Results 41 to 50 of about 2,294 (167)

Defective apoptosis of peripheral-blood lymphocytes in hyper-IgD and periodic fever syndrome.

, 2007
Hereditary periodic fever syndromes are characterized by incapacitating attacks of fever and generalized inflammation. While the mutated genes for the major syndromes in this group are known, the pathogenesis remains unclear. The aim of this study was to
Heerde, W.L. van, Bodar, E.J., Drenth, J.P.H., Simon, A., Meer, J.W.M. van der, Hilst, J.C.H. van der   +5 more
core   +1 more source

Review of Biological Agents in the Therapeutic Management of Monogenic Genodermatoses

Dermatologic Therapy, Volume 2026, Issue 1, 2026.
Monogenic genodermatoses encompass a diverse group of over 400 distinct disorders, presenting significant therapeutic challenges. Recent advancements in the clinical application of biological agents have heralded a new era in the management of these conditions.
Xueying Wang, Qing Zhao, Hong Liu, Furen Zhang, Zhiqiang Song   +4 more
wiley   +1 more source

Spectrum of clinical features and genetic variants in mevalonate kinase (MVK) gene of South Indian families suffering from Hyperimmunoglobulin D Syndrome.

PLoS ONE, 2020
Hyper-IgD syndrome (HIDS, OMIM #260920) is a rare autosomal recessive autoinflammatory disorder caused by pathogenic variants in the mevalonate kinase (MVK) gene.
Geeta Madathil Govindaraj, Abhinav Jain, Geetha Peethambaran, Rahul C Bhoyar, Shamsudheen Karuthedath Vellarikkal, Arvind Ganapati, Pulukool Sandhya, Athulya Edavazhippurath, Dhananjayan Dhanasooraj, Jayakrishnan Machinary Puthenpurayil, Krishnan Chakkiyar, Anushree Mishra, Arushi Batra, Anu Punnen, Sathish Kumar, Sridhar Sivasubbu, Vinod Scaria   +16 more
doaj   +1 more source

Cytokine Signatures Outperform Immune Subsets in Machine Learning Models for Predicting Acute Graft‐Versus‐Host Disease at Neutrophil Engraftment

Journal of Immunology Research, Volume 2026, Issue 1, 2026.
Background Acute graft‐versus‐host disease (aGvHD) is a major immune complication of allogeneic hematopoietic stem cell transplantation (Allo‐HSCT), driven by complex immune‐cytokine interactions. This study employed machine learning (ML) algorithms to develop early predictive models for aGvHD using immune and cytokine profiles of Allo‐HSCT recipients ...
Mohini Mendiratta, Praful Pandey, Shobhit Pandey, Sandeep Rai, Meenakshi Mendiratta, Hridayesh Prakash, Shuvadeep Ganguly, Archana Sasi, Ritu Gupta, Prabhat Singh Malik, Raja Pramanik, Sachin Kumar, Baibaswata Nayak, Riyaz Ahmed Mir, Sameer Bakhshi, Deepam Pushpam, Mukul Aggarwal, Aditya Kumar Gupta, Rishi Dhawan, Tulika Seth, Manoranjan Mahapatra, Ranjit Kumar Sahoo, Poorani Gurumallesh Prabu   +22 more
wiley   +1 more source

Vasculitis in Systemic Autoinflammatory Diseases

Frontiers in Pediatrics, 2018
Autoinflammatory diseases (AID) are diseases of the innate immune system, characterized by recurrent episodes of localized or systemic inflammation. Vasculitis may accompany AID.
Selcan Demir, Erdal Sag, Fatma Dedeoglu, Seza Ozen   +3 more
doaj   +1 more source

Multi‐omic analysis identifies biological processes underlying progressive interstitial lung disease in systemic sclerosis

The FEBS Journal, Volume 292, Issue 22, Page 6054-6074, November 2025.
Systemic sclerosis (SSc) is a rare autoimmune disease, and lung complications (ILD) are the main cause of death. This study compared SSc patients with and without lung disease to healthy volunteers. We found increased inflammation, specific proteins, and higher triglyceride levels linked to lung disease progression. These findings suggest triglycerides
Selena Bouffette, Perrine Soret, Alice Cole, Emmanuel Nony, Pierre Barbier Saint Hilaire, Grégory Leclerc, Lamine Alaoui, Audrey Aussy, Isabelle Wehrle, Aude Le Gall, Mark Coulon, Marc Pallardy, Voon Ong, Jeanne Allinne, Philippe Moingeon, David Abraham, Christopher P. Denton   +16 more
wiley   +1 more source

Hyper IgD syndrome (HIDS) associated with in vitro evidence of defective monocyte TNFRSF1A shedding and partial response to TNF receptor blockade with etanercept

, 2002
Hereditary periodic fever syndromes comprise a group of distinct disease entities linked by the defining feature of recurrent febrile episodes. Hyper IgD with periodic fever syndrome (HIDS) is caused by mutations in the mevalonate kinase (MVK) gene.
Houten, S. M., Waterham, H. R., Mirakian, R. M., Vijaydurai, P. I., Hammond, L. J., McDermott, M. F., Arkwright, P. D., Tomlin, P. I., Cant, A. J., Frenkel, J., Aganna, E.   +10 more
core   +1 more source

The Immunology of Alpha‐Gal Syndrome: History, Tick Bites, IgE, and Delayed Anaphylaxis to Mammalian Meat

Immunological Reviews, Volume 332, Issue 1, July 2025.
ABSTRACT The primary features of the alpha‐gal syndrome (AGS) are (i) The IgE ab that are causally related to anaphylaxis with infusions of Cetuximab are specific for galactose alpha‐1,3‐galactose. (ii) In the USA, this IgE ab is induced by bites of the tick Amblyomma americanum. (iii) The anaphylactic reactions to food derived from non‐primate mammals
Thomas A. E. Platts‐Mills, Roopesh Singh Gangwar, Lisa Workman, Jeffrey M. Wilson   +3 more
wiley   +1 more source

Immunoglobulin class‐switch recombination: Mechanism, regulation, and related diseases

MedComm
Maturation of the secondary antibody repertoire requires class‐switch recombination (CSR), which switches IgM to other immunoglobulins (Igs), and somatic hypermutation, which promotes the production of high‐affinity antibodies.
Jia‐Chen Liu, Ke Zhang, Xu Zhang, Fei Guan, Hu Zeng, Masato Kubo, Pamela Lee, Fabio Candotti, Louisa Katherine James, Niels Olsen Saraiva Camara, Kamel Benlagha, Jia‐Hui Lei, Huamei Forsman, Lu Yang, Wei Xiao, Zheng Liu, Chao‐Hong Liu   +16 more
doaj   +1 more source

AUTOINFLAMMATORY DISEASES IN RHEUMATOLOGY: RUSSIAN EXPERIENCE

Научно-практическая ревматология, 2016
Autoinflammatory diseases (AIDs) are characterized by periodic, sometimes self-limiting attacks that appear as fever and clinical symptoms resembling rheumatic ones, in the absence of autoimmune or infectious diseases.
S. O. Salugina, E. S. Fedorov, N. N. Kuzmina, E. A. Kamenets, E. Yu. Zakharova   +4 more
doaj   +1 more source