Article
Immunology
Suying Liu, Linna Han, Yanhui Liu, Jun Yang, Yu Zhang, Mengtao Li, Xinping Tian, Xiaofeng Zeng, Li Wang, Fengchun Zhang
Summary: This study investigates the clinical significance of myeloperoxidase (MPO)-antineutrophil cytoplasmic antibody (ANCA) on eosinophilic granulomatosis with polyangiitis (EGPA). The results suggest that MPO-ANCA positivity is associated with distinct clinical features and renal involvement in EGPA patients. MPO-ANCA may serve as a valuable biomarker for stratifying EGPA and predicting disease activity and renal involvement.
FRONTIERS IN IMMUNOLOGY
(2022)
Review
Medicine, General & Internal
Ryu Watanabe, Motomu Hashimoto
Summary: This review summarizes the latest knowledge on the pathophysiology of eosinophilic granulomatosis with polyangiitis (EGPA) and focuses on the roles of eosinophils and antineutrophil cytoplasmic antibody (ANCA). Furthermore, it introduces the current treatment recommendations and accumulated evidence for mepolizumab on EGPA. Based on current unmet clinical needs, potential future therapeutic strategies for EGPA are discussed.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Medicine, General & Internal
Peng-Fang Tang, Long-Chuan Xu, Wen-Ting Hong, Hong-Ying Shi
Summary: This case report describes a 40-year-old male patient with granulomatosis with polyangiitis (GPA) who showed no improvement with multiple rounds of drugs, but achieved symptom relief and normalization of inflammatory markers after tocilizumab treatment.
WORLD JOURNAL OF CLINICAL CASES
(2023)
Review
Immunology
Mitsuhiro Akiyama, Yuko Kaneko, Tsutomu Takeuchi
Summary: Rituximab was found effective in inducing and sustaining remission, reducing glucocorticoid dose, and potentially providing greater benefit in ANCA-positive patients with EGPA. Scheduled maintenance treatment with rituximab significantly decreased relapse rates compared to on-demand administration, with no new safety concerns reported.
AUTOIMMUNITY REVIEWS
(2021)
Article
Gastroenterology & Hepatology
Hiroko Sato, Tsuyoshi Shirai, Hiroshi Fujii, Tomonori Ishii, Hideo Harigae
Summary: Immediate discontinuation of CPA and intensive supportive therapy are crucial for the survival of patients with CPA-associated severe enteritis.
WORLD JOURNAL OF GASTROENTEROLOGY
(2021)
Article
Medicine, General & Internal
Lirong Lin, Rongjie Yu, Luquan Zheng, Shuyu Gong, Jurong Yang
Summary: EGPA can present with a rare initial presentation of oral granuloma. Methylprednisolone and cyclophosphamide can be a suitable choice of treatment.
FRONTIERS IN MEDICINE
(2022)
Article
Medicine, General & Internal
Hiroyuki Ide, Toshimasa Shimizu, Yuta Koike, Kuniko Abe, Kazuto Shigematsu, Shinya Nishihata, Kanako Kojima, Kunihiro Ichinose, Atsushi Kawakami
Summary: This case report presents a patient who developed eosinophilic granulomatosis with polyangiitis (EGPA) during the clinical course of microscopic polyangiitis (MPA), suggesting that EGPA can be induced by eosinophilic inflammation in other subgroups of antineutrophil cytoplasmic autoantibody-associated vasculitis (AAV).
Article
Rheumatology
Peter C. Grayson, Cristina Ponte, Ravi Suppiah, Joanna C. Robson, Anthea Craven, Andrew Judge, Sara Khalid, Andrew Hutchings, Raashid A. Luqmani, Richard A. Watts, Peter A. Merkel
Summary: The revised classification criteria for EGPA demonstrate strong performance characteristics and are validated for use in research.
