Article
Medicine, Research & Experimental
M. Amelie Jule, J. Kacie Hoyt, Kevin Wei, Maria Gutierrez-Arcelus, L. Maria Taylor, Julie Ng, A. James Lederer, M. Siobhan Case, H. Margaret Chang, M. Ezra Cohen, Fatma Dedeoglu, M. Melissa Hazen, S. Jonathan Hausmann, Olha Halyabar, Erin Janssen, Jeffrey Lo, S. Mindy Lo, Esra Meidan, E. Jordan Roberts, F. Mary Beth Son, P. Robert Sundel, Y. Pui Lee, Talal Chatila, A. Peter Nigrovic, A. Lauren Henderson
Summary: Oligoarticular juvenile idiopathic arthritis (oligo JIA) is characterized by Th1 polarization, particularly prominent in CD4+ T cells, while not compromising the regulatory identity of Tregs. Single-cell RNA-Seq revealed heterogeneous effector and regulatory subpopulations in synovial fluid, including IFN-induced Tregs, peripheral helper T cells, and cytotoxic CD4+ T cells.
Article
Pharmacology & Pharmacy
Avtar Singh Gautam, Chinna Babu Pulivarthi, Rakesh Kumar Singh
Summary: IL-17 is a proinflammatory cytokine associated with various chronic inflammatory diseases. Its association with neurodegenerative diseases is inconclusive. A meta-analysis study showed a significant increase in serum IL-17 level in patients with Alzheimer's disease and amyotrophic lateral sclerosis, but no significant differences were found in Parkinson's disease and multiple sclerosis. IL-17 may be involved in regulating neuronal inflammation in the pathogenesis of these neurodegenerative diseases.
NAUNYN-SCHMIEDEBERGS ARCHIVES OF PHARMACOLOGY
(2023)
Article
Biochemistry & Molecular Biology
Marlos Aureliano Dias de Sousa, Chamberttan Souza Desiderio, Jonatas da Silva Catarino, Rafael Obata Trevisan, Djalma Alexandre Alves da Silva, Vinicius Ferreira Resende Rocha, Weslley Guimaraes Bovi, Rodolfo Pessato Timoteo, Renata Cristina Franzon Bonatti, Alex Eduardo da Silva, Alfredo Leboreiro Fernandez, Helioswilton Sales-Campos, Virmondes Rodrigues Junior, Marcos Vinicius da Silva, Carlo Jose Freire de Oliveira
Summary: The immune response in multiple sclerosis involves CD4(+) T cells that produce cytokines and chemokines. Changes in certain cytokines and chemokines, along with the presence of double positive lymphocytes, can help better understand the clinical forms and progression of the disease.
Article
Rheumatology
Justine Maller, Emily Fox, K. T. Park, Sarah Sertial Paul, Kevin Baszis, Charlotte Borocco, Sampath Prahalad, Pierre Quartier, Adam Reinhardt, Dieneke Schonenberg-Meinema, Lauren Shipman-Duensing, Maria Teresa Terreri, Julia Simard, Idit Lavi, Elizabeth Chalom, Joyces Hsu, Devy Zisman, Elizabeth D. Mellins
Summary: The occurrence of inflammatory bowel disease in the setting of systemic juvenile idiopathic arthritis is rare, with patients showing favorable response to tumor necrosis factor-alpha inhibitors.
JOURNAL OF RHEUMATOLOGY
(2021)
Article
Immunology
Sanjukta Majumder, Shivika Guleria, Amita Aggarwal
Summary: IL-36 may play a role in the pathogenesis of juvenile spondyloarthropathies. Elevated levels of IL-36 gamma in ERA patients are associated with disease activity. Pro-inflammatory cytokines can upregulate IL-36 gamma and IL-6 expression, potentially leading to an inflammatory feedback loop in ERA.
CLINICAL AND EXPERIMENTAL IMMUNOLOGY
(2022)
Review
Medicine, General & Internal
Duilio Petrongari, Paola Di Filippo, Francesco Misticoni, Giulia Basile, Sabrina Di Pillo, Francesco Chiarelli, Marina Attanasi
Summary: Systemic juvenile idiopathic arthritis associated with lung disorders (sJIA-LD) is a subtype of sJIA characterized by chronic life-threatening pulmonary disorders. It was exceptionally rare before 2013, but the reported prevalence is now approximately 6.8%. Clinically, children with sJIA-LD exhibit relatively mild symptoms, but the severity of pulmonary inflammation is high. sJIA-LD is often associated with early onset, macrophage activation syndrome, and high interleukin-18 circulating levels.
