Myositis International Health and Research Collaborative Alliance (MIHRA) is a newly formed purpose-built non-profit charitable research organization dedicated to accelerating international clinical trial readiness, global professional and lay education, career development and rare disease advocacy in IIM-related disorders. In its long form, the name expresses the community's scope of engagement and intent. In its abbreviation, MIHRA, conveys linguistic roots across many languages, that reflects the IIM community's spirit with meanings such as kindness, community, goodness, and peace.
View Article and Find Full Text PDFBackground And Purpose: Elevation of serum creatine kinase (CK) or hyperCKemia is considered a biological marker of myopathies. However, selective elevation of serum aldolase with normal CK has been reported in a few myopathies, including dermatomyositis, immune-mediated myopathy with perimysial pathology and fasciitis with associated myopathy. The aim was to investigate the disease spectrum of myopathies with isolated aldolase elevation.
View Article and Find Full Text PDFBackground/objective: A subset of patients with idiopathic inflammatory myopathy (IIM) develops highly fatal, rapidly progressive interstitial lung disease (RP-ILD). Treatment strategies consist of glucocorticoid and adjunctive immunosuppressive therapies. Plasma exchange (PE) is an alternative therapy, but its benefit is unclear.
View Article and Find Full Text PDFObjectives: Symptomatic myopathy in sarcoidosis patients is not always due to sarcoid myopathy (ScM). We investigated the clinical and pathological spectrum including myxovirus resistance protein A (MxA) expression among sarcoidosis patients.
Methods: We reviewed the Mayo Clinic database (May 1980-December 2020) to identify sarcoidosis patients with myopathic symptoms and pathological evidence of myopathy.
Background: Intravenous immune globulin (IVIG) for the treatment of dermatomyositis has not been extensively evaluated.
Methods: We conducted a randomized, placebo-controlled trial involving patients with active dermatomyositis. The patients were assigned in a 1:1 ratio to receive IVIG at a dose of 2.
Objectives: To investigate immune-mediated necrotizing myopathy (IMNM) association with cancer and its clinical implications.
Methods: IMNM cases were identified 1 January 2000 to 31 December 2020 matching sex and age controls (4:1).
Results: A total of 152 patients with IMNM were identified and among serologically tested, 60% (83/140) were HMGCR-IgG+, 14% (20/140) were SRP-IgG+ and 26% (37/140) were seronegative.
Background: We performed expression quantitative trait locus (eQTL) analysis in single classical (CL) and non-classical (NCL) monocytes from patients with systemic lupus erythematosus (SLE) to quantify the impact of well-established genetic risk alleles on transcription at single-cell resolution.
Methods: Single-cell gene expression was quantified using qPCR in purified monocyte subpopulations (CD14CD16 CL and CD14CD16 NCL) from SLE patients. Novel analysis methods were used to control for the within-person correlations observed, and eQTLs were compared between cell types and risk alleles.
Arthritis Care Res (Hoboken)
February 2023
Objective: We aimed to determine the population-based incidence, prevalence, and mortality of dermatomyositis (DM) using European Alliance of Associations for Rheumatology (EULAR)/American College of Rheumatology (ACR) criteria.
Methods: This population-based cohort study included incident DM from January 1, 1995 to December 31, 2019. We manually reviewed all individuals with at least 1 code for DM or polymyositis to determine if they met EULAR/ACR criteria, subspecialty physician diagnosis, and/or Bohan and Peter criteria.
Objective: To evaluate survival and associated comorbidities in inclusion body myositis (IBM) in a population-based, case-control study.
Methods: We utilized the expanded Rochester Epidemiology Project medical records-linkage system, including 27 counties in Minnesota and Wisconsin, to identify patients with IBM, other inflammatory myopathies (IIM), and age/sex-matched population-controls. We compared the frequency of various comorbidities and survival among groups.
We report a case of smoldering multiple myeloma patient who developed signs and symptoms consistent with polyarthritis. A PET-CT demonstrated marked FDG activity in multiple joints, concerning for inflammatory arthritis. Arthrocentesis from the glenohumeral joint was consistent with inflammatory synovial fluid with no evidence for infection or crystals.
View Article and Find Full Text PDFObjectives: To determine the prevalence and natural history of sporadic inclusion body myositis (sIBM) and to test the hypothesis that patients with sIBM have higher cancer or mortality rates than the general population.
Methods: We sought patients with sIBM defined by the 2011 European Neuromuscular Centre (ENMC) diagnostic criteria among Olmsted County, Minnesota, residents in 40-year time period.
Results: We identified 20 patients (10 clinicopathologically defined, 9 clinically defined, and 1 probable) according to the ENMC criteria and 1 patient with all features of clinicopathologically defined sIBM except for symptom onset at <45 years of age.
Background: Anti-cytosolic 5'-nucleotidase 1A (cN1A) antibodies are commonly detected in patients with sporadic inclusion body myositis (sIBM). However, their pathogenic role has not been established. Moreover, efforts toward identifying sIBM distinct clinicopathologic characteristics associated with these antibodies have yielded conflicting results.
View Article and Find Full Text PDFObjective: Patients with dermatomyositis (DM) and polymyositis (PM) have reduced muscle endurance.The aim of this study was to streamline the Functional Index-2 (FI-2) by developing the Functional Index-3 (FI-3) and to evaluate its measurement properties, content and construct validity, and intra- and interrater reliability.
Methods: A dataset of the previously performed and validated FI-2 (n = 63) was analyzed for internal redundancy, floor, and ceiling effects.
Objective: Type I interferon (IFN) is important to systemic lupus erythematosus (SLE) pathogenesis, but it is not clear how chronic elevations in IFN alter immune function. We compared cytokine responses after whole blood stimulation with Toll-like receptor (TLR) agonists in high- and low-IFN SLE patient subgroups.
Methods: SLE patients and nonautoimmune controls were recruited, and SLE patients were categorized as either high or low IFN.