Article
Urology & Nephrology
Fernanda Florez-Barros, Siobhan Bearder, Bengt Kull, Adrian Freeman, Attila Mocsai, Michael G. Robson
Summary: This study found that myeloid-specific Mcl1 deletion protected against murine anti-myeloperoxidase vasculitis, but myeloperoxidase inhibition did not provide protection.
KIDNEY INTERNATIONAL
(2022)
Article
Urology & Nephrology
Cong-Rong Shen, Xiao-Yu Jia, Wentian Luo, Florina Olaru, Zhao Cui, Ming-Hui Zhao, Dorin-Bogdan Borza
Summary: In patients with anti-GBM antibody GN, circulating IgG autoantibodies binding to laminin-521 were found in about one third of patients, but not in healthy controls or patients with other glomerular diseases. Autoreactivity towards laminin-521 was significantly more common in patients with anti-GBM GN and lung hemorrhage than in patients with kidney-limited disease.
JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY
(2021)
Article
Immunology
Xueling Hu, Chanjuan Shen, Ting Meng, Joshua D. Ooi, Peter J. Eggenhuizen, Ya-ou Zhou, Hui Luo, Jin-biao Chen, Wei Lin, Yizi Gong, Qi Xiong, Jia Xu, Ning Liu, Xiangcheng Xiao, Rong Tang, Yong Zhong
Summary: The study found that DPPs tended to involve multi-organ damage rather than being limited to the kidney, compared to anti-GBM disease. The clinical and pathological characteristics of DPPs differed from those of MPO-AAV and anti-GBM disease patients. DPPs had a worse prognosis compared to MPO-AAV and anti-GBM disease patients.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Urology & Nephrology
Adel Molnar, Andras Tisler, Dejan Dobi, Akos Petho
Summary: This case report is the first to document the co-occurrence of anti-GBM disease and anti-PLA2R positive membranous nephropathy, confirmed by both serologic and histologic methods. Microbial kidney injury can trigger multiple autoimmune diseases, and the simultaneous occurrence of anti-GBM disease and membranous nephropathy is extremely rare. Delayed recognition and treatment can result in worse renal and patient outcomes, as well as increased financial costs.
Article
Biochemistry & Molecular Biology
Fuye Yang, Jiaoyi Chen, Xiao Ru Huang, Wai Han Yiu, Xueqing Yu, Sydney C. W. Tang, Hui Yao Lan
Summary: The study found that deletion of TLR4 from myeloid cells can suppress renal injury caused by anti-GBM GN by regulating immune cell types and related signaling pathways.
CELLULAR AND MOLECULAR LIFE SCIENCES
(2021)
Article
Medicine, General & Internal
Camille Le Moine, Noemie Jourde-Chiche, Delphine Kemlin, Benjamin Terrier, Frederic Vandergheynst
Summary: In cases of AAV with kidney involvement, physicians should investigate anti-GBM antibodies and be vigilant of the potential sequential development of AAV, especially with MPO-ANCA and anti-GBM glomerulonephritis. This sequential disease progression is linked to poor renal outcomes, underscoring the importance of prompt diagnosis and management.
CLINICAL CASE REPORTS
(2021)
Article
Rheumatology
Maliha A. Alikhan, Juli Jaw, Lani R. Shochet, Kate J. Robson, Joshua D. Ooi, Elisabeth Brouwer, Peter Heeringa, Stephen R. Holdsworth, A. Richard Kitching
Summary: The study found that cellular immunity to MPO increases with age in mice, which may contribute to the increased incidence and severity of AAV in older individuals.
Article
Urology & Nephrology
Charles Ronsin, Marie Georges, Agnes Chapelet-Debout, Jean-Francois Augusto, Vincent Audard, Ludivine Lebourg, Sebastien Rubin, Thomas Quemeneur, Pierre Bataille, Alexandre Karras, Eric Daugas, Dimitri Titeca-Beauport, Jean-Jacques Boffa, Cecile Vigneau, Jean-Michel Halimi, Corinne Isnard-Bagnis, Sandrine Durault, Eric Renaudineau, Frank Bridoux, Angelo Testa, Moglie Le Quintrec, Karine Renaudin, Fadi Fakhouri
Summary: Within the spectrum of ANCA-negative PING, infection and malignancy-associated forms represent distinct clinical subsets. Despite immunosuppressive treatments, ANCA-negative PING remains a severe form of vasculitis with high morbidity and mortality rates.
