Results 41 to 50 of about 1,238,132 (184)

Complement Activation Linked to Type II Interferon Signaling in Still Disease

Arthritis &Rheumatology, EarlyView.
Objective Still disease (SD) is an autoinflammatory syndrome characterized by innate immune dysregulation. Although complement can drive inflammation, its involvement in SD remains to be defined. Thus, we aimed to assess complement activation in SD. Methods Complement was assessed using transcriptomic, proteomic, and in vitro approaches. RNA sequencing
Freya M. C. H. Huijsmans, Tabea Thalheim, Alejandra Bodelón, Greta Rogani, Lyanne J. P. M. Sijbers, Remco G. A. Erkens, Aafke de Ligt, Rianne Scholman, Aron Brinker, Gisella B. Beretta, Nienke M. Ter Haar, Thomas Vogl, Johannes Roth, Trang T. Duong, Sytze de Roock, Joost F. Swart, Deborah A. Marshall, Susanne M. Benseler, Rae S. M. Yeung, Christoph Kessel, Sebastiaan J. Vastert, Emely L. Verweyen, Jorg van Loosdregt, on behalf of the UCAN CAN‐DU/UCAN CURE Consortia and the One Child Every Child Initiative, Adam Huber, Bianca Lang, Chelsea DeCoste, Elizabeth Stringer, Suzanne Ramsey, Alan Rosenberg, Kate Neufeld, Mehul Jariwala, Tristan Kerr, Alexander Mosoiu, Alisa Rachlis, Amy Xu, Arthur Cheng, Brenleigh Jebb, Brian Feldman, Bruno Pereira, Deborah Levy, Dilan Dissanayake, Elizaveta Limenis, Evelyn Rozenblyum, Harper Cheng, Jennifer Ji Young Lee, Lynn Spiegel, Rayfel Schneider, Ronald Laxer, Ruud Verstegen, Shirley Tse, Andrea Human, David Cabral, Herman Tam, Jaime Guzman, Kim Morishita, Kristin Houghton, Lori Tucker, Mercedes Chan, Ross Petty, Tommy Gerschman, Annet van Royen‐Kerkhof, Berent Prakken, Erika Van Nieuwenhove, Marc Jansen, Nico Wulffraat, Ciarán Duffy, Nadia Luca, Roman Jurencak, Tala El Tal, Claire LeBlanc, Gaëlle Chédeville, Piya Lahiry, Rosie Scuccimarri, Sarah Campillo, Clare Hutchinson, Daniah Basodan, Dax G Rumsey, Hon Yan Ng, Jeanine McColl, Lillian Lim, Tara McGrath, Danielle Brinkman, Petra Hissink Muller, Elizabeth Legger, Wineke Armbrust, Ellen Schatorje, Esther Hoppenreijs, Elodie Boudes, Gillian Currie, Heinrike Schmeling, Muhammed Dhalla, Nicole Johnson, Paivi Miettunen, Ravneet Sran, Rebeka Stevenson, Erkan Demirkaya, Jonathan Park, Roberta Berard, Giske Biesbroek, Mariken Gruppen, Gordon Soon, Joseph Cafazzo, Liane Heale, Michelle Batthish, Tania Cellucci, Lily Lim, Maarten IJzerman, Marinka Twilt, Marleen Verkaaik, Philomine van Pelt, Sylvia Kamphuis, Michelle Kip, Nicholas Blanchette, Paul Dancey, Regina de Geus   +115 more
wiley   +1 more source

Human Monocytic Models Reveal Genotype‐Dependent Inflammatory Programs in VEXAS Syndrome

Arthritis &Rheumatology, EarlyView.
Objective VEXAS syndrome is a severe X‐linked autoinflammatory disorder caused by somatic mutations in ubiquitin‐like modifier activating enzyme 1 (UBA1), with clinical outcomes that vary by UBA1 genotype. We aimed to elucidate genotype‐specific inflammatory programs and identify potential therapeutic targets.
Kana Higashitani, Tatsuma Ban, Yohei Kirino, Go R. Sato, Soichiro Adachi, Yuki Iizuka, Kayo Harita, Ayaka Maeda, Osamu Ohara, Hideaki Nakajima, Tomohiko Tamura   +10 more
wiley   +1 more source

Clinical heterogeneity of ocular Behçet’s syndrome versus intestinal Behçet’s syndrome: a cross-sectional study from Shanghai Behçet’s syndrome database

Arthritis Research & Therapy, 2022
Background Behçet’s syndrome (BS) is a rare variant vasculitis which can involve the eyes and gastrointestinal systems. However, ocular involvement rarely overlaps with intestinal lesions.
Cheng-cheng Hou, Dan Luo, Hua-fang Bao, Jing-fen Ye, Hai-fen Ma, Yan Shen, Jun Zou, Jian-long Guan   +7 more
doaj   +1 more source

