Results 31 to 40 of about 74,985 (254)

Vasculitis in Systemic Sclerosis [PDF]

International Journal of Rheumatology, 2010
Systemic sclerosis (SSc) is a multiorgan connective tissue disease characterized by autoantibody production and fibroproliferative stenosis of the microvasculature. The vascoluopathy associated with SSc is considered to be noninflammatory, yet frank vasculitis can complicate SSc, posing diagnostic and therapeutic challenges.
Kao, Lily, Weyand, Cornelia
openaire   +2 more sources

Noonan Syndrome Spectrum Disorders Predispose to Systemic Lupus Erythematosus: Case Report and Critical Review of the Literature

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT RASopathies are clinically overlapping neurodevelopmental syndromes resulting from germline mutations in genes involved in the rat sarcoma/mitogen‐activated protein kinases (RAS/MAPK) pathway. Historically, RASopathies have been described by clinical phenotypes, such as Noonan syndrome and Neurofibromatosis type I.
Anastasia‐Vasiliki Madenidou, Gillian I. Rice, James O'Sullivan, Ben Parker, Ian N. Bruce, Emma Burkitt‐Wright, Tracy A. Briggs   +6 more
wiley   +1 more source

Effect of Budesonide Nasal Irrigation in Patients With Chronic Rhinosinusitis With Nasal Polyps Without Prior Sinus Surgery: A Randomized, Double‐Blind, Placebo‐Controlled Study

International Forum of Allergy &Rhinology, EarlyView.
ABSTRACT Background The indication for nasal irrigation with corticosteroids after sinus surgery in patients with Chronic Rhinosinusitis with Nasal Polyps (CRSwNP) is well established, as surgery facilitates distribution throughout the sinonasal cavity.
Juliana Sant'Ana, Isabela Pontes, Caio Floriano, Renato Rios, Marlos Cortez, Marcel Miyake   +5 more
wiley   +1 more source

Establishment of a mouse model of propylthiouracil‐induced antineutrophil cytoplasmic antibody‐associated vasculitis

Animal Models and Experimental Medicine, EarlyView.
This graphical abstract summarizes our study in which we established a novel murine model of antineutrophil cytoplasmic antibody (ANCA)‐associated vasculitis. Neutrophil recruitment was induced using thioglycolate, followed by timed administration of propylthiouracil and phorbol 12‐myristate 13‐acetate to generate DNase I‐resistant neutrophil ...
Sakiko Masuda, Momo Uchizawa, Chihiro Terasaka, Takumi Abe, Tetsuro Nakamura, Yuka Nishibata, Yusho Ueda, Daigo Nakazawa, Utano Tomaru, Akihiro Ishizu   +9 more
wiley   +1 more source

American College of Rheumatology Guidance Statement for Diagnosis and Management of VEXAS Developed by the International VEXAS Working Group Expert Panel

Arthritis &Rheumatology, EarlyView.
Objective Vacuoles E1 enzyme X‐linked autoinflammatory somatic syndrome (VEXAS) is a recently identified rare genetic disorder associated with somatic mutations in the UBA1 gene. VEXAS presents with a combination of inflammatory and hematologic manifestations, leading to increased morbidity and mortality.
Arsene Mekinian, Sophie Georgin‐Lavialle, Marcela A. Ferrada, Sinisa Savic, Matthew J. Koster, Olivier Kosmider, Thibault Comont, Mael Heiblig, Juan I. Arostegui, Annmarie Bosco, Rim Bourguiba, Katherine R. Calvo, Catherine Cargo, Chiara Cattaneo, François Chasset, Henrique Coelho, Corrado Campochiaro, Francesca Crisafulli, Stephanie Ducharme‐Benard, Raquel Faria, Franco Franceschini, Micol Frassi, Emma M. Groarke, Carmelo Gurnari, Yervand Hakobyan, Yvan Jamilloux, Ciprian Jurcut, Yohei Kirino, Austin Kulasekararaj, Hiroyoshi Kunimoto, Lauren M. Madigan, Heřman F. Mann, Chiara Marvisi, Marcin Milchert, Sara Morais, Katja Sockel, Francesco Muratore, Hideaki Nakajima, Mrinal M. Patnaik, Luísa Regadas, Marie Robin, Abraham Rutgers, Carlo Salvarani, Anthony M. Sammel, Joerg Seebach, Pierre Sujobert, Alessandro Tomelleri, Geoffrey Urbanski, Frédéric Vandergheynst, Romana Vieira, David S. Viswanatha, Ewa Więsik‐Szewczyk, Elisa Diral, Benjamin Terrier, Bhavisha A. Patel, Pierre Fenaux, Peter C. Grayson, David B. Beck, on behalf of the International VEXAS working group, and with endorsement of EuroBloodNet, the European Reference Network in Rare Hematological Diseases, Heřman Mann, Benjamin Terrier, François Chasset, Sophie Georgin Lavialle, Alessandro Tomelleri, Campochiaro Corrado, Carlo Salvarani, Francesca Crisafulli, Franco Franceschini, Micol Frassi, Yohei Kirino, Ewa Więsik‐Szewczyk, Marcin Milchert, Raquel Faria, Ciprian Jurcut, Joerg Seebach, Sinisa Savic, David Beck, Lauren Madigan, Matthew Koster, Patnaik Mrinal, Olivier Kosmider, Pierre Sujobert, Juan I. Arostegui, Catherine Cargo, David Viswanatha, Yervand Hagopian, Mael Heilblig, Pierre Fenaux, Thibault Comont, Bruno Alessandro, Chiara Cattaneo, Elisa Diral, Sara Morais, Austin Kulasekarara, Emma Groarke, Katherine Calvo, Patel Bhavisha, Anthony Sammel, Arsene Mekinian, Benjamin Terrier, Marie Robin, Sophie Georgin Lavialle, Katja Sockel, Yvan Jamilloux, Carmelo Gurnari, Henrique Coelho, Romana Vieira, Rim Bourguiba, Marcela Ferrada, Peter Grayson   +111 more
wiley   +1 more source