AMERICAN JOURNAL OF KIDNEY DISEASES
(2022)
Article
Urology & Nephrology
Huang Kuang, Cong-rong Shen, Xiao-yu Jia, Meng Tan, Xue-fen Yang, Zhao Cui, Dorin-Bogdan Borza, Ming-hui Zhao
Summary: Anti-glomerular basement membrane (anti-GBM) disease is an autoimmune disorder characterized by autoantibodies against GBM components. This study suggests that autoantibodies against laminin-521 may play an independent pathogenic role in the development of anti-GBM disease, as supported by clinical and animal studies.
KIDNEY INTERNATIONAL
(2023)
Review
Dentistry, Oral Surgery & Medicine
Waleed A. Alamoudi, Thomas P. Sollecito, Eric T. Stoopler, Katherine France
Summary: Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) is a multisystem disorder of small blood vessels, which can be classified into granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). Oral manifestations (OMs) in AAVs mainly include mucosal ulceration, gingival enlargement, alveolar bone necrosis, tooth loss, oro-antral communication, palatal perforation, parotitis, and candidal infection, with GPA being the most common subtype. These OMs can be nonspecific and resemble various other conditions, making differential diagnosis challenging. This review not only updates the OMs of GPA but also includes OMs of other AAVs for the first time. It provides recommendations for overall assessment, diagnosis, and management of all AAV OMs, emphasizing the role of oral health care providers in multidisciplinary care.
ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY ORAL RADIOLOGY
(2023)
Article
Urology & Nephrology
Giorgio Trivioli, Seerapani Gopaluni, Maria L. Urban, Davide Gianfreda, Matthias A. Cassia, Paolo G. Vercelloni, Marta Calatroni, Alessandra Bettiol, Pasquale Esposito, Corrado Murtas, Federico Alberici, Federica Maritati, Lucio Manenti, Alessandra Palmisano, Giacomo Emmi, Paola Romagnani, Gabriella Moroni, Gina Gregorini, Renato A. Sinico, David R. W. Jayne, Augusto Vaglio
Summary: AAV can present with slow renal disease progression; this subset is characterized by advanced age at diagnosis, positive MPO-ANCA, subclinical interstitial lung lesions, and chronic damage at kidney biopsy. Partial renal recovery may occur following immunosuppression.
CLINICAL KIDNEY JOURNAL
(2021)
Article
Immunology
Mi Tao, Yiqing He, Lijuan Li, Yuyan Li, Wenwen Liao, Haihang Nie, Ping Gao
Summary: This study investigated the association between NETosis and ANCA-associated Glomerulonephritis (ANCA-GN). Differential gene expression analysis and machine learning algorithms were employed to identify NETosis-related genes (NRGs) and develop a NETosisScore model for predicting high-risk patients. Enrichment analysis, immune cell infiltration analysis, and correlation analysis revealed the involvement of immune pathways and the potential biomarker roles of NRGs in ANCA-GN.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Medicine, General & Internal
Fu Shaojie, Su Sensen, Huang Jingda, Wang Luyu, Zhang Fei, Yu Jinyu, Xu Zhonggao, Wu Hao
Summary: The causal relationship between anti-GBM disease and IgA nephropathy remains unclear. This case report presents a rare case of concurrent anti-GBM disease and IgA nephropathy with a good prognosis achieved through early application of corticosteroids and cyclophosphamide in combination with plasmapheresis.
Article
Immunology
Desiree Tampe, Peter Korsten, Philipp Stroebel, Samy Hakroush, Bjoern Tampe
Summary: This study systematically analyzed sex differences in patients with AAV and biopsy-proven ANCA GN, finding that female patients presented at an older age with more joint involvement and exhibited specific sex differences in laboratory parameters and histopathological findings. There were fewer associations between clinical, laboratory parameters, and histopathological findings in females compared to males, indicating the importance of considering sex differences in AAV, particularly ANCA GN.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Chemistry, Multidisciplinary
Jiaoyi Chen, Xiao Ru Huang, Fuye Yang, Wai Han Yiu, Xueqing Yu, Sydney C. W. Tang, Hui Yao Lan
Summary: This study reveals the critical role of myeloid-specific TLR4 in macrophage diversity regulation in anti-GBM cGN. Deletion of myeloid-TLR4 induces a novel reparative macrophage phenotype with upregulated anti-inflammatory and tissue repair-related signaling, suppressing M1 proinflammatory responses. The activation of the Nr4a1/Ear2-axis is identified as a key mechanism promoting anti-inflammatory macrophage differentiation.
