Anti-Sp4 and Anti-CCAR1 autoantibodies in UK vs. US patients with adult and juvenile-onset anti-TIF1Æ´-positive myositis.

Objectives: Anti-TIF1γ autoantibodies are associated with malignancy in adult-onset idiopathic inflammatory myopathy (IIM) and this risk is attenuated if patients are also positive for anti-specificity protein 4 (Sp4) or anti-cell division cycle apoptosis regulator protein 1 (CCAR1). In anti-TIF1γ positive US dermatomyositis (DM) patients, anti-Sp4 and anti-CCAR1 autoantibody frequencies are reported as 32% and 43% in adults and 9% and 19% in juveniles, respectively. This study aims to identify the frequency of anti-Sp4 and anti-CCAR1 in adult and juvenile UK anti-TIF1ƴ-positive myositis populations and report clinical associations.

Comparison of clinical features between patients with anti-synthetase syndrome and dermatomyositis: results from the MYONET registry.

Objectives: To compare clinical characteristics, including the frequency of cutaneous, extramuscular manifestations and malignancy, between adults with anti-synthetase syndrome (ASyS) and DM.

Methods: Using data regarding adults from the MYONET registry, a cohort of DM patients with anti-Mi2/-TIF1γ/-NXP2/-SAE/-MDA5 autoantibodies, and a cohort of ASyS patients with anti-tRNA synthetase autoantibodies (anti-Jo1/-PL7/-PL12/-OJ/-EJ/-Zo/-KS) were identified. Patients with DM sine dermatitis or with discordant dual autoantibody specificities were excluded.

Investigating characteristics of idiopathic inflammatory myopathy flares using daily symptom data collected via a smartphone app.

Objective: The objective of this study was to use daily data collected via a smartphone app for characterization of patient-reported and symptom-based (using an a priori definition) flares in an adult idiopathic inflammatory myopathy (IIM) cohort.

Methods: UK adults with an IIM answered patient-reported outcome measurements (PROMs) daily via a smartphone app during a 91-day study. Daily symptom PROMs addressed global activity, overall pain, myalgia, fatigue, and weakness (on a 0-100 visual analogue scale).

A systematic review and meta-analysis to inform cancer screening guidelines in idiopathic inflammatory myopathies.

Objectives: To identify clinical factors associated with cancer risk in the idiopathic inflammatory myopathies (IIMs) and to systematically review the existing evidence related to cancer screening.

Methods: A systematic literature search was carried out on Medline, Embase and Scopus. Cancer risk within the IIM population (i.

Patient insights on living with idiopathic inflammatory myopathy and the limitations of disease activity measurement methods - a qualitative study.

Background: The idiopathic inflammatory myopathies (IIMs) are chronic autoimmune conditions, typically resulting in proximal muscle weakness and impacting upon quality of life. Accurate measurement of IIM disease activity is imperative for appropriate medical management and carrying out valid clinical trials. The International Myositis Assessment and Clinical Studies Group (IMACS) "Disease Activity Core Set Measures" are the current gold-standard of IIM disease activity assessment.

Long-term strength and functional status in inclusion body myositis and identification of trajectory subgroups.

Introduction: Objective information on longitudinal disease progression in inclusion body myositis (IBM) is lacking.

Methods: Longitudinal dynamometry and functional status data were collated from a cohort of IBM patients. Annual change was calculated by means of linear modeling.

A review of accelerometer-derived physical activity in the idiopathic inflammatory myopathies.

Background: The idiopathic inflammatory myopathies (IIMs) are a group of rare conditions characterised by muscle inflammation (myositis). Accurate disease activity assessment is vital in both clinical and research settings, however, current available methods lack ability to quantify associated variation of physical activity, an important consequence of myositis.This study aims to review studies that have collected accelerometer-derived physical activity data in IIM populations, and to investigate if these studies identified associations between physical and myositis disease activity.

Increasing incidence of adult idiopathic inflammatory myopathies in the City of Salford, UK: a 10-year epidemiological study.

Objectives: The aim was to identify and characterize all incident adult cases of idiopathic inflammatory myopathies (IIM) between 1 January 2007 and 31 December 2016 in the City of Salford, UK.

