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| http://dx.doi.org/10.1016/j.yrmex.2019.100004 | DOI Listing |
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Granulomatous Tubulointerstitial Nephritis in a Kidney Allograft: Treatment with Interleukin-6 Receptor Antagonist Stabilises Kidney Function.
J Clin Med
June 2024
Department of Transplantation, Renal and Urology, Guy's and St Thomas' NHS Foundation Trust, London SE1 9RT, UK.
Granulomatous tubulointerstitial nephritis (GTIN) attributed to early onset sarcoidosis is an ultrarare finding in an allograft kidney biopsy. We present the case of a young man with allograft dysfunction who had GTIN upon biopsy. We performed a thorough case review based on recovered records from early childhood and reassessed genetic testing results.
View Article and Find Full Text PDFInterleukin 6: at the interface of human health and disease.
Front Immunol
October 2023
Feinberg School of Medicine, Department of Neurology, Northwestern University, Chicago, IL, United States.
Interleukin 6 (IL-6) is a pleiotropic cytokine executing a diverse number of functions, ranging from its effects on acute phase reactant pathways, B and T lymphocytes, blood brain barrier permeability, synovial inflammation, hematopoiesis, and embryonic development. This cytokine empowers the transition between innate and adaptive immune responses and helps recruit macrophages and lymphocytes to the sites of injury or infection. Given that IL-6 is involved both in the immune homeostasis and pathogenesis of several autoimmune diseases, research into therapeutic modulation of IL-6 axis resulted in the approval of a number of effective treatments for several autoimmune disorders like neuromyelitis optica spectrum disorder (NMOSD), rheumatoid arthritis, juvenile idiopathic arthritis, polyarticular juvenile idiopathic arthritis, giant cell arteritis (GCA), and cytokine release syndrome, associated with SARS-CoV2 pneumonia.
View Article and Find Full Text PDFDesign of a phase III, double-blind, randomised, placebo-controlled trial of BI 1015550 in patients with progressive pulmonary fibrosis (FIBRONEER-ILD).
BMJ Open Respir Res
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Department of Medicine, Cornell University, New York City, New York, USA.
Introduction: Progressive pulmonary fibrosis (PPF) includes any diagnosis of progressive fibrotic interstitial lung disease (ILD) other than idiopathic pulmonary fibrosis (IPF). However, disease progression appears comparable between PPF and IPF, suggesting a similar underlying pathology relating to pulmonary fibrosis. Following positive results in a phase II study in IPF, this phase III study will investigate the efficacy and safety of BI 1015550 in patients with PPF (FIBRONEER-ILD).
View Article and Find Full Text PDFDisseminated Cryptococcosis in an Immunocompromised Patient with Altered Mental Status and a Lung Nodule.
J Community Hosp Intern Med Perspect
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Division of Pulmonary and Critical Care Medicine, University of Maryland School of Medicine, 110 South Paca Street, Baltimore, MD, 21201, USA.
Introduction: Disseminated cryptococcosis is an opportunistic infection that commonly affects the central nervous and respiratory systems and is often fatal in immunocompromised host patients. Diagnosing disseminated cryptococcosis is challenging at times due to the nonspecific presentation, resulting in delayed treatment and increased mortality.
Case Presentation: A 48-year-old man presented with altered mental status and shortness of breath requiring intubation.
A case of VEXAS syndrome (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) with decreased oxidative stress levels after oral prednisone and tocilizumab treatment.
Front Med (Lausanne)
December 2022
Department of Dermatology, Gifu University Graduate School of Medicine, Gifu, Japan.
VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome has recently been described as an autoinflammatory disease associated with severe adult-onset inflammatory manifestations. The various clinical manifestations include recurrent high-grade fever, neutrophilic dermatoses, cutaneous vasculitis, chondritis of the ear and nose, pulmonary infiltrates, cytopenia, uveitis, gastrointestinal pain or inflammation, aortitis, hepatosplenomegaly, and hematological disorders. VEXAS syndrome is caused by somatic mutations of the ubiquitin-like modifier activating enzyme 1 () gene in myeloid-lineage cells.
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