Article
Immunology
Shuya Kaneko, Masaki Shimizu, Futaba Miyaoka, Asami Shimbo, Hitoshi Irabu, Mao Mizuta, Yasuo Nakagishi, Naomi Iwata, Junya Fujimura, Masaaki Mori, Tomohiro Morio
Summary: This study validates the correlation between laboratory markers reflecting disease activity of macrophage activation syndrome (MAS) and serum cytokine levels, and identifies valuable laboratory markers that change over time for a prompt MAS diagnosis.
CLINICAL IMMUNOLOGY
(2023)
Article
Rheumatology
Tanja Hinze, Christoph Kessel, Claas H. Hinze, Julia Seibert, Hermann Gram, Dirk Foell
Summary: In patients with SJIA treated with canakinumab, baseline serum biomarkers such as IL-18 and IFN-gamma levels are associated with treatment outcome. Higher IL-18: CXCL9 and IFN-gamma: CXCL9 ratios at baseline are correlated with a better clinical response to canakinumab treatment. Further studies are needed to validate these findings and their applicability to patients with recent MAS.
Review
Biochemistry & Molecular Biology
Laura Marinela Ailioaie, Constantin Ailioaie, Gerhard Litscher
Summary: Systemic juvenile idiopathic arthritis (sJIA) and its complication, macrophage activation syndrome (sJIA-MAS), are rare but serious diseases in childhood. Biomarkers are important for distinguishing these diseases and predicting treatment response and relapse risk.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Immunology
Emilia A. Gaviglio, Javier M. Peralta Ramos, Daniela S. Arroyo, Claudio Bussi, Pablo Iribarren, Maria C. Rodriguez-Galan
Summary: The development of neuroinflammation and progression of neurodegenerative diseases are associated with the activation and mobilization of the peripheral immune system due to systemic inflammation. This study investigated the effects of systemic sterile induced-co-expression of IL-12 and IL-18 on the establishment of a cytokine-mediated model of neuroinflammation.
INTERNATIONAL IMMUNOPHARMACOLOGY
(2022)
Review
Rheumatology
Natsumi Inoue, Grant S. Schulert
Summary: Macrophage activation syndrome (MAS) is a life-threatening complication that occurs most commonly in children with systemic juvenile idiopathic arthritis (SJIA). The underlying pathophysiology of MAS complicating SJIA or other disorders remains unclear, making it challenging to develop and utilize animal models to study MAS. This review examines the proposed animal models of SJIA and MAS, highlighting their reflection of the disorders and their potential for further research.
ARTHRITIS RESEARCH & THERAPY
(2023)
Review
Medicine, General & Internal
Marino Paroli, Luca Spadea, Rosalba Caccavale, Leopoldo Spadea, Maria Pia Paroli, Nicola Nante
Summary: Interleukin-17A plays a central role in the pathogenesis of juvenile idiopathic arthritis (JIA) and blocking its production can effectively treat JIA. The use of the human anti-IL17A monoclonal antibody secukinumab has been approved in JIA treatment and has shown improvement in the course of the disease.
MEDICINA-LITHUANIA
(2022)
Article
Pediatrics
Ninna Brix, Mia Glerup, Dirk Foell, Christoph Kessel, Helmut Wittkowski, Lillemor Berntson, Anders Fasth, Susan Nielsen, Ellen Nordal, Marite Rygg, Henrik Hasle, Troels Herlin
Summary: This study evaluated the predictive value of biomarkers of inflammation to differentiate children with ALL from JIA. The levels of S100A9, S100A12, IL-1β, IL-4, IL-13, IL-17, matrix metalloproteinase-3, and myeloperoxidase were low in ALL compared with JIA. IL-13, IL-4, and S100A9 showed high predictive performance and could be valuable markers to differentiate ALL from JIA.
JOURNAL OF PEDIATRICS
(2023)
Article
Oncology
Sandra Guallar-Garrido, Victor Campo-Perez, Miriam Perez-Trujillo, Cecilia Cabrera, Jordi Senserrich, Alejandro Sanchez-Chardi, Rosa Maria Rabanal, Elisabet Gomez-Mora, Estela Noguera-Ortega, Marina Luquin, Esther Julian
Summary: The mechanism of action of intravesical Mycobacterium bovis BCG immunotherapy treatment for bladder cancer is not completely understood. The culture conditions of BCG strains can impact their antitumor effect. This study found that each mycobacterium requires specific culture conditions for an effective antitumor response. M. brumae-A60 and BCG-G15 showed the highest survival rates and immune responses in a murine bladder cancer model.
