Publications by authors named "Jonathan S Hausmann"

Objective: Approximately one third of individuals worldwide have not received a COVID-19 vaccine. Although studies have investigated risk factors linked to severe COVID-19 among unvaccinated people with rheumatic diseases (RDs), we know less about whether these factors changed as the pandemic progressed. We aimed to identify risk factors associated with severe COVID-19 in unvaccinated individuals in different pandemic epochs corresponding to major variants of concern.

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Purpose Of Review: Although the concept of systemic autoinflammatory diseases (SAIDs) is still very young, our knowledge about them is exponentially growing. In the current review, we aim to discuss novel SAIDs and autoinflammatory pathways discovered in the last couple of years.

Recent Findings: Advances in immunology and genetics have led to the discovery of new pathways involved in autoinflammation, as well as several new SAIDs, including retinal dystrophy, optic nerve edema, splenomegaly, anhidrosis, and migraine headache (ROSAH syndrome), vacuoles, E1 enzyme, X-linked autoinflammatory somatic (VEXAS) syndrome, TBK1 deficiency, NEMO deleted exon 5 autoinflammatory syndrome (NDAS), and disabling pansclerotic morphea.

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Objective: Although interleukin-1 (IL-1)/IL-6 inhibitors are effective therapies for systemic juvenile idiopathic arthritis (JIA), some patients develop eosinophilia and lung disease during treatment. This study was undertaken to retrospectively evaluate incidence and risk factors for eosinophilia and describe lung disease outcomes in IL-1/IL-6 inhibitor-exposed patients with systemic JIA.

Methods: Among JIA patients at our institution exposed to interleukin-1 (IL-1)/IL-6 inhibitors (1995-2022), we compared incidence rate of eosinophilia in systemic JIA compared to other JIA, stratified by medication class (IL-1/IL-6 inhibitors, other cytokine inhibitors, methotrexate).

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Objective: To determine the association between race/ethnicity and COVID-19 outcomes in individuals with systemic lupus erythematosus (SLE).

Methods: Individuals with SLE from the US with data entered into the COVID-19 Global Rheumatology Alliance registry between March 24, 2020 and August 27, 2021 were included. Variables included age, sex, race, and ethnicity (White, Black, Hispanic, other), comorbidities, disease activity, pandemic time period, glucocorticoid dose, antimalarials, and immunosuppressive drug use.

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Objective: We investigated prolonged COVID-19 symptom duration, defined as lasting 28 days or longer, among people with systemic autoimmune rheumatic diseases (SARDs).

Methods: We analysed data from the COVID-19 Global Rheumatology Alliance Vaccine Survey (2 April 2021-15 October 2021) to identify people with SARDs reporting test-confirmed COVID-19. Participants reported COVID-19 severity and symptom duration, sociodemographics and clinical characteristics.

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Article Synopsis
  • The study aimed to identify factors that make individuals with idiopathic inflammatory myopathy (IIM) more likely to experience severe outcomes from COVID-19.
  • Data from the COVID-19 Global Rheumatology Alliance showed that factors such as older age, high disease activity, multiple comorbidities, and specific medication usage were linked to increased severity of the illness.
  • The findings highlight the need for further research on how these characteristics impact COVID-19 outcomes in people with IIM, being the first registry data of its kind.
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Background: Differences in the distribution of individual-level clinical risk factors across regions do not fully explain the observed global disparities in COVID-19 outcomes. We aimed to investigate the associations between environmental and societal factors and country-level variations in mortality attributed to COVID-19 among people with rheumatic disease globally.

Methods: In this observational study, we derived individual-level data on adults (aged 18-99 years) with rheumatic disease and a confirmed status of their highest COVID-19 severity level from the COVID-19 Global Rheumatology Alliance (GRA) registry, collected between March 12, 2020, and Aug 27, 2021.

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Objective: Some patients with rheumatic diseases might be at higher risk for coronavirus disease 2019 (COVID-19) acute respiratory distress syndrome (ARDS). We aimed to develop a prediction model for COVID-19 ARDS in this population and to create a simple risk score calculator for use in clinical settings.

Methods: Data were derived from the COVID-19 Global Rheumatology Alliance Registry from March 24, 2020, to May 12, 2021.

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Objective: To compare clinical outcomes in children with hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) who were managed before and after implementation of an evidence-based guideline (EBG).

Methods: A management algorithm for MAS-HLH was developed at our institution based on literature review, expert opinion, and consensus building across multiple pediatric subspecialties. An electronic medical record search retrospectively identified hospitalized patients with MAS-HLH in the pre-EBG (October 15, 2015, to December 4, 2017) and post-EBG (January 1, 2018, to January 21, 2020) time periods.

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Article Synopsis
  • IL-1 mediated systemic autoinflammatory diseases like CAPS, TRAPS, MKD, and DIRA primarily affect young children, showing various organ involvement, and require effective treatment strategies due to their severe nature and poor prognosis if untreated.* -
  • A multidisciplinary task force was created to establish evidence-based guidelines for diagnosing and managing these diseases, using systematic reviews and expert consensus methods.* -
  • The task force created comprehensive recommendations, including 5 guiding principles, 14 statements on diagnosis, 10 on treatment, and 9 on long-term monitoring, aiming to improve patient outcomes through standardized care.*
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Objective: Patients with undefined systemic autoinflammatory diseases (uSAIDs) are challenging to manage, as there are no guidelines or recommendations for targeted therapy. We aimed to evaluate the efficacy of empiric treatment with colchicine in our single-center uSAID population in the United States, as well as the patient characteristics associated with the most robust colchicine response.