Article
Transplantation
Yunan Zhou, Matthias Hellberg, Thomas Hellmark, Peter Hoglund, Naomi Clyne
Summary: Exercise training appears to be effective in preventing sarcopenia and maintaining muscle mass in non-dialysis-dependent patients with chronic kidney disease, with a significant increase in muscle mass in the balance group. However, further research is needed to understand the role of plasma myostatin on muscle mass and physical performance in CKD patients.
NEPHROLOGY DIALYSIS TRANSPLANTATION
(2021)
Article
Biochemical Research Methods
Mattias Ohlsson, Thomas Hellmark, Anders A. Bengtsson, Elke Theander, Carl Turesson, Cecilia Klint, Christer Wingren, Anna Isinger Ekstrand
Summary: This study successfully identified candidate biomarker signatures for differential classification of four systemic IRDs through protein expression profiling, with high accuracy in classifying individual IRDs and separating IRDs from healthy controls. The use of multiplexed affinity-based technologies reflects the biological complexity of autoimmune diseases and is essential for future diagnostic purposes.
JOURNAL OF PROTEOME RESEARCH
(2021)
Article
Hematology
M. Mortzell Henriksson, M. Weiner, W. Sperker, G. Berlin, M. Segelmark, A. Javier Martinez, J. Audzijoniene, A. Griskevicius, E. Newman, M. Blaha, H. Vrielink, V Witt, B. Stegmayr
Summary: The study found that therapeutic apheresis (TA) has a certain effect on improving renal function in patients with antibody-associated vasculitis, especially in patients with PR3-vasculitis, which is more effective and helpful to get out of dialysis.
TRANSFUSION AND APHERESIS SCIENCE
(2021)
Article
Immunology
Julia Petersson, Sandra Askman, Asa Pettersson, Stina Wichert, Thomas Hellmark, Asa C. M. Johansson, Markus Hansson
Summary: The study showed that NDGs from bone marrow of multiple myeloma patients suppress T-cell responses like MDSCs, while blood NDGs from both patients and healthy individuals can inhibit T-cell proliferation and IFN-gamma production. The inhibitory effect of NDGs is dependent on reactive oxygen species production and contact with activated T-cells, indicating the importance of their role in regulating T-cells. Bone marrow NDGs from MM patients exhibit MDSC-like function, which could play a significant role in promoting malignant plasma cell growth in MM patients.
JOURNAL OF IMMUNOLOGY RESEARCH
(2021)
Article
Immunology
Wael Bahnan, Sebastian Wrighton, Martin Sundwall, Anna Blackberg, Olivia Larsson, Urban Hoglund, Hamed Khakzad, Magdalena Godzwon, Maria Walle, Elisabeth Elder, Anna Soderlund Strand, Lotta Happonen, Oscar Andre, Johannes Kumra Ahnlide, Thomas Hellmark, Vidar Wendel-Hansen, Robert PA. Wallin, Johan Malmstom, Lars Malmstrom, Mats Ohlin, Magnus Rasmussen, Pontus Nordenfelt
Summary: The concentration of Spike-specific antibodies can enhance or reduce Spike-bead phagocytosis by monocytes, independent of antibody neutralization potential. The levels of anti-Spike antibodies correlate with the outcome in an experimental SARS-CoV-2 infection model, suggesting that non-neutralizing antibodies could confer protection to SARS-CoV-2 infection by mediating phagocytosis.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Rheumatology
Jens Rathmann, Pavlos Stamatis, Goran Jonsson, Martin Englund, Marten Segelmark, David Jayne, Aladdin J. Mohammad
Summary: Respiratory tract infections are positively associated with the development of MPO-ANCA vasculitis, but not with PR3-ANCA vasculitis. Prior infection is associated with higher disease activity at the time of AAV diagnosis, but does not have a significant impact on disease characteristics, comorbidities or outcome.
