Jason Springer
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View article: Genetic susceptibility and validation of angiographic patterns in Takayasu arteritis
Genetic susceptibility and validation of angiographic patterns in Takayasu arteritis Open
Genetic factors are associated with distinct subsets of TAK defined by angiographic pattern of disease.
View article: A Randomized, Double‐Blind, Placebo‐Controlled Trial of Abatacept for the Treatment of Relapsing, Nonsevere Granulomatosis With Polyangiitis
A Randomized, Double‐Blind, Placebo‐Controlled Trial of Abatacept for the Treatment of Relapsing, Nonsevere Granulomatosis With Polyangiitis Open
Objective To compare the efficacy of abatacept to placebo for the treatment of relapsing, nonsevere granulomatosis with polyangiitis (GPA). Methods In this multicenter trial, eligible patients with relapsing, nonsevere GPA were randomized …
View article: The 2024 Vasculitis Foundation Quality Care Summit: Seeking Strategies to Improve Care for All Patients
The 2024 Vasculitis Foundation Quality Care Summit: Seeking Strategies to Improve Care for All Patients Open
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View article: Stroke frequency, associated factors, and clinical features in primary systemic vasculitis: a multicentric observational study
Stroke frequency, associated factors, and clinical features in primary systemic vasculitis: a multicentric observational study Open
Objectives The cerebral vessels may be affected in primary systemic vasculitis (PSV), but little is known about cerebrovascular events (CVEs) in this population. This study aimed to determine the frequency of CVEs at the time of diagnosis …
View article: VEXAS‐Defining <i>UBA1</i> Somatic Variants in 245,368 Diverse Individuals in the NIH <i>All Of Us</i> Cohort
VEXAS‐Defining <i>UBA1</i> Somatic Variants in 245,368 Diverse Individuals in the NIH <i>All Of Us</i> Cohort Open
Objective Somatic variants in UBA1 cause VEXAS, a recently described, systemic autoinflammatory disease. Research on VEXAS has largely focused on highly symptomatic patients. We sought to determine the prevalence of canonical, VEXAS‐associ…
View article: Analysis of 245,368 diverse individuals from the NIH All of Us Cohort identifies incomplete penetrance of the VEXAS-defining UBA1 p.M41L somatic mutation
Analysis of 245,368 diverse individuals from the NIH All of Us Cohort identifies incomplete penetrance of the VEXAS-defining UBA1 p.M41L somatic mutation Open
Objective Somatic mutations in UBA1 cause the recently described systemic auto-inflammatory syndrome, VEXAS. Study of this disease has largely been limited to highly symptomatic patients. We sought to determine the prevalence of VEXAS-asso…
View article: Self-Reported Data and Physician-Reported Data in Patients With Eosinophilic Granulomatosis With Polyangiitis: Comparative Analysis
Self-Reported Data and Physician-Reported Data in Patients With Eosinophilic Granulomatosis With Polyangiitis: Comparative Analysis Open
Background Patient-based registries can help advance research on rare diseases such as eosinophilic granulomatosis with polyangiitis (EGPA), a complex multiorgan form of antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis. Obj…
View article: 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Kawasaki Disease
2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Kawasaki Disease Open
Objective To provide evidence‐based recommendations and expert guidance for the management of Kawasaki disease (KD), focusing on clinical scenarios more commonly addressed by rheumatologists. Methods Sixteen clinical questions regarding di…
View article: 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Kawasaki Disease
2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Kawasaki Disease Open
Objective To provide evidence‐based recommendations and expert guidance for the management of Kawasaki disease (KD), focusing on clinical scenarios more commonly addressed by rheumatologists. Methods Sixteen clinical questions regarding di…
View article: Dose-dependent Pharmacological Response to Rituximab in the Treatment of Antineutrophil Cytoplasmic Antibody-associated Vasculitis
Dose-dependent Pharmacological Response to Rituximab in the Treatment of Antineutrophil Cytoplasmic Antibody-associated Vasculitis Open
