Detection of Anti-RuvBL1/2 Autoantibodies by Targeted Liquid Chromatography-Tandem Mass Spectrometry.

Background: Anti-RuvBL1/2 autoantibodies are found in patients with systemic sclerosis and systemic sclerosis-myositis overlap syndrome. Anti-RuvBL1/2 antibodies recognize conformational epitopes of the RuvBL1/2 complex, which complicates detection by solid phase assays. Here, we propose a method for detection of anti-RuvBL1/2 autoantibodies based on liquid phase immunoprecipitation combined with quantification of the precipitated RuvBL1/2 by LC-MS/MS.

External validation of the polymyalgia rheumatica impact scale: a prospective cohort study.

Objectives: To externally validate the PMR impact scale (PMR-IS).

Methods: We conducted a prospective cohort study at the University Hospitals Leuven, Leuven, Belgium. Recently diagnosed PMR patients were included between July 2022 and December 2023 and followed until 1 year after diagnosis.

Anti-TIF1-beta autoantibody-positive dermatomyositis: a case-based review.

To describe the clinical features and cancer risk in patients with dermatomyositis (DM) and anti-TIF1β autoantibodies without other DM-specific autoantibodies (anti-TIF1β-monospecific DM) through a case-based review. Case report of anti-TIF1β-monospecific cancer-associated DM and literature review of cases of anti-TIF1β-monospecific DM. We describe a case of a person with cancer-associated DM in whom we identified anti-TIF1β autoantibodies through immunoprecipitation-mass spectrometry.

Interleukin 6 inhibition in refractory antisynthetase syndrome: case-based literature review.

Introduction: Antisynthetase syndrome (ASyS) is a rare idiopathic inflammatory myopathy (IIM), characterised by the presence of anti-aminoacyl tRNA synthetase antibodies. Significant clinical heterogeneity often results in delayed or missed diagnoses. While corticosteroids are the primary treatment for ASyS, immunosuppressants are frequently added as steroid-sparing agents.

Occupational history questionnaire for job coding and exposure assessment in systemic autoimmune rheumatic diseases.

Objectives: Systemic autoimmune rheumatic diseases (SARDs) develop in genetically susceptible individuals when exposed to environmental factors such as respirable crystalline silica (RCS) particles. Assessing occupational exposure in population-based studies is critical but resource intensive, often requiring expert interviews. This study aimed to develop and validate a self-administered occupational history questionnaire that allows for International Standard Classification of Occupations 1968 (ISCO-68) coding and exposure assessment as a cost-effective alternative to traditional interviews.

Validation of the polymyalgia rheumatica-activity score: A prospective cohort study.

Objective: To validate the polymyalgia rheumatica-activity score (PMR-AS).

Methods: Prospective cohort study at the University Hospitals Leuven (Belgium) between July 2022 and December 2023. We created a new alternative PMR-AS in which ability to elevate the upper limbs (EUL) was replaced by visual analogue scale (VAS) of functionality and both the severity and the duration of stiffness were evaluated.

Direct comparison of the diagnostic accuracy of PET/CT, cranial MRI, ultrasound and temporal artery biopsy in giant cell arteritis.

Purpose: To evaluate the diagnostic accuracy of PET/CT, cranial MRI, ultrasound and temporal artery biopsy (TAB) in patients with suspected giant cell arteritis (GCA) in a direct comparison.

Methods: Consecutive patients with a suspicion of GCA and at least 2 diagnostic tests ≤ 7 days after initiation of glucocorticoids between June 2021 and June 2024, were included retrospectively. The gold standard for the diagnosis of GCA was the judgment of experienced clinicians after a follow-up of ≥ 6 months.

Polymyalgia rheumatica is a risk factor for more recalcitrant disease in giant cell arteritis: A retrospective cohort study.

Objectives: To evaluate differences in presentation and outcome of giant cell arteritis (GCA) patients with and without polymyalgia rheumatica (PMR) symptoms.

Methods: Consecutive patients diagnosed with GCA between 2000 and 2020 and followed for ≥12 months at the University Hospitals Leuven (Belgium), were included retrospectively.