Letter
Urology & Nephrology
Marten Segelmark, Christian Kjellman
JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY
(2022)
Article
Immunology
Evelina Elmer, Per Nived, Asa Pettersson, Lillemor Skattum, Thomas Hellmark, Meliha C. Kapetanovic, Asa C. M. Johansson
Summary: This study investigates the role of the innate immune system in the impaired antibody response to PCV vaccination in RA patients treated with MTX. The study found that MTX suppresses monocyte concentration and frequency, which could serve as a biomarker to identify nonresponders to PCV vaccination.
JOURNAL OF IMMUNOLOGY RESEARCH
(2022)
Article
Medicine, Research & Experimental
Wael Bahnan, Lotta Happonen, Hamed Khakzad, Vibha Kumra Ahnlide, Therese de Neergaard, Sebastian Wrighton, Oscar Andre, Eleni Bratanis, Di Tang, Thomas Hellmark, Lars Bjorck, Oonagh Shannon, Lars Malmstrom, Johan Malmstrom, Pontus Nordenfelt
Summary: Group A streptococci have developed strategies to avoid human antibodies, complicating the development of effective vaccines. We have generated antibodies from the B cells of an individual successfully clearing a streptococcal infection. These antibodies bind to the central region of the M protein and promote immune functions. The concept of dual-Fab cis binding allows access to normally non-opsonic regions for antibody targeting.
EMBO MOLECULAR MEDICINE
(2023)
Article
Immunology
Julia Westerlund, Sandra Askman, Asa Pettersson, Thomas Hellmark, Asa C. M. Johansson, Markus Hansson
Summary: This study found that normal density granulocytes (NDGs) do not preferentially suppress Th1 cells, but generally suppress Th cells and cytotoxic T-cells, while specifically downregulating the Th1 marker CD183.
JOURNAL OF IMMUNOLOGY RESEARCH
(2022)
Article
Transplantation
Julia Wijkstrom, Aurora Caldinelli, Annette Bruchfeld, Alexandra Nowak, Angelica Artborg, Maria Stendahl, Marten Segelmark, Bengt Lindholm, Rino Bellocco, Helena Rydell, Marie Evans
Summary: Kidney replacement therapy (KRT) patients in Sweden, who were prioritized for vaccination, experienced reduced hospitalization and mortality rates after vaccination, while kidney transplant recipients (KTRs) did not see the same reduction.
NEPHROLOGY DIALYSIS TRANSPLANTATION
(2023)
Article
Transplantation
Linnea Tyrberg, Fanny Andersson, Fredrik Uhlin, Thomas Hellmark, Marten Segelmark
Summary: This study found that the characteristics of anti-GBM antibodies have an important impact on treatment outcomes in glomerulonephritis patients. The rebound of anti-GBM antibodies during treatment is associated with a worse prognosis, especially antibodies directed against the E-B epitope. Therefore, all means should be used to eliminate anti-GBM antibodies.
NEPHROLOGY DIALYSIS TRANSPLANTATION
(2023)
Article
Rheumatology
Jens Rathmann, Marten Segelmark, Aladdin J. Mohammad
Summary: The ACR/EULAR 2022 criteria for ANCA-associated vasculitides (AAV) classification were evaluated and compared with the European Medicines Agency (EMA) algorithm and classification based only on ANCA serology. The results showed that the EMA and ACR/EULAR classification gave similar results, but a small proportion of patients could not be classified or fell into two categories. The ANCA-based classification showed high agreement with ACR/EULAR criteria.
Article
Infectious Diseases
Marta Tobijaszewska, Giedre Martus, Torgny Sunnerhagen, Marten Segelmark, Oskar Ljungquist
Summary: This study investigated the incidence, trend, etiology, and antimicrobial susceptibility of PD-associated peritonitis and catheter-related infections in South Sweden between 2011-2020 among PD patients. The study found a stable incidence rate of PD-peritonitis, with gram-positive bacteria being the most common cause and limited antibiotic resistance. Monitoring the etiology, incidence, and resistance rates of PD-associated infections is important for empirical antibiotic regimens and prevention.