Objective Rituximab (RTX) is effective in the induction and maintenance of remission in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). However, uncertainty remains regarding the optimal maintenance dosing regimen. …
View article: Kawasaki Disease: A Systematic Review and Meta‐Analysis of Benefits and Harms of Common Treatments
Kawasaki Disease: A Systematic Review and Meta‐Analysis of Benefits and Harms of Common Treatments Open
Objective Kawasaki disease (KD) is a self‐limited vasculitis affecting medium‐sized vessels with a predilection for the coronary arteries. Although treatment reduces the likelihood of developing of coronary artery aneurysms, 5% of patients…
View article: 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Giant Cell Arteritis and Takayasu Arteritis
2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Giant Cell Arteritis and Takayasu Arteritis Open
Objective To provide evidence‐based recommendations and expert guidance for the management of giant cell arteritis (GCA) and Takayasu arteritis (TAK) as exemplars of large vessel vasculitis. Methods Clinical questions regarding diagnostic …
View article: 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody–Associated Vasculitis
2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody–Associated Vasculitis Open
Objective To provide evidence‐based recommendations and expert guidance for the management of antineutrophil cytoplasmic antibody–associated vasculitis (AAV), including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA)…
View article: 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Polyarteritis Nodosa
2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Polyarteritis Nodosa Open
Objective To provide evidence‐based recommendations and expert guidance for the management of systemic polyarteritis nodosa (PAN). Methods Twenty‐one clinical questions regarding diagnostic testing, treatment, and management were developed…
View article: Clinical Manifestations and Long‐Term Outcomes of Eosinophilic Granulomatosis With Polyangiitis in North America
Clinical Manifestations and Long‐Term Outcomes of Eosinophilic Granulomatosis With Polyangiitis in North America Open
Objective To describe clinical manifestations and outcomes in patients with eosinophilic granulomatosis with polyangiitis (EGPA) in North America. Methods Analysis of patients aged 18 years or older who fulfilled the 1990 American College …
View article: Giant Cell Arteritis: A Systematic Review and Meta‐Analysis of Test Accuracy and Benefits and Harms of Common Treatments
Giant Cell Arteritis: A Systematic Review and Meta‐Analysis of Test Accuracy and Benefits and Harms of Common Treatments Open
This systematic review compares treatment options for patients with giant cell arteritis (GCA) and evaluates the test accuracy of studies used in diagnosing and monitoring GCA. These studies were used to inform evidence‐based recommendatio…
View article: Granulomatosis With Polyangiitis and Microscopic Polyangiitis: A Systematic Review and Meta‐Analysis of Benefits and Harms of Common Treatments
Granulomatosis With Polyangiitis and Microscopic Polyangiitis: A Systematic Review and Meta‐Analysis of Benefits and Harms of Common Treatments Open
Objective The aim of this systemic review is to compare different treatments for patients with granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) to inform evidence‐based recommendations for the American College of R…
View article: Polyarteritis Nodosa: A Systematic Review of Test Accuracy and Benefits and Harms of Common Treatments
Polyarteritis Nodosa: A Systematic Review of Test Accuracy and Benefits and Harms of Common Treatments Open
Objective The object of this study was to analyze the benefits and harms of different treatment options and to analyze test accuracy used in the evaluation of patients with primary systemic polyarteritis nodosa (PAN). Methods A systematic …
View article: Eosinophilic Granulomatosis with Polyangiitis: A Systematic Review and Meta‐Analysis of Test Accuracy and Benefits and Harms of Common Treatments
Eosinophilic Granulomatosis with Polyangiitis: A Systematic Review and Meta‐Analysis of Test Accuracy and Benefits and Harms of Common Treatments Open
Objective Eosinophilic granulomatosis with polyangiitis (EGPA) is part of a group of vasculitides commonly referred to as antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV), in addition to granulomatosis with polyangiit…
