonal antibody that binds to and inhibits connective tissue growth factor activity) attenuated the progression of idiopathic pulmonary fibrosis without substantial adverse events.

Objective: To assess the efficacy and safety of pamrevlumab for patients with idiopathic pulmonary fibrosis.

Systemic sclerosis associated interstitial lung disease: a conceptual framework for subclinical, clinical and progressive disease.

Objectives: To establish a framework by which experts define disease subsets in systemic sclerosis associated interstitial lung disease (SSc-ILD).

Methods: A conceptual framework for subclinical, clinical and progressive ILD was provided to 83 experts, asking them to use the framework and classify actual SSc-ILD patients. Each patient profile was designed to be classified by at least four experts in terms of severity and risk of progression at baseline; progression was based on 1-year follow-up data.

Radiographic Graft Surveillance in Lung Transplantation: Prognostic Role of Parametric Response Mapping.

Chronic lung allograft dysfunction (CLAD) results in significant morbidity after lung transplantation. Potential CLAD occurs when lung function declines to 80-90% of baseline. Better noninvasive tools to prognosticate at potential CLAD are needed.

Effects of nintedanib by inclusion criteria for progression of interstitial lung disease.

Background: The INBUILD trial investigated nintedanib placebo in patients with progressive fibrosing interstitial lung diseases (ILDs). We investigated the decline in forced vital capacity (FVC) in subgroups based on the inclusion criteria for ILD progression.

Methods: Subjects had a fibrosing ILD other than idiopathic pulmonary fibrosis and met the following criteria for ILD progression within the 24 months before screening despite management deemed appropriate in clinical practice: Group A, relative decline in FVC ≥10% predicted; Group B, relative decline in FVC ≥5-<10% predicted with worsened respiratory symptoms and/or increased extent of fibrosis on high-resolution computed tomography (HRCT); Group C, worsened respiratory symptoms and increased extent of fibrosis on HRCT only.

Effect of Antimicrobial Therapy on Respiratory Hospitalization or Death in Adults With Idiopathic Pulmonary Fibrosis: The CleanUP-IPF Randomized Clinical Trial.

Importance: Alteration in lung microbes is associated with disease progression in idiopathic pulmonary fibrosis.

Objective: To assess the effect of antimicrobial therapy on clinical outcomes.

Design, Setting, And Participants: Pragmatic, randomized, unblinded clinical trial conducted across 35 US sites.

A 53-Year-Old Woman With Dyspnea, Wheezing, and Irreversible Airway Obstruction.

A previously healthy 53-year-old woman with 4 months of dyspnea and subjective wheezing presented to pulmonary clinic for a second opinion. Her medical history included hypertension, obesity, and OSA. She had been hospitalized 3 months prior at an outside hospital for evaluation of these symptoms.

Fibroproliferation in chronic lung allograft dysfunction: Association of mesenchymal cells in bronchoalveolar lavage with phenotypes and survival.

Background: Chronic lung allograft dysfunction (CLAD), the primary cause of poor outcome after lung transplantation, arises from fibrotic remodeling of the allograft and presents as diverse clinical phenotypes with variable courses. Here, we investigate whether bronchoalveolar lavage (BAL) mesenchymal cell activity at CLAD onset can inform regarding disease phenotype, progression, and survival.

Methods: Mesenchymal cell colony-forming units (CFUs) were measured in BAL obtained at CLAD onset (n = 77) and CLAD-free time post-transplant matched controls (n = 77).

Frailty and geriatric conditions in older patients with idiopathic pulmonary fibrosis.

Background: Functional status, an important predictor of health outcomes in older patients, has not been studied in an IPF population. This study aimed to determine the prevalence of frailty and geriatric conditions in older patients with IPF.

Methods: IPF patients age ≥65 years were identified prospectively at the University of Michigan.

Hypersensitivity Pneumonitis: Radiologic Phenotypes Are Associated With Distinct Survival Time and Pulmonary Function Trajectory.

Background: Hypersensitivity pneumonitis (HP) is an interstitial lung disease with a better prognosis, on average, than idiopathic pulmonary fibrosis (IPF). We compare survival time and pulmonary function trajectory in patients with HP and IPF by radiologic phenotype.

Methods: HP (n = 117) was diagnosed if surgical/transbronchial lung biopsy, BAL, and exposure history results suggested this diagnosis.

Development and validation of a radiological diagnosis model for hypersensitivity pneumonitis.

High-resolution computed tomography (HRCT) may be useful for diagnosing hypersensitivity pneumonitis. Here, we develop and validate a radiological diagnosis model and model-based points score.Patients with interstitial lung disease seen at the University of Michigan Health System (derivation cohort) or enrolling in the Lung Tissue Research Consortium (validation cohort) were included.

Parametric Response Mapping as an Imaging Biomarker in Lung Transplant Recipients.

Rationale: The predominant cause of chronic lung allograft failure is small airway obstruction arising from bronchiolitis obliterans. However, clinical methodologies for evaluating presence and degree of small airway disease are lacking.

Objectives: To determine if parametric response mapping (PRM), a novel computed tomography voxel-wise methodology, can offer insight into chronic allograft failure phenotypes and provide prognostic information following spirometric decline.

Idiopathic non-specific interstitial pneumonia.

Non-specific interstitial pneumonia (NSIP) is an interstitial lung disease that may be idiopathic or secondary to connective tissue disease, toxins or numerous other causes. Idiopathic NSIP is a rare diagnosis and requires exclusion of these other possible causes. Patients typically present in mid-adulthood with dyspnoea, cough and often constitutional symptoms including fever and fatigue.

Longitudinal Forced Vital Capacity Monitoring as a Prognostic Adjunct after Lung Transplantation.

Rationale: After lung transplantation, spirometric values are routinely followed to assess graft function. FEV1 is used to characterize chronic allograft dysfunction, whereas the course of FVC change has been less acknowledged and rarely used.

Objectives: To better understand the temporal relationship and prognostic ability of FEV1 and FVC decline after lung transplantation.