VEXAS syndrome: complete molecular remission after hypomethylating therapy.

The VEXAS syndrome, a genetically defined autoimmune disease, associated with various hematological neoplasms has been attracting growing attention since its initial description in 2020. While various therapeutic strategies have been explored in case studies, the optimal treatment strategy is still under investigation and allogeneic cell transplantation is considered the only curative treatment. Here, we describe 2 patients who achieved complete molecular remission of the underlying UBA1 mutant clone outside the context of allogeneic HCT.

SLE criteria are by necessity still based on clinical (and immunological) criteria items.

Introduction: The 2019 European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria for systemic lupus erythematosus (SLE) rely on clinical and routine immunological items. The criteria have anti-nuclear antibodies (ANA) as an obligatory entry criterion; items are weighted and ordered in domains. While demonstrating good sensitivity and specificity, the lack of a more molecular approach to some came as a disappointment.

Physical exercise is associated with less fatigue, less pain and better sleep in patients with systemic lupus erythematosus.

Objectives: While fatigue is an extremely common complaint of patients with systemic lupus erythematosus (SLE), the information on factors influencing SLE fatigue in the long term is still limited. This study aims to investigate the actual level of physical activity in the daily life of SLE outpatients and its association with fatigue.

Methods: In a cross-sectional survey on 93 SLE outpatients we combined clinical assessment with questionnaires to comprehensively assess SLE fatigue, its impact on function and health-related quality of life (hrQoL), and potential contributing factors, with particular emphasis on physical activity.

Effects of nintedanib in patients with limited cutaneous systemic sclerosis and interstitial lung disease.

Objectives: To investigate the course of interstitial lung disease (ILD) and the effects of nintedanib in patients with limited cutaneous systemic sclerosis (lcSSc).

Methods: In the SENSCIS trial, patients with SSc-ILD were randomized to receive nintedanib or placebo. Patients who completed the SENSCIS trial were eligible to enter SENSCIS-ON, in which all patients received open-label nintedanib.

Is the Rheumatoid Arthritis Impact of Disease (RAID) score a meaningful instrument for other inflammatory rheumatic diseases? A cross-sectional analysis of data from the German National Database.

ObjectiveTo analyse the performance of the rheumatoid arthritis impact of disease (RAID) score in patients with ankylosing spondylitis, polymyalgia rheumatica, systemic lupus erythematosus, primary Sjögren's syndrome, idiopathic inflammatory myositis and systemic sclerosis, as compared with rheumatoid arthritis (RA).A total of 12 398 patients from the German National Database were included. For each diagnosis, we calculated age-adjusted/sex-adjusted partial correlation coefficients between RAID and patient global (PtGl) health, PtGl disease activity, physician global (PhGl) disease activity, Well-Being Index (WHO-5) and EuroQoL-5 Dimensions (EQ-5D).

Advances in SLE classification criteria.

This year, the American College of Rheumatology (ACR) 1982 classification criteria for systemic lupus erythematosus (SLE) celebrate their 40th anniversary. From this start, the quest for optimal SLE criteria has led to the 1997 ACR update, the 2012 publication of the Systemic Lupus International Collaborating Clinics (SLICC) criteria, and, in 2019, the European League Against Rheumatism (EULAR)/ACR classification criteria. The latter have since been externally validated in more than two dozen studies and have become the gold standard inclusion criterion of SLE clinical trials.

Evaluation of the EULAR/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus in a Population-Based Registry.

Objective: Using the Manhattan Lupus Surveillance Program, a multiracial/ethnic population-based registry, we aimed to compare 3 commonly used classification criteria for systemic lupus erythematosus (SLE) to identify unique cases and determine the incidence and prevalence of SLE using the EULAR/American College of Rheumatology (ACR) criteria.

Methods: SLE cases were defined as fulfilling the 1997 ACR, the Systemic Lupus International Collaborating Clinics (SLICC), or the EULAR/ACR classification criteria. We quantified the number of cases uniquely associated with each and the number fulfilling all 3 criteria.

Assessing the EULAR/ACR classification criteria for patients with systemic lupus erythematosus.

Introduction: The European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria for systemic lupus erythematosus (SLE) established a new criteria system with antinuclear antibodies (ANA) as an entry criterion, domains, and weighted criteria. In the validation cohort, specificity and sensitivity each matched the best performance of the previous criteria sets.

Areas Covered: Groups worldwide have externally validated the EULAR/ACR SLE classification criteria.

Glucocorticoid dosing and relapses in giant cell arteritis-a single centre cohort study.

Objective: To investigate the relationship between real life glucocorticoid (GC) dosing and relapse rates in patients with new onset GCA in a single centre.

Methods: Complete clinical data taken from the inpatient and outpatient records of consecutive GCA patients followed beyond stopping GC were retrospectively analysed for GC doses, other immunomodulatory agents and relapses.

Results: We included 54 patients with GCA confirmed by biopsy or imaging and followed over their complete GC course.

Comorbidity and healthcare utilisation in persons with incident systemic lupus erythematosus followed for 3 years after diagnosis: analysis of a claims data cohort.