Article
Chemistry, Medicinal
Renata Virginia Cavalcanti Santos, Eudes Gustavo Constantino Cunha, Gabriela Souto Vieira de Mello, Jose Angelo Rizzo, Jamerson Ferreira de Oliveira, Maria do Carmo Alves de Lima, Ivan da Rocha Pitta, Maira Galdino da Rocha Pitta, Moacyr Jesus Barreto de Melo Rego
Summary: This study evaluated the IL-17A and IFN-gamma modulatory effect of two new oxazolidine derivatives on mononucleated cells of patients with moderate and severe asthma. The results showed that the compounds reduced IFN-gamma and IL-17 production in a concentrated manner in peripheral blood mononucleated cells from asthmatic patients. In silico analysis revealed the neurokinin-1 receptor as a common target for both compounds, indicating a potential mechanism for their effects.
MEDICINAL CHEMISTRY
(2021)
Article
Allergy
David Egg, Ina Caroline Rump, Noriko Mitsuiki, Jessica Rojas-Restrepo, Maria-Elena Maccari, Charlotte Schwab, Annemarie Gabrysch, Klaus Warnatz, Sigune Goldacker, Virginia Patino, Daniel Wolff, Satoshi Okada, Seiichi Hayakawa, Yoshiaki Shikama, Kenji Kanda, Kohsuke Imai, Manabu Sotomatsu, Makoto Kuwashima, Takahiro Kamiya, Tomohiro Morio, Kazuaki Matsumoto, Takeshi Mori, Yuri Yoshimoto, Ingunn Dybedal, Maria Kanariou, Zeynep Yesim Kucuk, Hugo Chapdelaine, Lenka Petruzelkova, Hanns-Martin Lorenz, Kathleen E. Sullivan, Jennifer Heimall, Michel Moutschen, Jiri Litzman, Mike Recher, Michael H. Albert, Fabian Hauck, Suranjith Seneviratne, Jana Pachlopnik Schmid, Antonios Kolios, Gary Unglik, Christian Klemann, Scott Snapper, Lisa Giulino-Roth, Michael Svaton, Craig D. Platt, Sophie Hambleton, Olaf Neth, Geraldine Gosse, Steffen Reinsch, Dirk Holzinger, Yae-Jean Kim, Shahrzad Bakhtiar, Faranaz Atschekzei, Reinhold Schmidt, Georgios Sogkas, Shanmuganathan Chandrakasan, William Rae, Beata Derfalvi, Hanne Vibeke Marquart, Ahmet Ozen, Ayca Kiykim, Elif Karakoc-Aydiner, Pavlina Kralickova, Godelieve de Bree, Dimitra Kiritsi, Markus G. Seidel, Robin Kobbe, Jennifer Dantzer, Laia Alsina, Thais Armangue, Vassilios Lougaris, Philipp Agyeman, Sofia Nystrom, David Buchbinder, Peter D. Arkwright, Bodo Grimbacher
Summary: Heterozygous germline mutations in cytotoxic T lymphocyte-associated antigen-4 (CTLA4) can lead to life-threatening autoimmune and lymphoproliferative complications. Treatment options include systemic immunosuppressants, immunoglobulin replacement, and stem cell transplantation for potential cure.
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY
(2022)
Article
Allergy
Miriam Gross, Carsten Speckmann, Annette May, Tania Gajardo-Carrasco, Katharina Wustrau, Sarah Lena Maier, Marcus Panning, Daniela Huzly, Abbas Agaimy, Yenan T. Bryceson, Sharon Choo, C. W. Chow, Gregor Dueckers, Anders Fasth, Sylvie Fraitag, Katja Graewe, Sabine Haxelmans, Dirk Holzinger, Ole Hudowenz, Judith M. Huebschen, Claudia Khurana, Korbinian Kienle, Roman Klifa, Klaus Korn, Heinz Kutzner, Tim Laemmermann, Svea Ledig, Dan Lipsker, Marie Meeths, Nora Naumann-Bartsch, Jelena Rascon, Anne Schaenzer, Maximilian Seidl, Bianca Tesi, Christelle Vauloup-Fellous, Beate Vollmer-Kary, Klaus Warnatz, Claudia Wehr, Benedicte Neven, Pablo Vargas, Fernando E. Sepulveda, Kai Lehmberg, Annette Schmitt-Graeff, Stephan Ehl
Summary: This study investigated the defective effector mechanism allowing rubella vaccine virus persistence in granulomas by studying patients with cytotoxicity defects and granuloma. The results suggest a critical role of T cells in rubella elimination.
