Publications by authors named "Floranne Ernste"

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.

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Background 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.

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Background/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.

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Objectives: 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.

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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.

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Article Synopsis
  • A clinical trial was conducted to evaluate the effectiveness and safety of tocilizumab in adults with refractory dermatomyositis (DM) and polymyositis (PM), involving 36 participants over 24 weeks.
  • Participants were randomly assigned to receive either tocilizumab or a placebo and were assessed based on specific disease activity measures and improvements in symptoms.
  • The results showed no significant differences in symptom improvement or disease activity between the tocilizumab and placebo groups, indicating that while tocilizumab was safe to use, it was not effective for treating DM or PM in this study.
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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.

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  • The study investigates if specific histopathological features in skin biopsies can predict systemic associations in patients with dermatomyositis (DM).
  • It finds that while vacuolar interface dermatitis was the most common feature, no specific biopsy result reliably indicated other serious conditions like myopathy or interstitial lung disease.
  • However, a correlation was noted between dense lichenoid infiltrate and severe itching, and MDA-5 antibody positive patients showed a higher likelihood of vasculopathy.
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Article Synopsis
  • This study aimed to find the incidence and prevalence of immune-mediated necrotizing myopathy (IMNM) among adults in Olmsted County, Minnesota, over a 20-year period.
  • Seven adult patients met the criteria for IMNM, with an incidence rate of 8.3 per million person-years during 2010-2019 and a prevalence of 1.85 per 100,000 people aged 50 and older in 2010.
  • The results indicated that IMNM is rare, with patients showing varying responses to treatment and no increased risk of cancer compared to the general population.
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Article Synopsis
  • - The study aimed to determine the demographic and clinical factors affecting the time between a psoriasis diagnosis and the development of psoriatic arthritis (PsA) in patients from Olmsted County, MN, between 2000 and 2017.
  • - Out of 164 PsA patients, 158 had a history of psoriasis, with a median duration between psoriasis and PsA of about 35.5 months; 41% had both conditions diagnosed within a year, while 59% developed psoriasis first.
  • - Key findings indicated that younger age at psoriasis onset and more severe psoriasis were linked to a longer interval before developing PsA, highlighting the progression patterns between the two conditions.
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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.

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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.

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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.

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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.

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Objectives: 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.

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Article Synopsis
  • - The study aimed to identify the incidence of psoriatic arthritis (PsA) in a US population and examine trends over the past 50 years, focusing particularly on the years 2000-2017.
  • - From 2000-2017, there were 164 new cases of PsA, with an overall age- and sex-adjusted incidence rate of 8.5 per 100,000 population, showing no significant increase compared to earlier decades, though there was a modest rise in women.
  • - The proportion of women diagnosed with PsA increased from 39% in 1970-1999 to 54% in 2010-2017, while survival rates for PsA patients were
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Article Synopsis
  • The study investigates the demographic and clinical factors contributing to the delay in diagnosing psoriatic arthritis (PsA) among adult patients in Olmsted County, Minnesota, over a span from 2000 to 2017.
  • It analyzed 164 new PsA cases, revealing a median diagnostic delay of 2.5 years from the onset of symptoms to diagnosis, with no improvement observed over time.
  • Factors like younger age at symptom onset, higher body mass index (BMI), and enthesitis were linked to longer diagnostic delays, while the presence of sebopsoriasis was associated with shorter delays.
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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.

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  • The study aimed to investigate the rate of erectile dysfunction (ED) among men with psoriatic arthritis (PsA) compared to those without it.
  • It found a baseline prevalence of 7% ED in men with PsA and 3% in the comparison group, with a slight increased risk of ED after the PsA diagnosis, although the results weren't statistically significant.
  • The study suggests that men with PsA may have a higher risk of ED, but further research is needed to confirm this and understand potential underlying factors.
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Objective: 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.

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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.

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