Article
Rheumatology
D. Waki, K. Nishimura, T. Yoshida, N. Tanaka, K. Mizukawa, M. Fukushima, O. Iri, M. Anegawa, T. Yokota
Summary: The study found that lower doses of TMP/SMX have a protective effect against early severe infections in Japanese patients with AAV, even with the occurrence of side effects leading to discontinuation in some patients. Further research is needed to determine the optimal dose of TMP/SMX to prevent severe infections, especially in patients with renal impairment.
CLINICAL AND EXPERIMENTAL RHEUMATOLOGY
(2021)
Article
Endocrinology & Metabolism
Xin Chen, Shuo Zhang, Xia Peng, Xiaoxiao Shi, Haiting Wu, Yubing Wen, Yan Qin, Xinping Tian, Huijuan Zhu, Limeng Chen
Summary: This study investigated the clinical characteristics and prognosis of patients with anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) presenting as central diabetes insipidus (CDI). The results showed that AAV patients with CDI had more ear, nose, and throat involvement and less renal impairment compared to the control group. Patients in Asian countries tended to be older men and have higher MPO-ANCA positivity, and PR3-ANCA positivity may predict disease recurrence.
FRONTIERS IN ENDOCRINOLOGY
(2023)
Article
Rheumatology
Eduardo Martin-Nares, Gabriela Hernandez-Molina
Summary: In patients with IgG4-related disease, a significant number of patients have ANCA positivity, and these patients differ from the ANCA-negative group in terms of clinical and serological features.
Article
Medicine, General & Internal
Jang Woo Ha, Yong-Beom Park, Sang-Won Lee
Summary: This study demonstrates, for the first time, that 83.3% of ANCA-positive patients with PM/DM could be diagnosed with overlap syndrome consisting of PM/DM and AAV according to the 2022 ACR/EULAR criteria for AAV.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Urology & Nephrology
Ebru Dirikgil, Jacqueline T. Jonker, Sander W. Tas, Cornelis A. Verburgh, Darius Soonawala, A. Elisabeth Hak, Hilde H. F. Remmelts, Daphne IJpelaar, Gozewijn D. Laverman, Abraham Rutgers, Jacob M. van Laar, Hein J. Bernelot Moen, Peter M. J. Verhoeven, Ton J. Rabelink, Willem Jan W. Bos, Y. K. Onno Teng
Summary: The study investigated the real-life management and outcomes of AAV patients in the Netherlands, revealing oral cyclophosphamide as the most frequently used induction therapy, azathioprine for maintenance therapy, and an increasing use of rituximab over time. Major infection and relapses were found to be the most prevalent adverse outcomes. This audit provided important indicators for the treatment of AAV patients that can be implemented in future national audits to improve patient outcomes.
KIDNEY INTERNATIONAL REPORTS
(2021)
Article
Medicine, General & Internal
Jang-Woo Ha, Sung-Soo Ahn, Jason-Jungsik Song, Yong-Beom Park, Sang-Won Lee
Summary: This study investigates the prognostic value of total globulin fraction (TGF) at diagnosis on all-cause mortality in patients with ANCA-associated vasculitis (AAV). The results show that TGF at AAV diagnosis is significantly correlated with ANCA positivity and patients with TGF ≥ 3.1 g/dL have a lower cumulative survival rate. In the multivariable analysis, TGF ≥ 3.1 g/dL is independently associated with all-cause mortality, along with age, male sex, and body mass index.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Medicine, General & Internal
Tae-Geom Lee, Pil-Gyu Park, Yong-Beom Park, Ji-Hye Huh, Sang-Won Lee
Summary: This study investigated whether the BARD score at diagnosis could predict all-cause mortality in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV). The study found that among AAV patients, a BARD score ≥ 3 could predict all-cause mortality during follow-up.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Urology & Nephrology
Hyo Jin Kim, Miyeun Han, Sang Heon Song, Eun Young Seong
Summary: This study investigated the clinical characteristics and outcomes of patients aged >= 65 years with ANCA-positive AAV in Korea. The results showed that patients in the elderly group had higher mortality rates, and older age, lower hemoglobin, higher serum creatinine level, and nontreatment were associated with mortality compared to oral CYC + steroids.