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY
(2022)
Article
Rheumatology
Carolin Park, Maria Miranda-Garcia, Rainer Berendes, Gerd Horneff, Jasmin Kuemmerle-Deschner, Gerd Ganser, Hans-Iko Huppertz, Kirsten Minden, Johannes-Peter Haas, Annette F. Jansson, Michael Borte, Catharina Schuetz, Prasad Oommen, Michael Frosch, Bernhard Schlueter, Annette Richter-Unruh, Christoph Kessel, Claas Hinze, Helmut Wittkowski, Johannes Roth, Dirk Foell, Dirk Holzinger
Summary: This study validated the usefulness of MRP8/14 serum analyses in supporting the diagnosis of SJIA in febrile children. The results could be confirmed with commercial ELISA and LFIA, enabling a rapid diagnostic point-of-care screening test.
Article
Rheumatology
Joachim Gerss, Monika Tedy, Ariane Klein, Gerd Horneff, Maria Miranda-Garcia, Christoph Kessel, Dirk Holzinger, Valda Stanevica, Joost F. Swart, David A. Cabral, Hermine Brunner, Dirk Foell
Summary: This study investigates the use of high-sensitivity C-reactive protein (hsCRP) and S100A12 as predictive biomarkers for successful drug withdrawal in children with clinical remission of juvenile idiopathic arthritis (JIA). The results show that using these predictive markers of subclinical inflammation is a promising tool in the decision-making process of therapy withdrawal, resulting in direct benefit for patients.
ANNALS OF THE RHEUMATIC DISEASES
(2022)
Article
Rheumatology
Emma J. Sumner, Beverley Almeida, Jason Palman, Peter Bale, Clare Heard, Dirk Holzinger, Johannes Roth, Dirk Foell, Emily Robinson, Simona Ursu, Chris Wallace, Kimberly Gilmour, Lucy R. Wedderburn, Elizabeth Ralph
Summary: The objective of this study was to determine the effectiveness of MRP8/14 as a predictor of disease flare in patients with juvenile idiopathic arthritis (JIA) following the withdrawal of methotrexate (MTX) in a routine clinical setting. The study found that there was no significant difference in flares between patients with an elevated or low MRP8/14 value. However, in those who stopped MTX, no patients with a low MRP8/14 result flared.
CLINICAL RHEUMATOLOGY
(2022)
Article
Rheumatology
Linda Ziegler, Sabrina Fuehner, Christoph Kessel, Claas Hinze, Jens Klotsche, Martina Niewerth, Kirsten Minden, Dirk Foell
Summary: This study investigated the changes in serum biomarkers during inactive disease in JIA patients to evaluate their usefulness in identifying patients at risk for disease relapse. The results showed that sIL-2R serum levels were significantly higher in patients with future active disease, particularly in certain subtypes of JIA. Higher sIL-2R serum levels during inactive disease may help identify patients with subclinical disease activity at risk for future active disease, compared to conventional inflammation parameters.
Article
Pediatrics
Kristina Vollbach, Catharina Schuetz, Christian M. Hedrich, Fabian Speth, Kirsten Moenkemoeller, Juergen Brunner, Ulrich Neudorf, Christoph Rietschel, Anton Hospach, Tilmann Kallinich, Claas Hinze, Norbert Wagner, Burkhard Toenshoff, Lutz T. Weber, Kay Latta, Julia Thumfart, Martin Bald, Dagobert Wiemann, Hildegard Zappel, Klaus Tenbrock, Dieter Haffner
Summary: The majority of pediatric rheumatologists and nephrologists in Germany and Austria recommend the use of corticosteroids, typically in combination with either MMF or CP, for the treatment of proliferative LN in children. The heterogeneity of responses highlights the need for a treat-to-target protocol in the management of juvenile proliferative LN.
