Publications by authors named "Jason Michael Springer"
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. The objective of this study was to determine the association between RTX exposure and pharmacological response.
Methods:: Thirty patients with GPA (25) and MPA (5) receiving maintenance therapy with RTX were longitudinally followed in a single tertiary care center.
Purpose Of Review: We aim to describe the pathophysiology, clinical findings, diagnosis, and treatment of deficiency of adenosine deaminase 2 (DADA2).
Recent Findings: DADA2 is a multi-organ disease of children and less often adults, which can present with wide-ranging manifestations including strokes, medium vessel vasculitis, hematologic disease, and immunodeficiency. Diagnosis is through detection of reduced activity level of the adenosine deaminase 2 (ADA2) enzyme and/or identification of bi-allelic mutations in the ADA2 gene.
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.
Objective: This report outlines the clinical characteristics of a large internet registry cohort of participants with a self-reported diagnosis of granulomatosis with polyangiitis or microscopic polyangiitis.
Objective: Describe the clinical characteristics and histopathology findings in a family with two siblings affected with deficiency of adenosine deaminase 2 (DADA2). Both patients presented in childhood with polyarthritis and developed significant neurological and gastrointestinal features of DADA2 in ear, including variable degrees of immunologic and hematologic manifestations.
Methods: Adenosine Deaminase 2 (ADA2; also known as exon sequencing and serum ADA2 levels were performed to confirm the diagnosis of DADA2.
Background/objectives: Serum S100A8/S100A9 and S100A12 levels have been shown to be elevated in giant cell arteritis (GCA). This study aimed to determine if levels of serum S100 proteins perform as markers in a comparable fashion to standard markers of disease activity in large-vessel vasculitis.
Methods: Serum samples were obtained from the Vasculitis Clinical Research Consortium (VCRC) Longitudinal Prospective Cohort Study of GCA and Takayasu’s arteritis (TAK).