ANNALS OF THE RHEUMATIC DISEASES
(2022)
Article
Medicine, General & Internal
Yume Nakamura, Fumihiko Okumura, Mayumi Nihei, Kei Chubachi, Hiroaki Sakamoto, Yoshiko Ishioka, Tomonori Makiguchi, Hisashi Tanaka, Kageaki Taima, Nobuhisa Yajima, Akira Kurose, Sadatomo Tasaka
Summary: We report a case of GPA following COVID-19 in an adolescent girl, who presented with facial allodynia, fever, and weight loss one month after mild COVID-19. Multiple nodular shadows were found on a chest roentgenogram. GPA was diagnosed based on pathological findings, and the patient showed improvement with methylprednisolone and rituximab treatment.
Article
Geriatrics & Gerontology
Hirokazu Sugiyama, Makoto Yamaguchi, Takayuki Katsuno, Shiho Iwagaitsu, Hironobu Nobata, Hiroshi Kinashi, Shogo Banno, Masahiko Ando, Yoko Kubo, Takuji Ishimoto, Yasuhiko Ito
Summary: In a single-center retrospective cohort study in Japan, it was found that low BMI was associated with a higher risk of severe infection in older adults with microscopic polyangiitis (MPA), highlighting the need for careful management to prevent this complication in this vulnerable group. Further research is needed to determine the optimal treatment strategy for these patients.
Article
Multidisciplinary Sciences
Takumi Tashiro, Urara Fujiwara, Yuichi Kira, Chihiro Karashima, Norihisa Maeda
Summary: A 77-year-old woman with sinusitis developed embolic stroke and splenic infarctions, and was diagnosed with eosinophilic granulomatosis with polyangiitis characterized by eosinophilic infiltration. Cardiac imaging also revealed evidence of endo-myocarditis. The patient received immunosuppressive and anticoagulation therapy, and no recurrence of infarctions was observed.
Review
Cell Biology
Wolfgang H. Goldmann
Summary: Wegener's granulomatosis is a form of systemic vasculitis characterized by inflammation, vasculitis, and glomerulonephritis. PR3-ANCAs are strongly associated with the disease, but their pathogenic significance in vivo is still unclear. Animal models have been used to study the autoimmune origin and pathophysiology of WG, with the goal of better understanding and treating the disease.
CELL BIOLOGY INTERNATIONAL
(2022)
Article
Medicine, General & Internal
Gaku Kuwabara, Koichi Yamada, Kiyomi Tanaka, Satoshi Nozuchi, Waki Imoto, Wataru Shibata, Mitsunori Tohda, Shigenori Kyoh, Yoshiaki Itoh, Motomu Hashimoto, Hiroshi Kakeya
Summary: We present a case of tuberculosis with muscle biopsy-proven microscopic polyangiitis (MPA). The patient developed persistent fever after tuberculosis treatment and tested positive for myeloperoxidase-antineutrophil cytoplasmic antibody (ANCA). Despite the absence of conventional symptoms, a muscle biopsy of the biceps femoris confirmed small vessel vasculitis, leading to the diagnosis of MPA. Glucocorticoids successfully alleviated the fever. Tuberculosis and antituberculosis drugs can induce ANCA-associated vasculitis (AAV), highlighting the importance of muscle biopsy in the diagnosis of AAV.
Article
Rheumatology
Peter C. Grayson, Cristina Ponte, Ravi Suppiah, Joanna C. Robson, Anthea Craven, Andrew Judge, Sara Khalid, Andrew Hutchings, Raashid A. Luqmani, Richard A. Watts, Peter A. Merkel
Summary: This study aimed to develop and validate revised classification criteria for EGPA. Through the identification, collection, and analysis of candidate criteria items, a risk score system was established for disease classification in EGPA patients and comparator diseases. The criteria demonstrated strong sensitivity and specificity after validation.
ARTHRITIS & RHEUMATOLOGY
(2022)
Article
Rheumatology
Maxime Samson, Herve Devilliers, Sara Thietart, Pierre Charles, Christian Pagnoux, Pascal Cohen, Alexandre Karras, Luc Mouthon, Benjamin Terrier, Xavier Puechal, Loic Guillevin
Summary: The study aimed to develop a score for assessing the likelihood of relapse in granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). Long-term follow-up data from patients with GPA and MPA included in five randomized controlled trials were pooled. Variables associated with relapse were identified and used to build a score, which was validated in an independent cohort.
