Review
Immunology
Hiroto Tsuboi, Seiji Segawa, Mizuki Yagishita, Hirofumi Toko, Fumika Honda, Ayako Kitada, Haruka Miki, Ayako Ohyama, Shinya Hagiwara, Yuya Kondo, Isao Matsumoto, Takayuki Sumida
Summary: Adult onset Still disease (AOSD) is a systemic inflammatory disorder characterized by skin rash, spiking fever, arthritis, sore throat, lymphadenopathy, and hepatosplenomegaly. Both innate and acquired immune responses contribute to the pathogenesis of AOSD, involving hyperactivation of macrophages, neutrophils, Thi and Th17 cells, and low activation of NK cells and regulatory T cells.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Public, Environmental & Occupational Health
Bowen Xu, Jian Wang, Xiaoying Meng, Binghao Bao
Summary: This study analyzed the research hotspots and frontiers in the field of Adult-onset Still Disease (AoSD) using bibliometrics. The research focuses on the diagnosis and differential diagnosis of the disease, and targeted therapy will become a research hotspot in the future. Authors and institutions from France and Italy have a high number of published articles and citations.
FRONTIERS IN PUBLIC HEALTH
(2022)
Review
Biochemistry & Molecular Biology
Chiara Baggio, Sara Bindoli, Irina Guidea, Andrea Doria, Francesca Oliviero, Paolo Sfriso
Summary: IL-18 is a potent pro-inflammatory cytokine involved in various immune processes. It plays a key role in autoinflammatory diseases and may be a useful diagnostic and monitoring tool. Researchers are investigating IL-18 as a therapeutic target for inflammatory diseases.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Rheumatology
Sara Bindoli, Paola Galozzi, Andrea Doria, Paolo Sfriso
Summary: Objective: Adult-onset Still's disease (AOSD) is an auto-inflammatory polygenic disorder, for which clinical diagnosis is essential. Anti-interleukin (IL)-1 therapy is considered a milestone in treating AOSD. This study presents the short-term efficacy of intravenous (IV) anakinra, an IL-1 receptor blocker, in patients with newly-diagnosed AOSD or upcoming macrophage activation syndrome (MAS).
Article
Medicine, General & Internal
Samar O. Alharbi
Summary: This case report describes a patient with AOSD complicated by COVID-19 infection. The patient developed COVID-19 shortly after having AOSD, suggesting a higher risk of COVID-19 in AOSD patients. However, distinguishing between the symptoms of the two conditions was difficult. Therefore, early diagnosis, differentiation, and prompt treatment are crucial.
Review
Immunology
Frederik Staels, Tom Collignon, Albrecht Betrains, Margaux Gerbaux, Mathijs Willemsen, Stephanie Humblet-Baron, Adrian Liston, Steven Vanderschueren, Rik Schrijvers
Summary: Inborn errors of immunity (IEI) are a group of disorders caused by genetic defects affecting the development and function of the immune system. While most of these disorders have a polygenic background, the use of next-generation sequencing has led to the identification of more monogenic causes, allowing for a better understanding of the disease's pathophysiology.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Medicine, General & Internal
Yugo Horiuchi, Kenichi Hashimoto, Hideyuki Horikoshi, Azusa Sano, Yusuke Kawamura, Naoya Fujita, Motohiro Kimata, Yosuke Ono, Yasuhiro Obuchi, Arisa Makino, Mayuko Kaneko, Fumihiko Kimura, Kenji Itoh, Yuji Tanaka
Summary: This article presents a severe case of elderly-onset Still disease (AOSD) with rapid progression, which was effectively treated with a combination of methotrexate (MTX), tocilizumab (TCZ), and prednisolone (PSL). The repeated monitoring of IL-18 levels proved to be useful in diagnosing and treating elderly AOSD.
Article
Medicine, General & Internal
Iftach Sagy, Alona Finkel-Oron, Eviatar Naamany, Leonid Barski, Mahmoud Abu-Shakra, Yair Molad, Shachaf Shiber
Summary: Diagnosing adult-onset Still disease (AOSD) is challenging due to its ambiguous clinical presentation and lack of specific serological markers. This study aimed to evaluate the diagnostic utility of clinical, laboratory, and serum ferritin features in established AOSD patients. The results showed that joint involvement had the highest diagnostic utility, while serum ferritin showed variation and poorer results depending on the chosen cutoff. Clinicians should rely on thorough clinical evaluation rather than solely elevated ferritin levels for the diagnosis of AOSD.
