Sarcoidosis Treatment Patterns in the United States: 2016-2022.

Background: There are limited US Federal Drug Administration-approved medications and real-world data on sarcoidosis treatment in the United States. Concordance of practice patterns with guideline recommendations have not been well characterized.

Research Question: What are the practice patterns and factors associated with treatment for patients with sarcoidosis in the year following diagnosis?

Study Design And Methods: A retrospective analysis was conducted of patients with sarcoidosis from 2016 to 2022 using a multicenter, all-payer claims database (TriNetX).

A prospective, single-center, randomized phase 2 trial of etoposide in severe COVID-19.

The systemic inflammatory response seen in patients with severe COVID-19 shares many similarities with the changes observed in hemophagocytic lymphohistiocytosis (HLH); a disease characterized by excessive immune activation. Many patients with severe COVID qualify for a diagnosis of HLH. Etoposide, an inhibitor of topoisomerase II is used to control inflammation in HLH.

Critical Care of Patients With Cardiopulmonary Complications of Sarcoidosis.

Sarcoidosis is a systemic inflammatory disease defined by the presence of aberrant granulomas affecting various organs. Due to its multisystem involvement, care of patients with established sarcoidosis becomes challenging, especially in the intensive care setting. While the lungs are typically involved, extrapulmonary manifestations also occur either concurrently or exclusively within a significant proportion of patients, complicating diagnostic and management decisions.

Treatment of newly diagnosed sarcoid-associated pulmonary hypertension with ambrisentan and tadalafil combination therapy.

Sarcoid Associated Pulmonary Hypertension (SAPH) is a common complication of sarcoidosis and is associated with poor prognosis. SAPH can be due to multiple synergistic mechanisms and current therapeutic strategies treat systemic sarcoidosis and pulmonary hypertension separately. Several studies have been performed to develop an effective therapy for SAPH but have been met with mixed results.

Long-term outcomes of epoprostenol therapy in sarcoid associated pulmonary hypertension.

Sarcoidosis-Associated Pulmonary Hypertension (SAPH) is a common finding in patients with chronic sarcoidosis and is associated with increased mortality. The optimal treatment for SAPH is not known; however, therapies approved for Group 1 pulmonary hypertension have improved hemodynamics and functional status. Prostanoids, including epoprostenol, have been therapeutic in short-term studies of SAPH, but long-term efficacy is unknown.

Diagnosis and Detection of Sarcoidosis. An Official American Thoracic Society Clinical Practice Guideline.

The diagnosis of sarcoidosis is not standardized but is based on three major criteria: a compatible clinical presentation, finding nonnecrotizing granulomatous inflammation in one or more tissue samples, and the exclusion of alternative causes of granulomatous disease. There are no universally accepted measures to determine if each diagnostic criterion has been satisfied; therefore, the diagnosis of sarcoidosis is never fully secure. Systematic reviews and, when appropriate, meta-analyses were performed to summarize the best available evidence.

Comparison of Ultrasound Features of Major Salivary Glands in Sarcoidosis, Amyloidosis, and Sjögren's Syndrome.

Objective: While salivary gland ultrasound (SGU) has gained prominence for evaluating Sjögren's syndrome, little information exists on SGU appearance of sarcoidosis and amyloidosis, potential mimics of Sjögren's syndrome. Our goal in this study was to estimate the diagnostic accuracy of major SGU features in differentiating Sjögren's syndrome from sarcoidosis, amyloidosis, and controls.

Methods: We enrolled consecutive adult ambulatory patients with a clinical diagnosis of Sjögren's syndrome fulfilling the 2016 American College of Rheumatology (ACR) classification criteria; we also enrolled patients with a clinical diagnosis of sarcoidosis or systemic immunoglobulin light chain (AL) amyloidosis, with histologic confirmation from any tissue, and rheumatology outpatients without diagnoses affecting salivary glands.

Obesity and sarcoidosis: consequence or contributor?

