Intra- and interobserver reliability in measuring aortic root rotation with cardiac magnetic resonance imaging.

Background: The rotational position of the aortic root (AoR) is of substantial clinical interest as it has been associated with severe aortic complications, such as aortic dissections. We described a cardiac magnetic resonance (CMR)-based method for measuring AoR rotation and evaluated the reliability of measurements.

Methods: CMR was used for measuring AoR rotation in 50 consecutive healthy subjects.

Neurosarcoidosis and Neurologic Complications of Sarcoidosis Treatment.

Sarcoidosis is an immune-mediated multisystem granulomatous disorder. Neurosarcoidosis (NS) accounts for 5% to 35% of cases. The diagnostic evaluation of NS can be a clinical challenge.

Pharmacokinetic and pharmacodynamic properties of once-weekly insulin icodec in individuals with type 2 diabetes.

Aims: To characterize the pharmacokinetic and pharmacodynamic properties of once-weekly insulin icodec in type 2 diabetes (T2D).

Materials And Methods: In an open-label trial, 46 individuals with T2D (18-75 years; body mass index 18.0-38.

Prognostication of progressive pulmonary fibrosis in connective tissue disease-associated interstitial lung diseases: A cohort study.

Background: Connective tissue diseases-associated interstitial lung disease (CTD-ILD) is a heterogeneous condition that impairs quality of life and is associated with premature death. Progressive pulmonary fibrosis (PPF) has been identified as an important risk factor for poor prognosis. However, different criteria for PPF are used in clinical studies, which may complicate comparison between trials and translation of study findings into clinical practice.

High serum neurofilament light chain levels correlate with brain atrophy and physical disability in multiple sclerosis.

Background And Purpose: Serum neurofilament light chain (sNfL) is a promising biomarker of neuroaxonal damage in persons with multiple sclerosis (pwMS). In cross-sectional studies, sNfL has been associated with disease activity and brain magnetic resonance imaging (MRI) changes; however, it is still unclear to what extent in particular high sNfL levels impact on subsequent disease evolution.

Methods: sNfL was quantified by an ultrasensitive single molecule array (Simoa) in 199 pwMS (median age = 34.

Natalizumab in cerebrospinal fluid and breastmilk of patients with multiple sclerosis.

Background: Natalizumab is a highly effective monoclonal antibody for the treatment of multiple sclerosis (MS), which can diffuse in different anatomical compartments, including cerebrospinal fluid (CSF) and milk.

Objectives: Starting from incidental detection of natalizumab in the CSF of MS patients, the objective of this study was to develope a flow-cytometry-based assay and apply it to quantify natalizumab in body fluids, including milk collected from nursing patients over 180 days and in patients with neutralizing antibodies against natalizumab.

Methods: CSF, milk and sera samples from patients with multiple sclerosis were tested by flow-cytometry for binding to a VLA-4 expressing cell line or to a control cell line.

Strategies for low-molecular-weight heparin management in pregnant women with mechanical prosthetic heart valves: A nationwide survey of Dutch practice.

Background: In this study we investigated current Dutch practice of low molecular weight heparin (LMWH) treatment in pregnant women with mechanical prosthetic heart valves (MPHV) in order to evaluate how management can be optimized.

Methods: Between December 2020 and February 2021, we conducted a survey among Dutch congenital cardiologists of tertiary centers in the Netherlands. We collected and analyzed written, unstructured, open questionnaires that were send to all 8 specialized pregnancy heart teams.

Clinical features and immune-related protein patterns of anti-MDA5 positive clinically amyopathic dermatomyositis Dutch patients.

Objectives: The presence of melanoma differentiation-associated protein 5 (MDA5) antibodies in patients with DM is associated with the development of a rapidly progressive interstitial lung disease (RPILD), unresponsive to conventional treatment. We characterize patients and provide more insight into potential biomarkers to identify patients with RPILD.

Methods: Patients diagnosed with anti-MDA5 positive DM between December 2015 and November 2017 were included in this study.

Decreased Cerebrospinal Fluid Antioxidative Capacity Is Related to Disease Severity and Progression in Early Multiple Sclerosis.

Oxidative stress-induced neuronal damage in multiple sclerosis (MS) results from an imbalance between toxic free radicals and counteracting antioxidants, i.e., antioxidative capacity (AOC).

Predictors for progressive fibrosis in patients with connective tissue disease associated interstitial lung diseases.

Background: Connective tissue disease associated interstitial lung disease (CTD-ILD) is associated with decreased quality of life and high mortality risk. Outcome and treatment response is unpredictable. This study aimed to identify clinical predictors for CTD-ILD with poor outcome.

Autologous stem-cell transplantation in systemic sclerosis-associated interstitial lung disease: early action in selected patients rather than escalation therapy for all.

Systemic sclerosis (SSc) is a rare rheumatic disease characterised by inflammation, vasculopathy and fibrosis of skin and internal organs. A common complication and a leading cause of death in SSc is interstitial lung disease (ILD). The current armamentarium of treatments in SSc-ILD mainly includes immunosuppressive therapies and has recently been expanded with anti-fibrotic agent nintedanib.