Article
Medicine, Research & Experimental
Larissa Valor-Mendez, Bernhard Manger, Alexander Cavallaro, Stephan Achenbach, Georg Schett, Jurgen Rech
Summary: This case report describes a 68-year-old Caucasian male initially diagnosed with acute endocarditis leading to severe aortic insufficiency, ultimately diagnosed with AOSD. The patient was treated with anakinra, but later developed dilated cardiomyopathy and gouty arthritis. Treatment required dosage adjustments, and the patient eventually passed away due to deteriorating cardiac disease.
EUROPEAN JOURNAL OF MEDICAL RESEARCH
(2021)
Review
Immunology
Long-Fang Chen, Cheng-De Yang, Xiao-Bing Cheng
Summary: Adult-onset immunodeficiency syndrome triggered by anti-interferon (IFN)-gamma autoantibodies has gained attention, with chronic, recurrent infections that respond well to aggressive antibiotic therapy. Anti-type I IFN (IFN-I) autoantibodies have been linked to severe COVID-19, affecting susceptibility and severity of various infectious diseases, including SARS-CoV-2 infection, and potentially contributing to autoimmune disease pathogenesis.
FRONTIERS IN IMMUNOLOGY
(2021)
Review
Clinical Neurology
Gorka Fernandez-Eulate, Christophe Carreau, Jean-Francois Benoist, Foudil Lamari, Benoit Rucheton, Natalia Shor, Yann Nadjar
Summary: Neurometabolic diseases are a group of rare but diverse genetic diseases with a significant impact on pediatric medicine. While many of these diseases are treatable, they often face a diagnostic delay and underdiagnosis due to the complexity of the diseases and the availability of specialized biochemical tests. To assist neurologists in diagnosing and treating these diseases, a simplified diagnostic approach is proposed.
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY
(2022)
Article
Rheumatology
Corrado Campochiaro, Nicola Farina, Alessandro Tomelleri, Giacomo De Luca, Elena Baldissera, Giulio Cavalli, Lorenzo Dagna
Summary: The study assessed drug retention rates and reasons for discontinuation of biologic disease-modifying antirheumatic drugs in patients with adult onset Still's disease. The results showed that TCZ and ANK had similar and significantly higher drug retention rates compared to TNFi, and previous use of biologic agents did not affect drug retention rates.
SEMINARS IN ARTHRITIS AND RHEUMATISM
(2021)
Article
Immunology
Jianfen Meng, Yuning Ma, Jinchao Jia, Mengyan Wang, Jialin Teng, Hui Shi, Honglei Liu, Yutong Su, Junna Ye, Yue Sun, Xiaobing Cheng, Huihui Chi, Tingting Liu, Dehao Zhu, Zhuochao Zhou, Liyan Wan, Zhihong Wang, Fan Wang, Xin Qiao, Xia Chen, Hao Zhang, Zihan Tang, Chengde Yang, Qiongyi Hu
Summary: This study compared the cytokine and ferritin levels between severe COVID-19 and active AOSD patients, revealing significant differences in IL-6, IL-10, and ferritin levels, laying the groundwork for developing therapeutic strategies targeting the hyperinflammatory process in COVID-19.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Rheumatology
Yudong Liu, Shulan Zhang, Chang-sheng Xia, Jiali Chen, Chunhong Fan
Summary: The study found that the levels of G-CSF are closely related to disease activity in AOSD patients, indicating its potential involvement in the pathogenesis of AOSD. Targeting G-CSF may have therapeutic potential for AOSD, and incorporating circulating G-CSF levels into the clinical assessment system could help monitor disease activity.
JOURNAL OF RHEUMATOLOGY
(2021)
Article
Medicine, General & Internal
Roberto Caricchio, Antonio Abbate, Ivan Gordeev, Jamie Meng, Priscilla Y. Hsue, Tuhina Neogi, Roberto Arduino, Daria Fomina, Roman Bogdanov, Tatiana Stepanenko, Pilar Ruiz-Seco, Andres Gonzalez-Garcia, Yu Chen, Yuhan Li, Sarah Whelan, Stephanie Noviello
Summary: This clinical trial compared the efficacy of canakinumab vs placebo on survival without invasive mechanical ventilation (IMV) in hospitalized patients with severe COVID-19. The results showed that treatment with canakinumab did not significantly increase the likelihood of survival without IMV compared to placebo.