Methods: Adults first diagnosed with IIM within the study period were identified by: a Salford Royal NHS Foundation Trust (SRFT) inpatient episode IIM-specific ICD-10 coding search; all new patient appointments to SRFT neuromuscular outpatient clinics; and all Salford residents enrolled within the UKMYONET study. All patients with definite IIM by the 2017 EULAR/ACR classification criteria were included, as were probable cases if consensus expert opinion agreed.

The temporal relationship between cancer and adult onset anti-transcriptional intermediary factor 1 antibody-positive dermatomyositis.

Objectives: To characterize the 10 year relationship between anti-transcriptional intermediary factor 1 antibody (anti-TIF1-Ab) positivity and cancer onset in a large UK-based adult DM cohort.

Methods: Data from anti-TIF1-Ab-positive/-negative adults with verified diagnoses of DM from the UK Myositis Network register were analysed. Each patient was followed up until they developed cancer.

The performance of the European League Against Rheumatism/American College of Rheumatology idiopathic inflammatory myopathies classification criteria in an expert-defined 10 year incident cohort.

Objectives: To assess the performance of the EULAR/ACR idiopathic inflammatory myopathies (IIMs) classification criteria in a cohort of incident IIM cases and examine how criteria-assigned IIM subtype correlates with expert opinion.

Methods: Adults with newly diagnosed IIM attending Salford Royal NHS Foundation Trust were identified over a 10 year period. A retrospective review of all putative cases was performed and those fulfilling a consensus expert opinion diagnosis of IIM were included.

Drug-Induced Interstitial Lung Disease: A Systematic Review.

Background: Drug-induced interstitial lung disease (DIILD) occurs as a result of numerous agents, but the risk often only becomes apparent after the marketing authorisation of such agents.

Methods: In this PRISMA-compliant systematic review, we aimed to evaluate and synthesise the current literature on DIILD.

Results: Following a quality assessment, 156 full-text papers describing more than 6000 DIILD cases were included in the review.

Recent developments in classification criteria and diagnosis guidelines for idiopathic inflammatory myopathies.

Purpose Of Review: The aim of this review was to summarize key developments in classification and diagnosis of the idiopathic inflammatory myopathies (IIMs).

Recent Findings: The recently published European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria for the IIMs provide a comprehensive, accurate and data-driven approach to identification of IIM cases appropriate for inclusion in research studies. Further, recent studies have advanced understanding of clinical manifestations of the IIMs and delineated the role of imaging, particularly magnetic resonance.

Genetic background may contribute to the latitude-dependent prevalence of dermatomyositis and anti-TIF1-γ autoantibodies in adult patients with myositis.

Background: The prevalence of dermatomyositis (DM) versus DM and polymyositis (PM) combined has been shown to be negatively associated with latitude. This observation has been attributed to increasing exposure to ultraviolet (UV) light towards the equator. In this study, we investigated whether differing genetic background in populations could contribute to this distribution of DM.

Long-term persistence with rituximab in patients with rheumatoid arthritis.

Objectives: To investigate the long term persistence of rituximab (RTX) in a large observational RA cohort, investigate persistence of RTX when used as a first or second line biologic DMARD (bDMARD), to characterize subsequent bDMARD treatment following RTX.

Methods: Patients with RA starting treatment with RTX (MabThera) between 2008 and 2011 were recruited into the British Society for Rheumatology Biologics Register for RA. Duration of RTX treatment over the first 4 years after initiation was estimated via Kaplan-Meier estimates and the reason for discontinuation was ascertained.

Idiopathic inflammatory myopathies - a guide to subtypes, diagnostic approach and treatment.

The idiopathic inflammatory myopathies are a group of conditions characterised by inflammation of muscles (myositis) and other body systems. The diagnosis can be challenging because of the many potential clinical features and extra-muscular manifestations, which may be seemingly unrelated. An accurate diagnosis requires up-to-date understanding of the clinical manifestations, different clinical subtypes and appropriate interpretation of investigations, including newly described serological subtypes.

Frailty and bone health in European men.

Background: frailty is associated with an increased risk of fragility fractures. Less is known, however, about the association between frailty and bone health.

Methods: men aged 40-79 years were recruited from population registers in eight European centres for participation in the European Male Aging Study.