Purpose Of Review: Sarcoidosis is a multisystem disease of unknown cause. Obesity can affect many physiological factors. The relationship between obesity and sarcoidosis is unclear, and can been described as posing a 'chicken and egg' scenario for the patient as it is not always clear whether it is a consequence of, or a risk factor for any disease.

Transbronchial biopsies safely diagnose amyloid lung disease.

Background: Autopsy identifies lung involvement in 58-92% of patients with the most prevalent forms of systemic amyloidoses. In the absence of lung biopsies, amyloid lung disease often goes unrecognized. Report of a death following transbronchial biopsies in a patient with systemic amyloidosis cautioned against the procedure in this patient cohort.

The response of FDG uptake to immunosuppressive treatment on FDG PET/CT imaging for cardiac sarcoidosis.

Background: Immunosuppression is used to treat cardiac sarcoidosis, despite limited data. FDG PET/CT is used for detecting cardiac inflammation in patients with CS, yet there is variability in interpretation of FDG PET/CT. Our aim was to compare quantitative and qualitative interpretation of FDG PET/CT for CS in defining the FDG response to immunosuppression.

The Diagnosis of Sarcoidosis.

Sarcoidosis is a diagnosis of exclusion; there exists neither a pathognomonic clinical feature nor a perfect diagnostic test. Missed diagnosis and overdiagnosis are common. A careful history and physical examination look for "footprints" of sarcoidosis or features suggesting alternative diagnoses.

Obesity and weight gain in relation to incidence of sarcoidosis in US black women: data from the Black Women's Health Study.

Background: Sarcoidosis, a systemic disorder characterized by chronic granulomatous inflammation, occurs more frequently among US black women, as do overweight and obesity. Little is known about the relation of overweight and obesity, which induce chronic inflammation, to incidence of sarcoidosis.

Methods: We assessed the relation of obesity and weight gain to the incidence of sarcoidosis in the Black Women's Health Study, a follow-up study of 59,000 US black women aged 21 to 69 years at baseline in 1995.

Quantitative interpretation of FDG PET/CT with myocardial perfusion imaging increases diagnostic information in the evaluation of cardiac sarcoidosis.

Background: FDG PET/CT with myocardial perfusion imaging is a useful method for evaluating cardiac sarcoidosis (CS), but interpretation is not standardized. We developed a method for quantification of cardiac FDG PET/CT and evaluated its relationship to conventional interpretation, perfusion defects, clinical events, and immunosuppressive treatment.

Methods And Results: FDG PET/CT with MPI studies performed for CS (n = 38) were retrospectively compared to negative control studies acquired for oncologic indications (n = 10).

Cavin1; a regulator of lung function and macrophage phenotype.

Caveolae are cell membrane invaginations that are highly abundant in adipose tissue, endothelial cells and the lung. The formation of caveolae is dependent on the expression of various structural proteins that serve as scaffolding for these membrane invaginations. Cavin1 is a newly identified structural protein whose deficiency in mice leads to loss of caveolae formation and to development of a lipodystrophic phenotype.

Proteomics and the lung: Analysis of bronchoalveolar lavage fluid.

Our knowledge of the complex bronchoalveolar lavage fluid (BALF) proteome has increased significantly over the last decade; but still, there remain many aspects of the BALF proteome that need characterization. Current proteomic methodologies resolve proteins within limited dynamic ranges: thereby, being limited in their ability to examine important areas of the BALF proteome, such as low molecular weight, low abundance proteins. To ensure proper coverage of these proteins in the BALF proteome, a refined 2-DE standard operation protocol is presented, highlighting important issues in sample collection, sample preparation, and 2-D DIGE analysis.

Role of proteomics in the investigation of pulmonary fibrosis.

Pulmonary fibrosis arises as a consequence of aberrant remodeling and defective repair mechanisms within the lung. This destructive process is the cause of much of the morbidity and mortality in many pulmonary disorders. Unfortunately, therapeutic options are limited.