Article
Rheumatology
Xavier Puechal, Michele Iudici, Christian Pagnoux, Pascal Cohen, Mohamed Hamidou, Achille Aouba, Francois Lifermann, Marc Ruivard, Olivier Aumaitre, Bernard Bonnotte, Francois Maurier, Thomas Le Gallou, Eric Hachulla, Alexandre Karras, Chahera Khouatra, Noemie Jourde-Chiche, Jean-Francois Viallard, Claire Blanchard-Delaunay, Pascal Godmer, Alain Le Quellec, Thomas Quemeneur, Claire de Moreuil, Luc Mouthon, Benjamin Terrier, Loic Guillevin
Summary: This study compared patients with ANCA-negative, MPO-ANCA-positive, and PR3-ANCA-positive GPA, and found that ANCA-negative patients had more limited disease and less kidney involvement compared to ANCA-positive patients. MPO-ANCA-positive patients were more often female, older, and had more kidney involvement compared to PR3-ANCA-positive patients. PR3-ANCA-positive patients had lower relapse-free survival compared to the other groups, but this difference was not seen when comparing with MPO-ANCA-positive patients.
Article
Rheumatology
Matthias Papo, Renato A. Sinico, Vitor Teixeira, Nils Venhoff, Maria-Letizia Urban, Michele Iudici, Juliane Mahrhold, Francesco Locatelli, Giulia Cassone, Franco Schiavon, Benjamin Seeliger, Thomas Neumann, Claus Kroegel, Matthieu Groh, Chiara Marvisi, Maxime Samson, Thomas Barba, David Jayne, Arianna Troilo, Jens Thiel, Bernhard Hellmich, Sara Monti, Carlomaurizio Montecucco, Carlo Salvarani, Jean-Emmanuel Kahn, Bernard Bonnotte, Cecile-Audrey Durel, Xavier Puechal, Luc Mouthon, Loic Guillevin, Giacomo Emmi, Augusto Vaglio, Benjamin Terrier
Summary: PR3-ANCA EGPA patients differ from MPO-ANCA and ANCA-negative patients in clinical presentation, but share similarities with granulomatosis with polyangiitis, suggesting it may be a distinct form of PR3-ANCA-associated vasculitis.
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.
Article
Rheumatology
Burak Ince, Sevil Kamali, Murat Bektas, Yeliz Duvarci Ogret, Fatma Oguz Savran, Yasemin Yalcinkaya, Bahar Artim-Esen, Murat Inanc, Lale Ocal, Ahmet Gul
Summary: The study aimed to compare the distribution of HLA Class 2 genotypes among Turkish patients and healthy subjects, finding significant differences in certain HLA-DQB1 and DPB1 allele frequencies between the PR3-AAV subgroup and controls. Amino acid sequence comparison and structural analysis revealed potential associations between specific HLA-DPB1 alleles and PR3-ANCA positivity.
RHEUMATOLOGY INTERNATIONAL
(2021)
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
Immunology
Christoph Brieske, Peter Lamprecht, Anja Kerstein-Staehle
Summary: Cell death and dysregulated clearance of dead cells play crucial roles in chronic inflammatory processes and autoimmune diseases. This review discusses how regulated cell death and the release of damage-associated molecular patterns (DAMP) contribute to the granulomatous tissue inflammation and autoimmune responses in granulomatosis with polyangiitis (GPA).
FRONTIERS IN IMMUNOLOGY
(2022)
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.
Article
Medicine, Research & Experimental
Dhruti P. Chen, Claudia P. Aiello, DeMoris McCoy, Taylor Stamey, Jiajin Yang, Susan L. Hogan, Yichun Hu, Vimal K. Derebail, Eveline Y. Wu, J. Charles Jennette, Ronald J. Falk, Dominic J. Ciavatta
Summary: A GWAS identified an association between PR3-ANCA and a SNP (rs62132293) upstream of the PRTN3 gene. The variant (G allele) was shown to be an expression quantitative trait locus in healthy controls, but its clinical impact is still unknown. Patients carrying the variant allele had elevated leukocyte PRTN3 expression compared to non-carriers, while healthy controls had low PRTN3 regardless of genotype.
Article
Medicine, General & Internal
James Hesford, Andrew R. L. Medford, Harsha Gunawardena
Summary: This case highlights the multisystem nature of granulomatosis with polyangiitis (GPA) with unusual dural and large vessel aortic and pulmonary trunk involvement.
Article
Medicine, General & Internal
Yongzhen Chen, Qiuxia Wan, Bo Liu
Summary: A 29-year-old male patient was admitted to the hospital with fever and rash on both lower extremities, and symptoms of muscle pain, nausea, anorexia, abdominal pain, and diarrhea. After diagnosis, the patient was confirmed to have systemic EGPA and was treated with medication, resulting in the improvement of most symptoms but no improvement in limb weakness.
Review
Immunology
Brandon S. Walker, Lisa K. Peterson, Curry Koening, Sandra K. White, Robert L. Schmidt, Anne E. Tebo
Summary: The impact of using MPO-ANCA and PR3-ANCA in the stratification of AAV patients was investigated. The results showed that PR3-ANCA had higher sensitivity for GPA, while MPO-ANCA had higher sensitivity for MPA. Both MPO-ANCA and PR3-ANCA had high specificity in diagnosing AAV.