KIDNEY RESEARCH AND CLINICAL PRACTICE
(2022)
Article
Medicine, General & Internal
Minyoung Kevin Kim, Hyeok Chan Kwon, Jason Jungsik Song, Yong-Beom Park, Sang-Won Lee
Summary: The presence of ANCA positivity in patients with Behcet's disease may be associated with vascular involvement, particularly in the upper extremities and visceral arteries, at the time of diagnosis, but it does not predict poor outcomes during follow-up.
YONSEI MEDICAL JOURNAL
(2021)
Review
Immunology
Yuji Nozaki
Summary: Biologics targeting inflammation-related molecules have revolutionized the treatment of rheumatoid arthritis and ANCA-associated vasculitis. However, challenges remain in treating vasculitis effectively. New drugs in clinical trials offer promising options for vasculitis treatment.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Medicine, General & Internal
Lucy Eunju Lee, Wooyong Jeong, Yong-Beom Park, Su Jin Jeong, Sang-Won Lee
Summary: This study investigates the rate of ANCA positivity in patients infected with SARS-CoV-2 and its clinical significance. The results showed that SARS-CoV-2 infection may increase the rate of ANCA positivity, but it does not affect poor outcomes. ANCA positivity may contribute to the classification of granulomatosis with polyangiitis and microscopic polyangiitis.
JOURNAL OF CLINICAL MEDICINE
(2022)
Article
Immunology
Sha-sha Chen, Hao-jun Luo, Huan Li, Hong Zhang, Yuan-yuan Li, Yi Li, Wei Wang, Han-lu Ding
Summary: This study found that plasma FXII levels are positively correlated with disease activity and histologic activity in AAV, and may serve as markers for active AAV.
INTERNATIONAL IMMUNOPHARMACOLOGY
(2023)
Article
Biochemistry & Molecular Biology
Yan Qin, Yanlin Wang, Yanyao Wu, Min Feng, Xiangcong Zhao, Chong Gao, Hui Guo, Jing Luo
Summary: DN T cells, a subset of T lymphocytes producing inflammatory cytokines, are significantly elevated in patients with AAV, suggesting their potential role in the pathogenesis of autoimmune disorders like AAV. Additional investigations are needed to further understand the precise function of DN T cells in AAV patients.
MOLECULAR IMMUNOLOGY
(2021)
Review
Immunology
Menglu Pan, Huanhuan Zhao, Ruimin Jin, Patrick S. C. Leung, Zongwen Shuai
Summary: This article summarizes the importance of immune checkpoints in the pathogenesis of anti-neutrophil cytoplasmic autoantibodies associated vasculitis (AAV), as well as the potential role of immune checkpoint inhibitors (ICIs) in AAV treatment.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Medical Laboratory Technology
Taejun Yoon, Lucy Eunju Lee, Sung Soo Ahn, Jung Yoon Pyo, Jason Jungsik Song, Yong-Beom Park, Sang-Won Lee
Summary: This study found that serum progranulin is closely associated with disease activity of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), and higher levels of serum progranulin may indicate higher risk of AAV disease activity.