The Effect of Benralizumab and Mepolizumab on Use of Oral Glucocorticoids in Patients With Eosinophilic Granulomatosis With Polyangiitis

Arthritis &Rheumatology, EarlyView.
Objective The phase 3 MANDARA study demonstrated noninferiority of benralizumab versus mepolizumab for remission in patients with eosinophilic granulomatosis with polyangiitis (EGPA). More benralizumab‐treated patients achieved complete withdrawal of oral glucocorticoids (OGCs).
Parameswaran K. Nair, Bernhard Hellmich, Arnaud Bourdin, David R. W. Jayne, Florence Roufosse, Nader Khalidi, Lena Börjesson Sjö, Ying Fan, Christopher McCrae, Sofia Necander, Eva Rodríguez‐Suárez, Anat Shavit, Claire Walton, Peter A. Merkel, Michael E. Wechsler   +14 more
wiley   +1 more source

Sjögren Disease—B Cells at the Brink: From Autoimmunity to Lymphomagenesis and the Rise of Novel B Cell–Targeted Therapies

Arthritis &Rheumatology, EarlyView.
Sjögren disease (SjD) is a common systemic autoimmune disorder characterized by inflammation of the exocrine glands, resulting in dryness. Patients frequently exhibit extraglandular manifestations affecting various organ systems. To date, there are no US Food and Drug Administration (FDA)‐approved disease‐modifying therapies for SjD. In this review, we
Rachael A. Gordon, Sara S. McCoy
wiley   +1 more source

Peripheral neuropathy in systemic vasculitis and other autoimmune diseases – a report of five cases emphasizing the importance of etiologic characterization

eNeurologicalSci, 2020
Introduction: Peripheral neuropathies may present in the context of systemic vasculitis and other autoimmune diseases. The etiologic characterization is crucial to define the treatment and prognosis in secondary vasculitis.
Rita Rodrigues, Mariana Branco, Renata Silva, Luís Ruano, Luís Fontão, Marta Lopes, Horácio Scigliano, Ricardo Taipa, Manuel Melo Pires, Catarina Santos   +9 more
doaj   +1 more source

Interleukin‐5, Eosinophils, and Interleukin‐5 Pathway Inhibitors in Eosinophilic Granulomatosis With Polyangiitis

Arthritis &Rheumatology, EarlyView.
Interlukin‐5 (IL‐5) plays a crucial role in the pathogenesis of eosinophilic granulomatosis with polyangiitis (EGPA) by promoting eosinophil differentiation, activation, and survival. We present here a typical case of EGPA in which treatment with IL‐5 pathway inhibitors is prescribed, showing to be beneficial for the patient.
Alvise Berti, Christian Pagnoux
wiley   +1 more source

Urticarial vasculitis coexisting with primary myelofibrosis – a case report

Przegląd Dermatologiczny, 2017
Introduction . Urticarial vasculitis is a subtype of cutaneous leukocytoclastic vasculitis. The disease often accompanies systemic disorders, including hematologic malignancies, connective tissue diseases, and infectious diseases, or may be drug-induced.
Radomir Reszke, Adam Reich
doaj   +1 more source