Article
Hematology
Arianna Troilo, Claudia Wehr, Iga Janowska, Nils Venhoff, Jens Thiel, Justyna Rawluk, Natalie Frede, Julian Staniek, Raquel Lorenzetti, Marei-Theresa Schleyer, Georg W. Herget, Lukas Konstantinidis, Miriam Erlacher, Michele Proietti, Nadezhda Camacho-Ordonez, Reinhard Edmund Voll, Bodo Grimbacher, Klaus Warnatz, Ulrich Salzer, Marta Rizzi
Editorial Material
Immunology
Tomas Kalina, Roshini S. Abraham, Marta Rizzi, Mirjam van der Burg
FRONTIERS IN IMMUNOLOGY
(2020)
Article
Allergy
Patrick Kury, Julian Staniek, Oliver Wegehaupt, Iga Janowska, Matthias Eckenweiler, Rudolf Korinthenberg, Natia Japaridze, Manuela Pendziwiat, Ingo Helbig, Els Verhoeyen, Johannes Jung, Andres Caballero Garcia de Oteyza, Michele Proietti, Tamar Phirtskhalaishvili, Irakli Rtskhiladze, Peter J. Nielsen, Stephan Ehl, Carsten Speckmann, Marta Rizzi
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY
(2021)
Editorial Material
Cell Biology
Olivier Micheau, Marta Rizzi, Cristian R. Smulski
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Article
Multidisciplinary Sciences
Valerie Oberhardt, Hendrik Luxenburger, Janine Kemming, Isabel Schulien, Kevin Ciminski, Sebastian Giese, Benedikt Csernalabics, Julia Lang-Meli, Iga Janowska, Julian Staniek, Katharina Wild, Kristi Basho, Mircea Stefan Marinescu, Jonas Fuchs, Fernando Topfstedt, Ales Janda, Oezlem Sogukpinar, Hanna Hilger, Katarina Stete, Florian Emmerich, Bertram Bengsch, Cornelius F. Waller, Siegbert Rieg, Sagar, Tobias Boettler, Katharina Zoldan, Georg Kochs, Martin Schwemmle, Marta Rizzi, Robert Thimme, Christoph Neumann-Haefelin, Maike Hofmann
Summary: After vaccination, CD8(+) T cells become important effector cells in providing early protection, being effectively mobilized one week after primary vaccination and maintaining stability after booster vaccination. Compared with natural infection, vaccine-induced CD8(+) T cells exhibit similar functional capacities but with a different subset distribution.
Article
Immunology
Anna-Lena Schaefer, Alexandra Eichhorst, Carolin Hentze, Antoine N. N. Kraemer, Anais Amend, Dalina T. L. Sprenger, Cara Fluhr, Stephanie Finzel, Christoph Daniel, Ulrich Salzer, Marta Rizzi, Reinhard E. Voll, Nina Chevalier
Summary: This study found that low fiber intake exacerbates disease progression in lupus-prone mice, microbiota suppression does not affect disease course as originally assumed, low fiber diet leads to increased white adipose tissue mass, fat inflammation and disrupted intestinal homeostasis, driving systemic low-grade inflammation and autoimmunity. The research highlights the overlooked effects of dietary fiber on energy homeostasis and obesity prevention, showing the complex interplay between inflammatory immune-mediated conditions such as obesity and autoimmunity.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Immunology
Markus Zeisbrich, Nina Chevalier, Bettina Sehnert, Marta Rizzi, Nils Venhoff, Jens Thiel, Reinhard E. Voll
Summary: This study reveals a deficiency of the immunoinhibitory checkpoint PD-L1 in monocytes from AAV patients. Reduced expression of CMTM6 leads to increased lysosomal degradation of PD-L1, providing insufficient negative signaling to T cells. Correcting this defect by targeting lysosomal function may offer a novel strategy for treating AAV.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Multidisciplinary Sciences