Methods: Children with uSAID 18 years old at initial evaluation during 2000-2019 were included if they received 3 months of colchicine therapy.

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Objectives: The experiences of children with pediatric rheumatic diseases (PRD) during the initial phase of the COVID-19 pandemic have not been well-documented. We sought to determine the effects of the COVID-19 pandemic on protective behaviors, healthcare access, medication management, and education among an international cross-sectional parental survey of children with PRDs.

Methods: The COVID-19 Global Rheumatology Alliance Patient Experience Survey was distributed online, and parents of children with parental-reported PRD, with or without COVID-19 infection, were eligible to enroll.

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Social distancing during the COVID-19 pandemic has led to unprecedented challenges in medical education, including for rheumatology training programs. Many programs have adapted by transitioning educational curricula into virtual classrooms. Herein, we review strategies to optimize learning within the virtual classroom.

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Objective: We aimed to determine the feasibility and efficacy of online strategies to recruit parents of children with pediatric rheumatic diseases (PRDs) for research and to evaluate the degree to which known features of various rheumatic disease groups were present in the online cohort.

Methods: We studied two cohorts; the first was composed of respondents from a cross-sectional parental survey of children with PRDs contacted through patient support groups and social media platforms, and the second cohort was composed of participants from the Childhood Arthritis and Rheumatology Research Alliance (CARRA) legacy clinical registry.

Results: In the social media cohort, 712 complete surveys were analyzed.

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The advent of social media has revolutionized and empowered communities of people living with rare diseases. Social media has enabled families of children with similar pediatric rheumatic diseases (PRDs) to meet regardless of geographic distance, allowing them to support each other and join together to advocate on behalf of their children. Researchers have also leveraged social media to learn about the health of patients and their communities.

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Background: Patients with primary systemic vasculitis or polymyalgia rheumatica might be at a high risk for poor COVID-19 outcomes due to the treatments used, the potential organ damage cause by primary systemic vasculitis, and the demographic factors associated with these conditions. We therefore aimed to investigate factors associated with COVID-19 outcomes in patients with primary systemic vasculitis or polymyalgia rheumatica.

Methods: In this retrospective cohort study, adult patients (aged ≥18 years) diagnosed with COVID-19 between March 12, 2020, and April 12, 2021, who had a history of primary systemic vasculitis (antineutrophil cytoplasmic antibody [ANCA]-associated vasculitis, giant cell arteritis, Behçet's syndrome, or other vasculitis) or polymyalgia rheumatica, and were reported to the COVID-19 Global Rheumatology Alliance registry were included.

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Article Synopsis
  • The study aims to investigate how TNF inhibitor treatment impacts the risk of hospitalization or death from COVID-19 in patients with immune-mediated inflammatory diseases (IMIDs).
  • It analyzes data collected from three international COVID-19 registries involving adults with conditions like inflammatory arthritis, inflammatory bowel disease, and psoriasis between March 2020 and February 2021.
  • The findings will contribute to understanding the safety and risks of TNF inhibitors during the pandemic compared to other immunomodulatory treatments.
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Objective: Active learning opportunities within graduate medical education may be underused. We aimed to assess whether active learning strategies increase after implementing a faculty development workshop and transitioning rheumatology fellowship didactics to a virtual flipped classroom.

Methods: We measured baseline faculty use of active learning strategies during lectures within the Introductory Rheumatology Curriculum by calculating an "active learning score" from a cognitive learning theory assessment tool.

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Background: We describe the early experiences of adults with systemic rheumatic disease who received the COVID-19 vaccine.

Methods: From 2 April to 30 April 2021, we conducted an online, international survey of adults with systemic rheumatic disease who received COVID-19 vaccination. We collected patient-reported data on clinician communication, beliefs and intent about discontinuing disease-modifying antirheumatic drugs (DMARDs) around the time of vaccination, and patient-reported adverse events after vaccination.

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Objective: To describe coronavirus disease 2019 (COVID-19) and pregnancy outcomes in patients with rheumatic disease who were pregnant at the time of infection.

Methods: Since March 2020, the COVID-19 Global Rheumatology Alliance has collected cases of patients with rheumatic disease with COVID-19. We report details of pregnant women at the time of COVID-19 infection, including obstetric details separately ascertained from providers.

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Article Synopsis
  • Oligoarticular juvenile idiopathic arthritis (oligo JIA) is the most common chronic inflammatory arthritis in kids, with an unclear cause, prompting research into immune responses.
  • Studies using advanced techniques like flow cytometry and RNA sequencing showed a strong Th1 polarization in T cells within synovial fluid, linked to more severe disease symptoms.
  • Despite being Th1-skewed, Tregs (regulatory T cells) preserved their function and identity, suggesting that treatments targeting Th1 inflammation and boosting Treg activity could help manage oligo JIA.
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Background: The impact and consequences of the COVID-19 pandemic on people with rheumatic disease are unclear. We developed the COVID-19 Global Rheumatology Alliance Patient Experience Survey to assess the effects of the COVID-19 pandemic on people with rheumatic disease worldwide.

Methods: Survey questions were developed by key stakeholder groups and disseminated worldwide through social media, websites, and patient support organisations.

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Objectives: The severity of familial Mediterranean fever (FMF) may vary in different areas, suggesting a role for environmental factors. We analysed the composition of gut microbiota among children with FMF and healthy controls from Turkey and the USA and determined its effect on disease severity.

Methods: Children with FMF with pathogenic MEFV mutations and healthy controls from Turkey and the USA were enrolled.

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