Results: We included 398 GCA patients, of which 181 (45%) with PMR symptoms.

Extracorporeal membrane oxygenation for acute lung injury in idiopathic inflammatory myopathies-a potential lifesaving intervention.

Objectives: Idiopathic inflammatory myopathies (IIM) can present with acute IIM-related lung injury and respiratory failure, leading to a high mortality risk in intensive care units (ICU). Extracorporeal membrane oxygenation (ECMO) in acute respiratory distress syndrome can be lifesaving. We aimed to report a case series of IIM patients that received ECMO.

Biomarker analysis from the phase 2b randomized placebo-controlled trial of riociguat in early diffuse cutaneous systemic sclerosis.

Objective: To examine disease and target engagement biomarkers in the RISE-SSc trial of riociguat in early diffuse cutaneous systemic sclerosis and their potential to predict the response to treatment.

Methods: Patients were randomized to riociguat (n = 60) or placebo (n = 61) for 52 weeks. Skin biopsies and plasma/serum samples were obtained at baseline and week 14.

Molecular characterisation of lupus low disease activity state (LLDAS) and DORIS remission by whole-blood transcriptome-based pathways in a pan-European systemic lupus erythematosus cohort.

Objectives: To unveil biological milieus underlying low disease activity (LDA) and remission versus active systemic lupus erythematosus (SLE).

Methods: We determined differentially expressed pathways (DEPs) in SLE patients from the PRECISESADS project (NTC02890121) stratified into patients fulfilling and not fulfilling the criteria of (1) Lupus LDA State (LLDAS), (2) Definitions of Remission in SLE remission, and (3) LLDAS exclusive of remission.

Results: We analysed data from 321 patients; 40.

Systemic sclerosis and primary biliary cholangitis: Longitudinal data to determine the outcomes.

Background: Several studies described the cross-sectional characteristics of systemic sclerosis patients and coexisting primary biliary cholangitis, but longitudinal prognostic data are lacking.

Aims: To describe the systemic sclerosis-primary biliary cholangitis phenotype, including baseline characteristics and outcomes.

Methods: We performed a multicentre the European Scleroderma Trials and Research Group study of systemic sclerosis patients with primary biliary cholangitis or with primary biliary cholangitis-specific antibodies, matched with systemic sclerosis controls free from hepatobiliary involvement matched for disease duration and cutaneous subset.

Autoantibodies against the melanoma differentiation-associated protein 5 in patients with dermatomyositis target the helicase domains.

Objectives: Clinical observations in patients with dermatomyositis (DM) and autoantibodies against the melanoma differentiation-associated protein 5 (MDA5) suggest that the autoantibodies contribute to the pathogenesis of MDA5(+) DM. To gain insight into the role of the anti-MDA5 autoantibodies, we aimed to identify their binding sites on the different domains of the MDA5 protein.

Methods: We developed an in-house ELISA to assess the reactivity against the MDA5 domains (conformational epitopes) in plasma (n = 8) and serum (n = 24) samples from MDA5(+) patients with varying clinical manifestations and disease outcomes.

Plasma cells are not restricted to the CD27+ phenotype: characterization of CD27-CD43+ antibody-secreting cells.

Unlabelled: Circulating antibody-secreting cells are present in the peripheral blood of healthy individuals reflecting the continued activity of the humoral immune system. Antibody-secreting cells typically express CD27. Here we describe and characterize a small population of antibody-secreting class switched CD19+CD43+ B cells that lack expression of CD27 in the peripheral blood of healthy subjects.

Autoantibodies against the NineTeen complex and U5 RNP in systemic sclerosis.

ObjectiveMultiple spliceosome components are known autoantigens in systemic sclerosis (SSc). Here we aim to identify new and characterize rare anti-spliceosomal autoantibodies in patients with SSc without known autoantibody specificity. MethodsSera that precipitated spliceosome subcomplexes, as detected by immunoprecipitation-mass spectrometry (IP-MS), were identified from a database of 106 patients with SSc without known autoantibody specificity.