A5013000204- Vasculitis and related conditions
- Otitis Media and Relapsing Polychondritis
- Histiocytic Disorders and Treatments
- Renal Diseases and Glomerulopathies
- Systemic Lupus Erythematosus Research
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- Urticaria and Related Conditions
- Vascular Anomalies and Treatments
- Eosinophilic Disorders and Syndromes
- Sarcoidosis and Beryllium Toxicity Research
- IgG4-Related and Inflammatory Diseases
- Tumors and Oncological Cases
- Parvovirus B19 Infection Studies
- Mast cells and histamine
- Atherosclerosis and Cardiovascular Diseases
- Cell Adhesion Molecules Research
- Genomic variations and chromosomal abnormalities
- Myeloproliferative Neoplasms: Diagnosis and Treatment
- Extracellular vesicles in disease
- Genomics and Rare Diseases
- Autoimmune Bullous Skin Diseases
- Kawasaki Disease and Coronary Complications
- Immunodeficiency and Autoimmune Disorders
- Peripheral Neuropathies and Disorders
- Amyloidosis: Diagnosis, Treatment, Outcomes
Mayo Clinic in Arizona
2016-2025
Mayo Clinic
2016-2025
WinnMed
2015-2025
Jacksonville College
2024
Mayo Clinic in Florida
2017-2024
Massachusetts General Hospital
2022
Harvard University
2022
Center for Rheumatology
2015-2022
Franklin Pierce University
2021
Goodyear (United States)
2021
Objective. To evaluate characteristics of relapse, relapse rates, treatment and outcomes among patients with biopsy-proven GCA in a large, single-institution cohort. Methods. We conducted retrospective review all from 1998 to 2013. Demographic, clinical, laboratory data at presentation during follow-up were collected. Comparisons by rate performed using chi-square tests. Prednisone discontinuation initial oral dose ≤40 >40 mg/day was compared Cox models. Results. The cohort included 286 [74%...
Preclinical vascular inflammation models have demonstrated effective suppression of arterial wall lesional T cells through inhibition Janus kinase 3 and JAK1. However, JAK in patients with giant cell arteritis (GCA) has not been prospectively investigated.
To develop an Outcome Measures in Rheumatology (OMERACT) ultrasonography score for monitoring disease activity giant cell arteritis (GCA) and evaluate its metric properties.The OMERACT Instrument Selection Algorithm was followed. Forty-nine members of the large vessel vasculitis working group were invited to seven Delphi rounds. An online reliability exercise conducted using images bilateral common temporal arteries, parietal frontal branches as well axillary arteries from 16 patients with...
VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is an autoinflammatory disease with frequent cutaneous manifestations.We conducted a retrospective study of all patients genetically confirmed seen at our institution. Available clinical photographs and skin biopsy slides were reviewed.Cutaneous manifestations developed in 22/25 (88%) syndrome. From this group, 10/22 (45%) involvement before or the time other features VEXAS. Twenty distinct dermatologic presentations...
Objective. To test the reliability of Outcome Measures in Rheumatology Clinical Trials (OMERACT) consensus-based ultrasound definitions for normal and vasculitic temporal axillary arteries patients with giant cell arteritis (GCA) controls. Methods. A preliminary 1-day meeting a full 3-day fulfilling OMERACT Ultrasound Group guidelines were held. Temporal examined at 2 timepoints by 12 sonographers on 4 GCA The aim was to inter- intrareader findings, halo sign, compression sign. In both...
Rosai-Dorfman disease (RDD) is a rare histiocytic neoplasm with recent studies showing alterations in the MAPK pathway, most commonly KRAS and MEK genes about 40% of patients. Reports on use MEK-inhibitor therapy RDD have been limited to small case studies. There are no approved treatments for this neoplasm, therefore patients need efficacious treatments.To study outcomes after treatment cobimetinib based pathway RDD.This retrospective cohort conducted at 2 tertiary care centers included who...
Objective To evaluate the incidence and outcomes of large artery (LA) involvement among patients with giant cell arteritis (GCA) to compare LA non-GCA patients. Methods The study included Olmsted County, Minnesota, USA residents incident GCA between 1950 2016 follow-up through 31 December 2020, death or migration. A population-based age-matched/sex-matched comparator cohort without was assembled. aortic aneurysm, dissection, stenosis in aorta its main branches diagnosed within 1 year prior...
Objective Avacopan, an activated complement factor 5 receptor antagonist, has been approved as adjunct therapy for severe active antineutrophil cytoplasmic antibody–associated vasculitis (AAV). Current evidence supports the management of AAV presenting with diffuse alveolar hemorrhage (DAH) by administering glucocorticoids combined either rituximab or cyclophosphamide in addition to supportive care. The role avacopan patients DAH a primary disease manifestation not well established....
Objective. To determine the effect of methotrexate (MTX) on relapse risk and glucocorticoid (GC) use in a large single-institution cohort patients with giant cell arteritis (GCA). Methods. Patients diagnosed GCA from 1998 to 2013 confirmed evidence temporal artery biopsy and/or radiographic vessel vasculitis were identified. Each patient treated adjunct MTX (case) was matched similar only GC (control). requirements events before after initiation (or corresponding index date) compared using...
Objective To evaluate the safety and efficacy of tocilizumab (TCZ) in giant cell arteritis (GCA) a large North American cohort. Methods Patients with GCA treated TCZ between January 1, 2010, May 15, 2020, were retrospectively identified. Kaplan-Meier methods used to estimate time discontinuation first relapse after discontinuation. Poisson regression models compare annualized rates before, during, use. Age- sex-adjusted risk factors associated on off development adverse events significant...
IgG4-related disease (IgG4-RD) is a systemic immune-mediated fibroinflammatory whose pathomechanisms remain poorly understood. Here, we identified gene variants in familial IgG4-RD and determined their functional consequences. All 3 affected members of the family shared transcription factor IKAROS, encoded by IKZF1, E3 ubiquitin ligase UBR4. The IKAROS variant increased binding to FYN promoter, resulting higher T cells. UBR4 prevented lysosomal degradation phosphatase CD45. In presence...
Objective Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis of medium‐sized visceral vessels. However, cutaneous arteritis (CA) and gastrointestinal (GI) are forms single‐organ having indistinguishable histopathologic findings from PAN. The aim this study was to evaluate compare the clinical characteristics, treatment, outcomes patients with PAN, CA, GI vasculitis. Methods Retrospective cohorts were assembled, consisting between 1980 2014. demographics, abstracted medical...