CSF SERPINA3 Levels Are Elevated in Patients With Progressive MS.

Objective: To identify biomarkers associated with progressive phases of MS and with neuroprotective potential.

Methods: Combined analysis of the transcriptional and proteomic profiles obtained in CNS tissue during chronic progressive phases of experimental autoimmune encephalomyelitis (EAE) with the transcriptional profile obtained during the differentiation of murine neural stem cells into neurons. Candidate biomarkers were measured by ELISA in the CSF of 65 patients with MS (29 with relapsing-remitting MS [RRMS], 20 with secondary progressive MS, and 16 with primary progressive MS [PPMS]) and 30 noninflammatory neurologic controls (NINCs).

The Burden of Neurosarcoidosis: Essential Approaches to Early Diagnosis and Treatment.

Neurosarcoidosis (NS) is an often severe, destructive manifestation with a likely under-reported prevalence of 5 to 15% of sarcoidosis cases, and in its active phase demands timely treatment intervention. Clinical signs and symptoms of NS are variable and wide-ranging, depending on anatomical involvement. Cranial nerve dysfunction, cerebrospinal parenchymal disease, aseptic meningitis, and leptomeningeal disease are the most commonly recognized manifestations.

Serum neurofilament light levels in normal aging and their association with morphologic brain changes.

Neurofilament light (NfL) protein is a marker of neuro-axonal damage and can be measured not only in cerebrospinal fluid but also in serum, which allows for repeated assessments. There is still limited knowledge regarding the association of serum NfL (sNfL) with age and subclinical morphologic brain changes and their dynamics in the normal population. We measured sNfL by a single molecule array (Simoa) assay in 335 individuals participating in a population-based cohort study and after a mean follow-up time of 5.

Serum neurofilament light levels correlate with change of olfactory function in multiple sclerosis.

Background: Serum neurofilament light chain levels (sNfL) and impairment of olfactory function emerge as biomarkers in multiple sclerosis (MS). However, the relation between sNfL and olfactory function in MS has not been investigated yet.

Objective: We aimed to determine whether sNfL levels correlate with olfactory function in relapsing-remitting (RR) MS.

Serum neurofilament levels correlate with retinal nerve fiber layer thinning in multiple sclerosis.

Background: Serum neurofilament light chain (sNfL) levels and peripapillary retinal nerve fiber layer (pRNFL) are both emerging biomarkers of neuro-axonal damage in multiple sclerosis (MS). However, data on the relation between sNfL and pRNFL are scarce.

Objective: We aimed to determine the relation of sNfL levels with pRNFL thinning in a large cohort of relapsing-remitting (RR) MS patients.

Quality of Life of Couples Living with Sarcoidosis.

Background: Consequences of sarcoidosis are wide ranging, and the symptom burden has a great impact on patients' quality of life (QoL). However, the QoL of couples living with sarcoidosis has not yet been studied.

Objectives: Our aim was to assess the QoL of couples living with sarcoidosis and to evaluate whether living with a partner with sarcoidosis influences the partner's QoL.

Kappa free light chains is a valid tool in the diagnostics of MS: A large multicenter study.

Objective: To validate kappa free light chain (KFLC) and lambda free light chain (LFLC) indices as a diagnostic biomarker in multiple sclerosis (MS).

Methods: We performed a multicenter study including 745 patients from 18 centers (219 controls and 526 clinically isolated syndrome (CIS)/MS patients) with a known oligoclonal IgG band (OCB) status. KFLC and LFLC were measured in paired cerebrospinal fluid (CSF) and serum samples.

Correction to: The Burden of Sarcoidosis Symptoms from a Patient Perspective.

The original version of this article unfortunately contained a mistake in coauthor's given name. The abbreviated given name "M.D.

Management of neurosarcoidosis: a clinical challenge.

Purpose Of Review: Sarcoidosis is a complex disease with many faces, and the clinical manifestation and course of neurosarcoidosis are particularly variable. Although neurosarcoidosis occurs in up to 10% of sarcoidosis patients, it can lead to significant morbidity and some mortality.

Recent Findings: Three criteria are usually required for a diagnosis of (neuro)sarcoidosis: clinical and radiologic manifestations, noncaseating granulomas, and no evidence of alternative disease.

The Burden of Sarcoidosis Symptoms from a Patient Perspective.

Purpose: The clinical manifestations of sarcoidosis vary widely, depending on the intensity of the inflammation and the organ systems affected. Hence, sarcoidosis patients may suffer from a great variety of symptoms. The aim of this study was to compare the self-reported burden of sarcoidosis patients in Denmark, Germany and the Netherlands, especially the prevalence of fatigue and small fiber neuropathy (SFN)-related symptoms, as well as differences in treatment strategies.

Everyday cognitive failure in patients suffering from neurosarcoidosis.

Background: Cognitive failure is associated with memory and concentration problems. Previously, a prevalence of one third was found in a general sarcoidosis population. The aim of this study was to assess if neurosarcoidosis patients are at higher risk for developing everyday cognitive failure using the Cognitive Failure Questionnaire (CFQ) and to determine what factors were associated with cognitive failure.