The genetic landscape of congenital diarrheas and very early onset inflammatory bowel disease in the Middle East

JPGN Reports, EarlyView.
Abstract Objectives Monogenic causes of congenital diarrheas and enteropathies (CoDE) and very early onset inflammatory bowel disease (VEOIBD) are mostly recessive and therefore more prevalent in populations with increased consanguinity rates. To assess the genetic basis of these disorders in a likely high‐prevalence population, we established a multi ...
Lily Gillette, Muna Al Safar, Ganeshwaran H. Mochida, Casey Johnson, Stephen Babcock, Klaus Schmitz‐Abe, Michael Field, Jessica Yang, Richelle Bearup, Ahmed Megahed, Mohammed Ezz El Regal Abbas, Tarek Baraket, Sherine Elziny, Ali Sobh, Gulin Eren, Fawaz AlRefaee, Rana Bitar, Mohamed Miqdady, Pankaj B. Agrawal, Scott B. Snapper, Jay R. Thiagarajah, Jodie Ouahed   +21 more
wiley   +1 more source

Factors Associated With Bilateral Involvement in Non‐Infectious Labyrinthitis: A Case–Control Study

The Laryngoscope, EarlyView.
Non‐infectious labyrinthitis may progress from unilateral to bilateral inner‐ear involvement, resulting in greater functional impairment. In this retrospective case–control study of 50 patients, younger age, female sex, and the presence of systemic disease were associated with bilateral involvement. These exploratory findings may help identify patients
Douglas Henderson, Blanca Amador Borrero, Aurélien Cadix, Karine Champion, Damien Sene, Philippe Herman, Charlotte Hautefort, Alain Londero, Michael Eliezer, Cassandre Djian   +9 more
wiley   +1 more source

Restoring immune balance with low‐dose interleukin‐2 in a patient with anti‐synthetase syndrome and recurrent infections: A case report

Rheumatology &Autoimmunity, EarlyView.
This figure summarizes the clinical course and immune reconstitution in a patient with anti‐synthetase syndrome (ASyS) and recurrent refractory infections. Left panel: conventional immunosuppression is associated with marked Treg deficiency and persistent infections. Middle panel: intervention with subcutaneous low‐dose interleukin (IL)−2. Right panel:
Tian Gao, Yuxi Wang, Xiaojing Jiang, Yang Yun, Qinyi Su, Zhe Zhao, Qi Wu, Han Zhang, Shengxiao Zhang   +8 more
wiley   +1 more source

Asymptomatic giant coronary aneurysm in an adolescent with Behcet's syndrome

Pediatric Rheumatology Online Journal, 2012
Objective Behcet's is an idiopathic multi-organ syndrome, which may have onset during childhood. Vascular involvement is uncommon, with rarely reported coronary aneurysm formation.
Kahn Philip J, Yazici Yusuf, Argilla Michael, Srichai Monvadi, Levy Deborah M   +4 more
doaj   +1 more source

Behcet’s Syndrome resembling complex perianal Crohn’s Disease

SAGE Open Medical Case Reports, 2021
Behcet’s syndrome is a systemic inflammatory disorder that involves several organ systems and is exceptionally rare in the Western world. The diagnosis is frequently difficult as it resembles several other disease processes.
Ali P Mourad, Marie Shella De Robles, Robert DR Winn   +2 more
doaj   +1 more source

Inflammation Unchecked: Concurrent Kawasaki Disease and Stevens‐Johnson Syndrome in an 18‐Month‐Old Child

Arthritis Care &Research, EarlyView.
Catherine Deffendall, Jodi Backalar, Elizabeth Acevedo Zapata, Adriana Della Porta, Lorin Bibb, Tyler Reese, Charlotte M. Brown   +6 more
wiley   +1 more source

Primary Cutaneous Gamma‐Delta T‐Cell Lymphoma Complicating Long‐Standing Immunosuppressed Dermatomyositis

Journal of Cutaneous Pathology, EarlyView.
ABSTRACT Primary cutaneous gamma‐delta T‐cell lymphoma (PCGD‐TCL) is a rare cytotoxic lymphoma with key oncogenic drivers in the JAK/STAT pathway. Also primarily involving the subcutaneous adipose tissue, subcutaneous panniculitis‐like T‐cell lymphoma (SPTCL) is more frequently encountered in scenarios of autoimmune disorders.
Bennett Christie‐Nguyen, Youn H. Kim, David F. Fiorentino, Danielle Tartar, Ryanne Brown, Robert A. Novoa, Kerri E. Rieger   +6 more
wiley   +1 more source