Results 171 to 180 of about 2,829,847 (276)

Early Differential Diagnosis of Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis and Macrophage Activation Syndrome in Children: A Clinical Prediction Model Based on 106 Patients. [PDF]

Pathogens
Pei M, Su Y, Ding J, Zhou W, Chu C.
europepmc   +1 more source

Complement activation linked to type II interferon signaling in Still disease

Arthritis &Rheumatology, Accepted Article.
Objective Still disease (SD) is an autoinflammatory syndrome characterized by innate immune dysregulation. While 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 of
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, 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

Adult-Onset Still's Disease in a Patient with Macrophage Activation Syndrome and Pre-Disseminated Intravascular Coagulation: A Case Report and Literature Review. [PDF]

J Inflamm Res
Xu J, Yang J, Hu Y, Zhao Z, Cai H, Yao Q, Shang W.   +6 more
europepmc   +1 more source

Persistent IL‐18 fuels expansion of CD38+HLA‐DR+CD8+ T cells in Still's disease and macrophage activation syndrome

Arthritis &Rheumatology, Accepted Article.
Objective Still's disease (SD) is an autoinflammatory disorder characterized by remarkably high IL‐18 levels. Increasing evidence suggests that adaptive immunity also contributes to its pathogenesis, particularly in refractory courses. Macrophage activation syndrome (MAS), one of SD's most severe complications, is associated with further IL‐18 ...
Greta Rogani, Remco G.A. Erkens, Alejandra Bodelón, Tim R. Mocking, Marein T.M. Putmans, Freya M.C.H. Huijsmans, Lyanne J.P.M. Sijbers, Aafke M. De Ligt, Maurice J.H. Van Haaren, Rianne C. Scholman, Noël M.M. Dautzenberg, Joyce I. Meesters‐Ensing, Martina Rossano, Laura Porretti, Stefan Nierkens, Francesca S. Minoia, Sebastian J. Vastert, Jorg van Loosdregt   +17 more
wiley   +1 more source

Single‐cell RNA sequencing of peripheral blood defines two immunological subtypes of Sjögren's disease distinguished by anti‐SSA antibodies and aberrant B cell populations

Arthritis &Rheumatology, Accepted Article.
Objectives Sjögren's disease (SjD) is a heterogeneous autoimmune disorder characterized by substantial clinical and molecular diversity. This heterogeneity raises key questions regarding the existence of distinct pathogenic mechanisms underlying disease subtypes.
Geoffrey Urbanski, Kimberly E. Taylor, Emily Flynn, Armita Norouzi, Catherine Chu, Brittany Davidson, Arnab Ghosh, Joanne Nititham, Ravi K. Patel, Annie W. Poon, Gabriela K. Fragiadakis, Alexis J. Combes, Walter L. Eckalbar, Lindsey A. Criswell, Chun Jimmie Ye, Caroline H. Shiboski   +15 more
wiley   +1 more source

Stormy Course of Adult-Onset Still's Disease With Macrophage Activation Syndrome and Concurrent Membranoproliferative Glomerulonephritis: A Case Report. [PDF]

Cureus
Adeyemo AW, Sharma S, Busari K, Momoh O.
europepmc   +1 more source

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

Arthritis &Rheumatology, Accepted Article.
Objectives 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

Efficacy of Rituximab in Successful Management of Refractory Juvenile Systemic Lupus Erythematosus Complicated with Macrophage Activation Syndrome: A Case Report with Literature Overview. [PDF]

Indian J Dermatol
Mishra S, Madhual S, Panda M.
europepmc   +1 more source

Navigating adverse immunostimulation: A practical guide for clinical researchers

British Journal of Clinical Pharmacology, EarlyView.
Problem Setting As drug development moves towards more complex products, early clinical development programmes are increasingly hampered by unwanted and/or unexpected activation of the immune system (adverse immune stimulation, AIS). Solution At the Centre for Human Drug Research, we have introduced standardized procedures to make AIS manageable, while
Juliette A. van den Noort, Anouk C. Meijs, Naomi B. Klarenbeek, Matthijs Moerland   +3 more
wiley   +1 more source

Severe Treatment-Refractory Macrophage Activation Syndrome: Hemophagocytic Lymphohistiocytosis Presenting With Neurologic Crisis in Systemic Lupus Erythematosus. [PDF]

Cureus
Rahman Z, Scotto T, Watford S, Woodbeck B, Ali K, Desai P, Abedi M, Reddy P.   +7 more
europepmc   +1 more source