Hanna Renk, Alex Dulovic, Alina Seidel, Matthias Becker, Dorit Fabricius, Maria Zernickel, Daniel Junker, Ruediger Gross, Janis Mueller, Alexander Hilger, Sebastian F. N. Bode, Linus Fritsch, Pauline Frieh, Anneke Haddad, Tessa Goerne, Jonathan Remppis, Tina Ganzemueller, Andrea Dietz, Daniela Huzly, Hartmut Hengel, Klaus Kaier, Susanne Weber, Eva-Maria Jacobsen, Philipp D. Kaiser, Bjoern Traenkle, Ulrich Rothbauer, Maximilian Stich, Burkhard Toenshoff, Georg F. Hoffmann, Barbara Mueller, Carolin Ludwig, Bernd Jahrsdoerfer, Hubert Schrezenmeier, Andreas Peter, Sebastian Hoerber, Thomas Iftner, Jan Muench, Thomas Stamminger, Hans-Juergen Gross, Martin Wolkewitz, Corinna Engel, Weimin Liu, Marta Rizzi, Beatrice H. Hahn, Philipp Henneke, Axel R. Franz, Klaus-Michael Debatin, Nicole Schneiderhan-Marra, Ales Janda, Roland Elling
Summary: This study compares the humoral immune response in children and adults following SARS-CoV-2 infection and finds that children are more likely to have asymptomatic infections and higher levels of specific antibodies that persist for a longer time. Symptomatic and asymptomatic infections induce similar humoral responses across all age groups.
NATURE COMMUNICATIONS
(2022)
Article
Immunology
Baerbel Keller, Valentina Strohmeier, Ina Harder, Susanne Unger, Kathryn J. Payne, Geoffroy Andrieux, Melanie Boerries, Peter Tobias Felixberger, Jonathan J. M. Landry, Alexandra Nieters, Anne Rensing-Ehl, Ulrich Salzer, Natalie Frede, Susanne Usadel, Roland Elling, Carsten Speckmann, Ina Hainmann, Elizabeth Ralph, Kimberly Gilmour, Marjolein W. J. Wentink, Mirjam van der Burg, Hye Sun Kuehn, Sergio D. Rosenzweig, Uwe Kolsch, Horst von Bernuth, Petra Kaiser-Labusch, Florian Gothe, Sophie Hambleton, Alexandru Daniel Vlagea, Ana Garcia Garcia, Laia Alsina, Gasper Markelj, Tadej Avcin, Julia Vasconcelos, Margarida Guedes, Jing-Ya Ding, Cheng-Lung Ku, Bella Shadur, Danielle T. Avery, Nils Venhoff, Jens Thiel, Heiko Becker, Lucia Erazo-Borras, Claudia Milena Trujillo-Vargas, Jose Luis Franco, Claire Fieschi, Satoshi Okada, Paul E. Gray, Gulbu Uzel, Jean-Laurent Casanova, Manfred Fliegauf, Bodo Grimbacher, Hermann Eibel, Stephan Ehl, Reinhard E. Voll, Marta Rizzi, Polina Stepensky, Vladimir Benes, Cindy S. Ma, Claudia Bossen, Stuart G. Tangye, Klaus Warnatz
Summary: The accumulation of CD21(low) B cells in peripheral blood is a hallmark of chronic activation of the adaptive immune system in certain infections and autoimmune disorders. The transcription factor T-bet plays a prominent role in the regulation of T-bet(high)CD21(low) B cells, and essential signals such as BCR and IFN gamma R are crucial for the differentiation and expansion of these cells. Identification of important pathways provides potential targets for auto-reactive T-bet(high)CD21(low) B cells in autoimmune conditions.
SCIENCE IMMUNOLOGY
(2021)
Review
Immunology
Susana Minguet, Alexander Nystrom, Dimitra Kiritsi, Marta Rizzi
Summary: B-cell tolerance to self-antigen is crucial for immune system function. Genetic defects can disrupt this tolerance and lead to immunodeficiency and autoimmune diseases. Research in humans has identified these defects and developed therapeutic interventions to restore tolerance and treat immunopathologies.