Objective: To analyse comorbidity and healthcare utilisation in individuals with SLE.

Methods: A cohort of individuals with incident SLE diagnosis in 2016 were investigated using claims data from a German statutory health insurance fund. Concomitant diagnoses, medical prescriptions, hospitalisation and sick leave were analysed in the year prior to diagnosis and during a 3-year follow-up in comparison with age-matched and sex-matched controls (1) without autoimmune diseases and (2) with incident diabetes mellitus.

What are the topics you care about making trials in lupus more effective? Results of an Open Space meeting of international lupus experts.

Despite promising candidates for new therapeutic options in the treatment of systemic lupus erythematosus (SLE), many clinical trials have failed in the past few years. The disappointing results have been at least partly be attributed to trial designs. With the aim of stimulating new developments in SLE trial design, an international open space meeting was held on occasion of the European Lupus Meeting 2018 in Duesseldorf, Germany about 'What are the topics you care about for making trials in lupus more effective?'.

Relevance of immunomodulatory therapy for interstitial lung disease in systemic sclerosis.

Systemic sclerosis is an autoimmune disease that prominently leads to skin and tissue fibrosis. The efficacy of autologous stem cell transplantation not only attests to the autoimmune pathophysiology for systemic sclerosis, but also for interstitial lung disease as its most frequent manifestation of fatal organ involvement. Accordingly, a variety of immunomodulatory therapies were tried on patients with systemic sclerosis-interstitial lung disease.

New lupus criteria: a critical view.

Purpose Of Review: To review the validation of the European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) 2019 classification criteria for systemic lupus erythematosus (SLE).

Recent Findings: Positive antinuclear antibodies, which constitute the obligatory entry criterion of the EULAR/ACR criteria, were found in the vast majority of SLE patients worldwide, with 97% (94-100%) of patients antinuclear antibodies positive in studies investigating EULAR/ACR criteria performance. Combined over the publications, EULAR/ACR criteria sensitivity was 92% (range 85-97%).

Classifying and diagnosing systemic lupus erythematosus in the 21st century.

The EULAR/ACR 2019 classification criteria for SLE constitute a current and optimized clinical approach to SLE classification. Classification is still not based on molecular approaches and the results from large studies using polyomics may be interpreted as demonstrating the relevance of the genetic and environmental background rather than splitting SLE into several entities. In fact, an association study within the EULAR/ACR classification criteria project found associations between manifestations only within organ domains.

Effect of Nintedanib on Lung Function in Patients With Systemic Sclerosis-Associated Interstitial Lung Disease: Further Analyses of a Randomized, Double-Blind, Placebo-Controlled Trial.

Objective: In the SENSCIS trial in subjects with systemic sclerosis-associated interstitial lung disease (SSc-ILD), nintedanib reduced the rate of decline in forced vital capacity (FVC) over 52 weeks by 44% versus placebo. This study was undertaken to investigate the effects of nintedanib on categorical changes in FVC and other measures of ILD progression.

Methods: In post hoc analyses, we assessed the proportions of subjects with categorical changes in FVC % predicted at week 52 and the time to absolute decline in FVC of ≥5% predicted or death and absolute decline in FVC of ≥10% predicted or death.

New classification criteria for systemic lupus erythematosus.

Purpose Of Review: To compare the recently published European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) classification criteria for SLE with the Systemic Lupus International Collaborating Centers (SLICC) criteria and the earlier ACR criteria, focusing on their key concepts.

Recent Findings: Although the SLICC criteria introduced numbers of new criteria items, the new EULAR/ACR criteria added only noninfectious fever, based on an early SLE cohort study and an SLE patient survey, and condensed hematological, mucocutaneous and neurological items. Whereas the SLICC criteria maintained the overall structure familiar from the ACR criteria, the EULAR /ACR criteria use antinuclear antibodies (ANA) as an obligatory entry criterion, have weighted criteria and group these in domains.

New Criteria for Lupus.

Purpose Of The Review: Classification criteria define the patient population for clinical trials and translational studies, but also influence current understanding of the disease. This review attempts to delineate the development from the American College of Rheumatology (ACR) 1982 to the European League Against Rheumatism (EULAR)/ACR 2019 classification criteria for systemic lupus erythematosus (SLE).

Recent Findings: The new EULAR/ACR classification criteria use antinuclear antibodies (ANA) as an entry criterion.

EULAR recommendations for the management of rheumatoid arthritis with synthetic and biological disease-modifying antirheumatic drugs: 2019 update.

Objectives: To provide an update of the European League Against Rheumatism (EULAR) rheumatoid arthritis (RA) management recommendations to account for the most recent developments in the field.

Methods: An international task force considered new evidence supporting or contradicting previous recommendations and novel therapies and strategic insights based on two systematic literature searches on efficacy and safety of disease-modifying antirheumatic drugs (DMARDs) since the last update (2016) until 2019. A predefined voting process was applied, current levels of evidence and strengths of recommendation were assigned and participants ultimately voted independently on their level of agreement with each of the items.