FRONTIERS IN PEDIATRICS
(2022)
Article
Hematology
Laura M. Moser, Julia Fekadu, Andre Willasch, Eva Rettinger, Jan Soerensen, Andrea Jarisch, Marta Kirwil, Adrian Lieb, Dirk Holzinger, Gabriele Calaminus, Peter Bader, Shahrzad Bakhtiar
Summary: This study reports on the successful treatment of inborn errors of immunity (IEI) patients with treatment-refractory inflammatory bowel disease (IBD) using allogeneic hematopoietic stem cell transplantation (alloHSCT). The patients achieved stable engraftment, immune reconstitution, and resolution of gastrointestinal symptoms. The overall survival rate was 100% over a median follow-up of 34.7 months.
BRITISH JOURNAL OF HAEMATOLOGY
(2023)
Article
Immunology
Rensheng Wan, Johannes Faender, Ia Zakaraia, Min Ae Lee-Kirsch, Christine Wolf, Nadja Lucas, Lisa Isabel Olfe, Corinna Hendrich, Danny Jonigk, Dirk Holzinger, Mathis Steindor, Gunnar Schmidt, Claudia Davenport, Christian Klemann, Nicolaus Schwerk, Matthias Griese, Brigitte Schlegelberger, Florian Stehling, Christine Happle, Bernd Auber, Doris Steinemann, Martin Wetzke, Sandra von Hardenberg
Summary: This study reports four novel cases and eight historic cases of autosomal recessive SAVI caused by c.841C>T homozygosity. Comprehensive clinical data and treatment regimens are provided, and the study aims to raise awareness for autosomal recessive inheritance in this rare, severe disease, which may aid in early diagnosis and development of optimized treatment strategies.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Immunology
Prasad T. Oommen, Timmy Strauss, Karen Baltruschat, Ivan Foeldvari, Christoph Deuter, Gerd Ganser, Johannes-Peter Haas, Claas Hinze, Dirk Holzinger, Anton Hospach, Hans-Iko Huppertz, Arnold Illhardt, Michael Jung, Tilmann Kallinich, Ariane Klein, Kirsten Minden, Kirsten Monkemoller, Sonja Mrusek, Ulrich Neudorf, Gregor Dueckers, Tim Niehues, Matthias Schneider, Philipp Schoof, Angelika Thon, Michael Wachowsky, Norbert Wagner, Susanne Bloedt, Michael Hofer, Klaus Tenbrock, Catharina Schuetz
Summary: This article summarizes the recent update of the interdisciplinary, consensus-based German guidelines on the management of juvenile idiopathic arthritis (JIA). Through multi-disciplinary and consensus participation, therapeutic principles and recommendations were proposed, providing physicians with evidence-based tailored treatment approaches.
CLINICAL IMMUNOLOGY
(2022)
Article
Rheumatology
Julia Kuehn, Susanne Schleifenbaum, Michaela Hendling, Sandra Siebenhandl, Julie Krainer, Sabrina Fuehner, Antje Hellige, Carolin Park, Claas Hinze, Helmut Wittkowski, Dirk Holzinger, Lorenz Thurner, Andreas Weinhaeusel, Dirk Foell, Christoph Kessel
Summary: This study found that aberrant or incomplete polarization of T helper cells may contribute to the pathological processes in systemic juvenile idiopathic arthritis (sJIA).
ARTHRITIS & RHEUMATOLOGY
(2023)
Meeting Abstract
Oncology
Kai Lehmberg, Svea Ledig, Katharina Wustrau, Udo Kontny, Silke Westphal, Patrick Hundsdoerfer, Norbert Jorch, Johanna Scheer-Preiss, Dominik Schneider, Sujal Ghosh, Nora Naumann-Bartsch, Dirk Holzinger, Shahrzad Bakhtiar, Christine Mauz-Koerholz, Christof Kramm, Rita Beier, Wolfgang Behnisch, Monika Streiter, Alfred Laengler, Rhoikos Furtwaengler, Bernd Gruhn, Michaela Nathrath, Uemmueguel Behr, Meinolf Siepermann, Lars Fischer, Antje Redlich, Alexandra Russo, Matthias Duerken, Fabian Hauck, Martina Ahlmann, Martin Irnich, Heiko-Manuel Teltschik, Peter Lang, Manfred Hoenig, Ansgar Schulz, Sharon Choo, Bruce Crooks, Renata Formankova, Marianne Ifversen, Thais Murciano-Carrillo, Itziar Astigarraga, Juana Gil Herrera, Tal Ben-Ami, Joanne Yacobovich, Iwona Malinowska, Michael Jordan, Melissa Hines, Julie-An Talano, Jana Pachlopnik Schmid, Stephan Ehl
PEDIATRIC BLOOD & CANCER
(2023)