JAMA-JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION
(2021)
Review
Biochemistry & Molecular Biology
Leonardo Astudillo, Nicole Therville, Celine Colacios, Bruno Segui, Nathalie Andrieu-Abadie, Thierry Levade
Article
Medicine, General & Internal
Leonardo Astudillo, Frederique Sabourdy, Guy Touati, Thierry Levade
Review
Biochemistry & Molecular Biology
Jerome Stirnemann, Nadia Belmatoug, Fabrice Camou, Christine Serratrice, Roseline Froissart, Catherine Caillaud, Thierry Levade, Leonardo Astudillo, Jacques Serratrice, Anais Brassier, Christian Rose, Thierry Billette de Villemeur, Marc G. Berger
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2017)
Letter
Gastroenterology & Hepatology
Gregory Pugnet, Vincent Soler, Leonardo Astudillo, Vincent Gualino
Letter
Rheumatology
K. Paricaud, G. Pugnet, G. Moulis, P. Arlet, L. Astudillo, L. Sailler
SCANDINAVIAN JOURNAL OF RHEUMATOLOGY
(2017)
Letter
Rheumatology
Sebastien De Almeida Chaves, Guillaume Moulis, Gregory Pugnet, Laurent Sailler, Leonardo Astudillo
Article
Cardiac & Cardiovascular Systems
Sarah Pradel, Stephanie Brun, Gerard Victor, Pierre Pascal, Pauline Fournier, David Ribes, Yoan Lavie-Badie, Michel Galinier, Didier Carrie, Isabelle Berry, Olivier Lairez, Stanislas Faguer, Stanislas Faguer, Pascal Cintas, Laurent Alric, Daniel Adoue, Laurent Sailler, Leonardo Astudillo, Benedicte Puissant, Gregoire Prevot, Dominique Chauveau, Antoine Huart, Magali Colombat, Audrey Delas, Joelle Guitard, Pauline Bernadet Monrozies, Murielle Roussel, Eve Cariou, Delphine Dupin-Deguine
JOURNAL OF NUCLEAR CARDIOLOGY
(2020)
Article
Hematology
M. -L. Piel-Julian, M. Mahevas, J. Germain, L. Languille, T. Comont, M. Lapeyre-Mestre, B. Payrastre, O. Beyne-Rauzy, M. Michel, B. Godeau, D. Adoue, G. Moulis
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2018)
Article
Medicine, General & Internal
L. Astudillo, A. Laure, V. Fabry, G. Pugnet, P. Maury, M. Labrunee, L. Sailler, A. Pavy-Le Traon
REVUE DE MEDECINE INTERNE
(2018)
Letter
Rheumatology
M-L Piel-Julian, G. Moulis, P. Fournier, R. Dupont, D. Geiger, L. Astudillo, T. Faurie, J. Pozzo, A. Petermann, O. Lairez, G. Pugnet, L. Sailler
SCANDINAVIAN JOURNAL OF RHEUMATOLOGY
(2018)
Review
Immunology
Romain Dupont, Melanie Longue, Anne Galinier, Christel Cinq Frais, Cecile Ingueneau, Leonardo Astudillo, Philippe Arlet, Daniel Adoue, Laurent Alric, Gregoire Prevot, Bastien Cabarrou, Laurent Sailler, Gregory Pugnet
AUTOIMMUNITY REVIEWS
(2018)
Article
Immunology
M. Mourguet, D. Chauveau, S. Faguer, J. B. Ruidavets, Y. Bejot, D. Ribes, A. Huart, L. Alric, L. Balardy, L. Astudillo, D. Adoue, L. Sailler, G. Pugnet
JOURNAL OF AUTOIMMUNITY
(2019)
Article
Medicine, General & Internal
M. Garric, X. Boumaza, K. Paricaud, P. Lansalot-Matras, L. Porte, L. Astudillo, G. Pugnet, L. Sailler, G. Moulis
REVUE DE MEDECINE INTERNE
(2019)
Article
Rheumatology
Stella Rousset, Emmanuel Treiner, Guillaume Moulis, Gregory Pugnet, Leonardo Astudillo, Kim Paricaud, Benedicte Puissant-Lubrano, Philippe Arlet, Antoine Blancher, Laurent Sailler
Article
Cardiac & Cardiovascular Systems
Stephanie Brun, Eve Cariou, Pauline Fournier, David Ribes, Stanislas Faguer, Antoine Huart, Didier Carrie, Michel Galinier, Olivier Lairez