JOURNAL OF CLINICAL LABORATORY ANALYSIS
(2021)
Review
Rheumatology
Bernhard Hellmich, Ana Agueda, Sara Monti, Frank Buttgereit, Hubert de Boysson, Elisabeth Brouwer, Rebecca Cassie, Maria C. Cid, Bhaskar Dasgupta, Christian Dejaco, Gulen Hatemi, Nicole Hollinger, Alfred Mahr, Susan P. Mollan, Chetan Mukhtyar, Cristina Ponte, Carlo Salvarani, Rajappa Sivakumar, Xinping Tian, Gunnar Tomasson, Carl Turesson, Wolfgang Schmidt, Peter M. Villiger, Richard Watts, Chris Young, Raashid Ahmed Luqmani
ANNALS OF THE RHEUMATIC DISEASES
(2020)
Article
Rheumatology
Alfred Mahr, Manon Belhassen, Marc Paccalin, Valerie Devauchelle-Pensec, Maeva Nolin, Sophie Gandon, Isabelle Idier, Eric Hachulla
Article
Dermatology
G. Chaby, C. Maldini, C. Haddad, B. Lebrun-Vignes, F. Hemery, S. Ingen-Housz-Oro, S. Gonzalez-Chiappe, P. Wolkenstein, O. Chosidow, A. Mahr, L. Fardet
BRITISH JOURNAL OF DERMATOLOGY
(2020)
Review
Rheumatology
Emma Rubenstein, Carla Maldini, Solange Gonzalez-Chiappe, Sylvie Chevret, Alfred Mahr
Article
Rheumatology
Sarah L. Mackie, Christian Dejaco, Simone Appenzeller, Dario Camellino, Christina Duftner, Solange Gonzalez-Chiappe, Alfred Mahr, Chetan Mukhtyar, Gary Reynolds, Alexandre Wagner S. de Souza, Elisabeth Brouwer, Marwan Bukhari, Frank Buttgereit, Dorothy Byrne, Maria C. Cid, Marco Cimmino, Haner Direskeneli, Kate Gilbert, Tanaz A. Kermani, Asad Khan, Peter Lanyon, Raashid Luqmani, Christian Mallen, Justin C. Mason, Eric L. Matteson, Peter A. Merkel, Susan Mollan, Lorna Neill, Eoin O' Sullivan, Maria Sandovici, Wolfgang A. Schmidt, Richard Watts, Madeline Whitlock, Elaine Yacyshyn, Steven Ytterberg, Bhaskar Dasgupta
Article
Rheumatology
Sarah L. Mackie, Christian Dejaco, Simone Appenzeller, Dario Camellino, Christina Duftner, Solange Gonzalez-Chiappe, Alfred Mahr, Chetan Mukhtyar, Gary Reynolds, Alexandre Wagner S. de Souza, Elisabeth Brouwer, Marwan Bukhari, Frank Buttgereit, Dorothy Byrne, Maria C. Cid, Marco Cimmino, Haner Direskeneli, Kate Gilbert, Tanaz A. Kermani, Asad Khan, Peter Lanyon, Raashid Luqmani, Christian Mallen, Justin C. Mason, Eric L. Matteson, Peter A. Merkel, Susan Mollan, Lorna Neill, Eoin O' Sullivan, Maria Sandovici, Wolfgang A. Schmidt, Richard Watts, Madeline Whitlock, Elaine Yacyshyn, Steven Ytterberg, Bhaskar Dasgupta
Letter
Rheumatology
Alfred Mahr, Ulrich Specks, David Jayne
Article
Rheumatology
Gulen Hatemi, Alexa Meara, Yesim Ozguler, Haner Direskeneli, Alfred Mahr, Beverly Shea, Esen Cam, Ahmet Gul, Yusuf Yazici, Peter Tugwell, Hasan Yazici, Peter A. Merkel
Summary: There is a need for reliable and widely-accepted outcome measures in randomized clinical trials for Behcet's syndrome. The Outcome Measures in Rheumatology (OMERACT) Behcet's Syndrome Working Group developed a core set of data-driven outcome measures through a comprehensive, iterative, and multistage project. The final core set includes 5 mandatory domains for all trials in Behcet's syndrome, as well as additional subdomains for specific organ-systems. The core set provides a foundation for harmonizing research and advancing effective therapies.
ARTHRITIS CARE & RESEARCH
(2022)
Article
Medicine, General & Internal
Alfred Mahr, Eric Hachulla, Hubert de Boysson, Nassim Guerroui, Emmanuel Heron, Stephane Vinzio, Jonathan Broner, Francois-Xavier Lapebie, Martin Michaud, Laurent Sailler, Thierry Zenone, Mohamed Djerad, Mathieu Jouvray, Emilie Shipley, Nathalie Tieulie, Guillaume Armengol, Bastien Bouldoires, Jean-Francois Viallard, Isabelle Idier, Marc Paccalin, Valerie Devauchelle-Pensec
Summary: This French study describes the characteristics, diagnosis, treatment, and management of giant cell arteritis (GCA) in real-life settings. The results show that most patients receive glucocorticoid therapy, with adjunct medications, relapses, and comorbidities related to treatment being common issues.