INFECTIOUS DISEASES
(2023)
Article
Immunology
Shane Kelly, Katherine J. L. Jackson, Timothy J. Peters, Dan Suan, Christopher C. Goodnow
Summary: This study successfully identified and characterized PR3-specific B cells from the peripheral blood of patients with PR3 autoantibodies. These cells exhibited specific immunological features, suggesting that PR3 self-reactivity may occur early in B-cell development.
JOURNAL OF AUTOIMMUNITY
(2024)
Article
Immunology
Ana Merino-Vico, Jan Piet van Hamburg, Paul Tuijnenburg, Giulia Frazzei, Aram Al-Soudi, Carlo G. Bonasia, Boy Helder, Abraham Rutgers, Wayel H. Abdulahad, Coen A. Stegeman, Jan-Stephan Sanders, Laura Bergamaschi, Paul A. Lyons, Theo Bijma, Laura van Keep, Kirsten Wesenhagen, Aldo Jongejan, Henric Olsson, Niek de Vries, Taco W. Kuijpers, Peter Heeringa, Sander W. Tas
Summary: B lineage cells play a critical role in ANCA-associated vasculitis (AAV), and the transcription factor NF-kappa B may be a potential therapeutic target for AAV and other autoimmune diseases with prominent B cell involvement.
JOURNAL OF AUTOIMMUNITY
(2024)
Article
Immunology
Christopher Nelke, Thomas Muentefering, Derya Cengiz, Lukas Theissen, Vera Dobelmann, Christina B. Schroeter, Helena Block, Corinna Preu, Alexander P. E. Michels, Stefanie Lichtenberg, Marc Pawlitzki, Steffen Pfeuffer, Niklas Huntemann, Alexander Zarbock, Thorben Briese, Christoph Kittl, Carsten Dittmayer, Thomas Budde, Ingrid E. Lundberg, Werner Stenzel, Sven G. Meuth, Tobias Ruck
Summary: K2P2.1 plays a regulatory role in the autoimmune response of idiopathic inflammatory myopathies (IIMs), by regulating inflammatory cell response, adhesion, and transmigration in both endothelial and skeletal muscle cells. Inhibiting K2P2.1 enhances the inflammatory response, while activating K2P2.1 improves the disease course.
JOURNAL OF AUTOIMMUNITY
(2024)
Article
Immunology
Xuan Zhang, Jun Xia, Ying Jiang, David S. Pisetsky, Josef S. Smolen, Rong Mu, Shengming Dai, Michael E. Weinblatt, Tore K. Kvien, Juan Li, Thomas Doerner, Yu Zhang, Liwei Lu, Chengde Yang, Pingting Yang, Yuan Zhang, Chenchen Xu, Zhan Zhao, Peter E. Lipsky
Summary: The study suggests that TwHF may be as effective as MTX in treating active RA, and combination therapy may be more effective than monotherapy.
JOURNAL OF AUTOIMMUNITY
(2024)
Article
Immunology
Maya F. Amjadi, Maxwell H. Parker, Ryan R. Adyniec, Zihao Zheng, Alex M. Robbins, S. Janna Bashar, Michael F. Denny, Sara S. Mccoy, Irene M. Ong, Miriam A. Shelef
Summary: Rheumatoid factors (RFs) are polyreactive antibodies that can bind disease-specific epitopes. Recent studies have found that RFs in COVID-19 can bind novel IgG epitopes, which provides new insights into the mechanism of RFs.
JOURNAL OF AUTOIMMUNITY
(2024)
Article
Immunology
Johanne Liberatore, Yann Nguyen, Jerome Hadjadj, Pascal Cohen, Luc Mouthon, Xavier Puechal, Loic Guillevin, Benjamin Terrier
Summary: B-cell depletion induced by rituximab (RTX) in ANCA-associated vasculitis (AAV) can lead to decreased gammaglobulin levels, which is associated with an increased risk of relapse and severe infections. Older age, low gammaglobulin levels, and receiving pulses of methylprednisolone at induction therapy are risk factors for gammaglobulin decline.
JOURNAL OF AUTOIMMUNITY
(2024)