Diagnosis and Prognosis of Isolated Cardiac Sarcoidosis: Multidisciplinary Diagnosis Versus Japanese Circulation Society Criteria.

The prognosis of isolated cardiac sarcoidosis (ICS) has not been fully elucidated. The only diagnostic criteria available for ICS, the Japanese Circulation Society (JCS) criteria, have not been validated. We aimed to study the prognosis of ICS according to 2 different diagnostic strategies.

Partnering With Patients With Sarcoidosis to Implement a Community Advisory Board.

Background: Community advisory boards (CABs) are increasingly recognized as a means of incorporating patient experience into clinical practice and research. The power of CABs is derived from engaging with community members as equals throughout the research process. Despite this, little is known of community member experience and views on best practices for running a CAB in a rare pulmonary disease.

Cardiac Sarcoidosis.

Cardiac involvement is a major cause of morbidity and mortality in patients with sarcoidosis. It is important to distinguish between clinical manifest diseases from clinically silent diseases. Advanced cardiac imaging studies are crucial in the diagnostic pathway.

Response to Acthar Gel in sarcoidosis uveitis: A prospective open label study.

Purpose: To investigate the response to Acthar Gel® in patients with moderate to severe sarcoidosis uveitis.

Methods: This is a prospective open-label study that enrolled patients with moderate to severe sarcoidosis uveitis to receive 80 units daily of Acthar Gel for ten days followed by maintenance treatment with 80 units twice weekly. The primary outcome was the proportion of patients meeting at least one of the following variables 1) improved visual acuity, 2) resolution of intraocular inflammation, 3) ability to taper ocular or oral steroids by at least 50% or 4) reduction of cystoid macular edema, with no worsening of any single measure and no need for additional sarcoidosis therapies at 24 weeks.

Optimal left ventricular ejection fraction in risk stratification of patients with cardiac sarcoidosis.

Aims: Identifying patients with cardiac sarcoidosis (CS) who are at an increased risk of sudden cardiac death (SCD) poses a clinical challenge. We sought to identify the optimal cutoff for left ventricular ejection fraction (LVEF) in predicting ventricular arrhythmia (VA) and all-cause mortality and to identify clinical and imaging risk factors in patients with known CS.

Methods And Results: This retrospective cohort included 273 patients with well-established CS.

Interstitial lung disease progression after genomic usual interstitial pneumonia testing.

Background: A genomic classifier for usual interstitial pneumonia (gUIP) has been shown to predict histological UIP with high specificity, increasing diagnostic confidence for idiopathic pulmonary fibrosis (IPF). Whether those with positive gUIP classification exhibit a progressive, IPF-like phenotype remains unknown.

Methods: A pooled, retrospective analysis of patients who underwent clinically indicated diagnostic bronchoscopy with gUIP testing at seven academic medical centres across the USA was performed.

Neurosarcoidosis: an update on diagnosis and therapy.

Introduction: Clinically overt granulomatous involvement of the nervous system (i.e. neurosarcoidosis) can be seen in up to 10% of patients with sarcoidosis.

Prospective cohort of cryobiopsy in interstitial lung diseases: a single center experience.

Rationale: Transbronchial cryobiopsy has been increasingly used to diagnose interstitial lung diseases. However, there is uncertainty regarding its accuracy and risks, mainly due to a paucity of prospective or randomized trials comparing cryobiopsy to surgical biopsy.

Objectives: To evaluate the diagnostic yield and complications of cryobiopsy in patients selected by multidisciplinary discussion.

Case report of isolated cardiac sarcoidosis presenting as hypertrophic obstructive cardiomyopathy-a clinical picture printed on lenticular paper.

Background: Cardiac sarcoidosis (CS) is an inflammatory granulomatous process of the myocardium that can be asymptomatic or have several different clinical phenotypes. One of its rarely described presentations consists of hypertrophy of the septal myocardium, similar to hypertrophic cardiomyopathy (HCM). Isolated cardiac sarcoidosis that haemodynamically mimics hypertrophic obstructive cardiomyopathy (HOCM) has been rarely described in the literature.

The utility of endobronchial ultrasound-transbronchial needle aspiration in patients with suspected extra-pulmonary sarcoidosis without thoracic lymphadenopathy.

Background: Diagnosis of extra-pulmonary sarcoidosis can be difficult, and a biopsy is usually required. We evaluated the utility of endobronchial ultrasound-transbronchial needle aspiration (EBUS-TBNA) in patients with suspected extra-pulmonary sarcoidosis with thoracic lymph nodes ≤10 mm on chest computed tomography (CT) and no or minimal pulmonary infiltrates.

Methods: The Cleveland Clinic bronchoscopy registry was screened.

Performance of diagnostic criteria in patients clinically judged to have cardiac sarcoidosis: Is it time to regroup?

Background: The diagnosis of cardiac sarcoidosis (CS) is challenging. Because of the current limitations of endomyocardial biopsy as a reference standard, physicians rely on advanced cardiac imaging, multidisciplinary evaluation, and diagnostic criteria to diagnose CS.

Aims: To compare the 3 main available diagnostic criteria in patients clinically judged to have CS.

Income and Other Contributors to Poor Outcomes in U.S. Patients with Sarcoidosis.

Socioeconomic factors are associated with worse disease severity at presentation in sarcoidosis, but the relative importance of socioeconomic variables on morbidity and disease burden has not been fully elucidated. To determine the association between income and sarcoidosis outcomes after controlling for socioeconomic and disease-related factors. Using the Sarcoidosis Advanced Registry for Cures database, we analyzed data from 2,318 patients with sarcoidosis in the United States to determine the effect of income and other variables on outcomes.

Infliximab for Refractory Cardiac Sarcoidosis.

Cardiac sarcoidosis (CS) is frequently difficult to treat. Infliximab (IFX) is useful for extracardiac sarcoidosis, but its use in CS has been limited due to concerns about cardiotoxicity and an FDA blackbox warning about use in heart failure. We reviewed 36 consecutive patients treated with infliximab for CS refractory to standard therapies.

Update in Cardiac Sarcoidosis.

Increasing awareness of cardiac manifestations of sarcoidosis and the widespread availability of advanced imaging tests have led to a tidal wave of interest in a condition that was once considered rare. In this Focused Review, we explore important clinical questions that may confront specialists faced with possible cardiac involvement. In the absence of an ideal reference standard, three main sets of clinical criteria exist: the Japanese Ministry of Health and Welfare, the Heart Rhythm Society, and the World Association for Sarcoidosis and Other Granulomatous Disorders criteria.

Toxicity risk from glucocorticoids in sarcoidosis patients.

Background: Glucocorticoids (GC) are considered first-line therapy for treating sarcoidosis, but there are few data about the adverse consequences of GC. Although there are several steroid-sparing medications available for treatment, a large proportion of patients are treated with prolonged courses of GC. The toxicities of GC in sarcoidosis populations have not been carefully evaluated.

Venous Thromboembolism After Adult Lung Transplantation: A Frequent Event Associated With Lower Survival.

Background: The incidence of venous thromboembolism (VTE) after lung transplantation (LTX) varies significantly across studies. Two studies have suggested that these thrombotic events are associated with a lower posttransplant survival. Herein, we sought to determine the incidence, predictors, and impact of VTE on survival after LTX at a quaternary referral center.