EUROPEAN JOURNAL OF IMMUNOLOGY
(2022)
Article
Cell Biology
Cristian R. Smulski, Luyao Zhang, Malte Burek, Ariadna Teixido Rubio, Jana-Susann Briem, Mauricio P. Sica, Eirini Sevdali, Michele Vigolo, Laure Willen, Patricia Odermatt, Duygu Istanbullu, Stephanie Herr, Marco Cavallari, Henry Hess, Marta Rizzi, Hermann Eibel, Pascal Schneider
Summary: TACI plays a crucial role in regulating class-switch recombination, plasma cell differentiation, and proliferation in mature B cells. Mutations in TACI, found in some CVID patients, impair its functions and disrupt immune responses.
Article
Immunology
Jakov Korzhenevich, Iga Janowska, Mirjam van der Burg, Marta Rizzi
Summary: This article reviews the similarities and differences in early B cell development between humans and mice, and provides a phenotypical classification of human and murine early progenitors and B cell stages based on protein expression. The role of key transcription factors and chemo- or cytokine signals during homeostatic and aberrant B lymphopoiesis in both species is critically compared.
IMMUNOLOGY LETTERS
(2023)
Article
Cell & Tissue Engineering
Xianyi Meng, Zhen Lin, Shan Cao, Iga Janowska, Koshiro Sonomoto, Darja Andreev, Knab Katharina, Jinming Wen, Karl Xaver Knaup, Michael Sean Wiesener, Gerhard Kroenke, Marta Rizzi, Georg Schett, Aline Bozec
Summary: In the bone marrow, B cells and bone-resorbing osteoclasts form a specific microenvironment. This study found that prolonged HIF-1 alpha signaling in B cells leads to increased RANKL production and osteoclast formation. Deletion of HIF-1 alpha in B cells prevents estrogen deficiency-induced bone loss. The HSP70/HIF-1 alpha axis may serve as a new therapeutic target for osteoporosis.
Article
Immunology
Maria Elena Maccari, Sebastian Fuchs, Patrick Kury, Geoffroy Andrieux, Simon Volkl, Bertram Bengsch, Myriam Ricarda Lorenz, Maximilian Heeg, Jan Rohr, Sabine Jagle, Carla N. Castro, Miriam Gross, Ursula Warthorst, Christoph Koenig, Ilka Fuchs, Carsten Speckmann, Julian Thalhammer, Friedrich G. Kapp, Markus G. Seidel, Gregor Duckers, Stefan Schoenberger, Catharina Schuetz, Marita Fuhrer, Robin Kobbe, Dirk Holzinger, Christian Klemann, Petr Smisek, Stephen Owens, Gerd Horneff, Reinhard Kolb, Nora Naumann-Bartsch, Maurizio Miano, Julian Staniek, Marta Rizzi, Tomas Kalina, Pascal Schneider, Anika Erxleben, Rolf Backofen, Arif Ekici, Charlotte M. Niemeyer, Klaus Warnatz, Bodo Grimbacher, Hermann Eibel, Andreas Mackensen, Andreas Philipp Frei, Klaus Schwarz, Melanie Boerries, Stephan Ehl, Anne Rensing-Ehl
Summary: The identification and characterization of a population of FAS-controlled TCR alpha beta(+) T cells in autoimmune lymphoproliferative syndrome show numerous unique features, including a distinct molecular signature, high proliferation rates, noncytotoxicity, and IL-10 cytokine bias. Mechanistically, the regulation of this population involves FAS and CTLA4 signaling, as well as mTOR and STAT3 signals, and genetic alterations in these pathways can lead to significant lymphoproliferative disease.
JOURNAL OF EXPERIMENTAL MEDICINE
(2021)
Article
Immunology
Nils Venhoff, Jens Thiel, Marta Rizzi, Ana Venhoff, Sebastian Rauer, Dominique Endres, Carolin Hentze, Julian Staniek, Daniela Huzly, Reinhard E. Voll, Ulrich Salzer, Tilman Hottenrott
FRONTIERS IN IMMUNOLOGY
(2019)