FRONTIERS IN MEDICINE
(2021)
Review
Medicine, General & Internal
Yingqi Xiao, Gordon Guyatt, Linan Zeng, David RW Jayne, Peter A. Merkel, Reed AC Siemieniuk, Jared E. Dookie, Tayler A. Buchan, Muhammad Muneeb Ahmed, Rachel J. Couban, Alfred Mahr, Michael Walsh
Summary: The study compares the efficacy and safety of different glucocorticoid regimens as induction therapy for patients with ANCA-associated vasculitis. The reduced-dose regimen of glucocorticoids may reduce the risk of death and serious infections without increasing end-stage kidney disease.
Editorial Material
Medicine, General & Internal
Linan Zeng, Michael Walsh, Gordon H. Guyatt, Reed A. C. Siemieniuk, David Collister, Michelle Booth, Paul Brown, Lesha Farrar, Mark Farrar, Tracy Firth, Lynn A. Fussner, Karin Kilian, Mark A. Little, Thomas A. Mavrakanas, Reem A. Mustafa, Maryam Piram, Lisa K. Stamp, Yingqi Xiao, Lyubov Lytvyn, Thomas Agoritsas, Per O. Vandvik, Alfred Mahr
Summary: The guideline recommends against plasma exchange in low or low-moderate risk patients, and in favor of plasma exchange in moderate-high or high risk patients. For patients with pulmonary hemorrhage, plasma exchange is not suggested. The panel strongly recommends using a reduced dose regimen of glucocorticoids in the first six months of therapy, instead of the standard dose regimen.
BMJ-BRITISH MEDICAL JOURNAL
(2022)
Article
Rheumatology
Emma Rubenstein, Pierrick Henneton, Sophie Riviere, Marie-Laure Casanova, Jonathan Broner, Erik Arnaud, Eric Oziol, Moglie Le Quintrec, Olivier Moranne, Christian Jorgensen, Bernard Combe, Arnaud Bourdin, Christophe Fontaine, Aurelie Schiffmann, Jean-Baptiste Fraison, Olivier Halle, Thibaut Fraisse, Frederic Veysseyre, Guillaume Taieb, Cecile Aerts, Louis Crampette, Caroline Alovisetti, Laurence Guis, Souad Mehlal, Laurence Papinaud, Alain Le Quellec, Philippe Guilpain, Alfred Mahr
Summary: This study aimed to estimate the prevalence of ANCA-associated vasculitis in Southern France in 2018 and evaluate differences among Europeans and non-Europeans. The results showed a higher prevalence of MPA in non-European residents.
Article
Rheumatology
Solange Gonzalez Chiappe, Sarah Lechtman, Carla Soledad Maldini, Arsene Mekinian, Thomas Papo, Thomas Sene, Alfred Daniel Mahr
Summary: This population-based study estimated the incidence of giant cell arteritis (GCA) in northeastern Paris and found that the incidence increased with age, with higher rates in women. Most cases presented with cranial symptoms, consistent with previous studies in Europe and France.
RHEUMATOLOGY INTERNATIONAL
(2022)
Review
Rheumatology
Sara Monti, Ana F. Agueda, Raashid Ahmed Luqmani, Frank Buttgereit, Maria Cid, Christian Dejaco, Alfred Mahr, Cristina Ponte, Carlo Salvarani, Wolfgang Schmidt, Bernhard Hellmich
Review
Rheumatology
Raymond Chu, Caylea Foster, Mohsin Ali, Todd Chaba, Alison H. Clifford, Alfred Mahr, Jason Soo, Jan Willem Cohen Tervaert, Elaine Yacyshyn
LANCET RHEUMATOLOGY
(2020)