View article: Takayasu Arteritis: a Systematic Review and Meta‐Analysis of Test Accuracy and Benefits and Harms of Common Treatments
Takayasu Arteritis: a Systematic Review and Meta‐Analysis of Test Accuracy and Benefits and Harms of Common Treatments Open
Objective Takayasu’s arteritis (TAK) is a granulomatous large‐vessel vasculitis primarily affecting the aorta and its proximal branches. TAK can be a difficult disease to diagnose and manage given the rarity of the disease as well as curre…
View article: Self-Reported Data and Physician-Reported Data in Patients With Eosinophilic Granulomatosis With Polyangiitis: Comparative Analysis (Preprint)
Self-Reported Data and Physician-Reported Data in Patients With Eosinophilic Granulomatosis With Polyangiitis: Comparative Analysis (Preprint) Open
BACKGROUND Patient-based registries can help advance research on rare diseases such as eosinophilic granulomatosis with polyangiitis (EGPA), a complex multiorgan form of antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis. …
View article: Defining a Therapeutic Window for Rituximab Maintenance Therapy in ANCA-Associated Vasculitis
Defining a Therapeutic Window for Rituximab Maintenance Therapy in ANCA-Associated Vasculitis Open
BACKGROUND/OBJECTIVE: Rituximab (RTX) has been shown to be effective at maintaining remission in patients with granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), however, the optimal regimen has not been established…
View article: Identification of susceptibility loci for Takayasu arteritis through a large multi-ancestral genome-wide association study
Identification of susceptibility loci for Takayasu arteritis through a large multi-ancestral genome-wide association study Open
Takayasu arteritis is a rare inflammatory disease of large arteries. We performed a genetic study in Takayasu arteritis comprising 6,670 individuals (1,226 affected individuals) from five different populations. We discovered HLA risk facto…
View article: Sequence‐Based Screening of Patients With Idiopathic Polyarteritis Nodosa, Granulomatosis With Polyangiitis, and Microscopic Polyangiitis for Deleterious Genetic Variants in <i>ADA2</i>
Sequence‐Based Screening of Patients With Idiopathic Polyarteritis Nodosa, Granulomatosis With Polyangiitis, and Microscopic Polyangiitis for Deleterious Genetic Variants in <i>ADA2</i> Open
Objective Deficiency of adenosine deaminase 2 (DADA2) is a monogenic form of vasculitis that can resemble polyarteritis nodosa (PAN). This study was undertaken to identify potential disease‐causing sequence variants in ADA2 in patients wit…
View article: Clinical Characteristics of an Internet-Based Cohort of Patient-Reported Diagnosis of Granulomatosis With Polyangiitis and Microscopic Polyangiitis: Observational Study
Clinical Characteristics of an Internet-Based Cohort of Patient-Reported Diagnosis of Granulomatosis With Polyangiitis and Microscopic Polyangiitis: Observational Study Open
Background Utilizing the traditional centers of excellence approach to conduct clinical trials involving rare diseases remains challenging. Patient-based registries have been shown to be both feasible and valid in several other diseases. O…
View article: Clinical Characteristics of an Internet-Based Cohort of Patient-Reported Diagnosis of Granulomatosis With Polyangiitis and Microscopic Polyangiitis: Observational Study (Preprint)
Clinical Characteristics of an Internet-Based Cohort of Patient-Reported Diagnosis of Granulomatosis With Polyangiitis and Microscopic Polyangiitis: Observational Study (Preprint) Open
BACKGROUND Utilizing the traditional centers of excellence approach to conduct clinical trials involving rare diseases remains challenging. Patient-based registries have been shown to be both feasible and valid in several other diseases. …
View article: Mast Cell Degranulation Decreases Lipopolysaccharide-Induced Aortic Gene Expression and Systemic Levels of Interleukin-6 <i>In Vivo</i>
Mast Cell Degranulation Decreases Lipopolysaccharide-Induced Aortic Gene Expression and Systemic Levels of Interleukin-6 <i>In Vivo</i> Open
Mast cells play an important role in immunomodulation and in the maintenance of vascular integrity. Interleukin-6 (IL-6) is one of the key biomarkers and therapeutic target in systemic vasculitis. The objective of the current study is to d…