AUTOIMMUNITY REVIEWS
(2022)
Article
Immunology
Pierre M. Bataille, Cecile-Audrey Durel, Dominique Chauveau, Arnaud Panes, Eric Simon Thervet, Benjamin Terrier
Summary: A study conducted in France revealed that despite advances in therapeutic management, the mortality rates of GPA and MPA patients remain high and stable, highlighting the need for further improvement in management.
JOURNAL OF AUTOIMMUNITY
(2022)
Article
Otorhinolaryngology
Alexander Walkden, Mohammed Salem, Marios Stavrakas, Hisham Khalil
Summary: GPA is a systemic necrotizing vasculitis that often involves the upper and lower airways and kidneys. ANCA negative GPA can present with nasal symptoms as the initial manifestation.
ENT-EAR NOSE & THROAT JOURNAL
(2021)
Review
Rheumatology
Jan Henrik Schirmer, Beatriz Sanchez-Alamo, Bernhard Hellmich, David Jayne, Sara Monti, Raashid Ahmed Luqmani, Gunnar Tomasson
Summary: This systematic literature review provides evidence on the treatment of antineutrophil cytoplasm antibody-associated vasculitis (AAV) and suggests that cyclophosphamide and rituximab have similar efficacy for remission induction, but rituximab is more effective in relapsing disease. Faster tapering glucocorticoid protocols result in similar remission rates but lower rates of serious infections. Avacopan shows potential for rapid tapering and replacing glucocorticoids. Use of rituximab for maintenance of remission is associated with lower relapse rates compared with azathioprine. Prolonged maintenance treatment reduces relapse rates for both azathioprine and rituximab.
Article
Immunology
Meiping Lu, Weizhong Gu, Yuanjian Sheng, Jingjing Wang, Xuefeng Xu
Summary: Activated phosphoinositide 3-kinase delta syndrome (APDS) is an autosomal dominant primary immunodeficiency caused by gain-of-function mutations in PIK3CD or PIK3R1 genes. The phenotypes can vary greatly, with some patients asymptomatic while others experiencing severe immunodeficiency. This report describes the first cases of APDS presenting as childhood-onset granulomatosis with polyangiitis, highlighting the importance of considering APDS in the differential diagnosis of similar symptoms in children. Additionally, bronchoscopy may be a valuable tool in diagnosing APDS in patients with respiratory symptoms.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Dermatology
Khalaf Kridin, Christoph M. Hammers, Ralf J. Ludwig, Erez Onn, Yochai Schonmann, Abed Abu-Elhija, Dana Tzur Bitan, Enno Schmidt, Orly Weinstein, Arnon D. Cohen
Summary: There is a bidirectional association between bullous pemphigoid (BP) and atopic dermatitis (AD) and allergic rhinitis (AR). A history of AD and AR increases the risk of developing BP, while BP patients are at an increased risk of subsequent AD. BP patients with comorbid AD and AR are more likely to be managed with drugs and corticosteroids, and have reduced all-cause mortality.
Article
Biochemistry & Molecular Biology
Axel Kuenstner, Paul Schilf, Hauke Busch, Saleh M. Ibrahim, Misa Hirose
Summary: This study reported on two mouse strains carrying single nucleotide variations in the mitochondrial complex I gene. Despite mild mitochondrial functional differences, the mice carrying specific variants exhibited longer lifespan and lower susceptibility to metabolic diseases. Analysis of gut microbiota composition revealed an association with mouse phenotypes, and predictions of functional profile suggested involvement of glucose metabolism pathways. These findings suggest that both host gene expression and gut microbial changes caused by mtDNA variant differences may contribute to the aging and metabolic phenotypes observed in these mice strains.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Editorial Material
Dermatology
Katharina Boch, Ewan A. Langan, Enno Schmidt, Detlef Zillikens, Ralf J. Ludwig, Katja Bieber, Christoph M. Hammers
ACTA DERMATO-VENEREOLOGICA
(2022)
Article
Rheumatology
S. T. Jendrek, M. Glatzel, D. Pauli, S. Schinke, J. Y. Humrich, P. Lamprecht, G. Riemekasten
Summary: This article describes a rare differential diagnosis of hantavirus-associated myositis compared to polymyositis. The literature on the pathogenesis of hantavirus disease focuses more on secondary immune dysregulation and capillary leak rather than direct viral cytopathology. This article presents the first case of successful treatment for prolonged hantavirus myositis using high-dose glucocorticoids and cyclophosphamide, followed by ciclosporin and MTX.
ZEITSCHRIFT FUR RHEUMATOLOGIE
(2022)
Meeting Abstract
Rheumatology
S. Arnold, P. Wallmeier, F. Schubach, G. Ihorst, P. Aries, R. Bergner, J. P. Bremer, N. Goerl, B. Hellmich, J. Henes, B. Hoyer, A. Kangowski, I. Koetter, C. Metzler, U. Mueller-Ladner, M. Schaier, U. Schoenermark, J. Thiel, L. Unger, N. Venhoff, J. Weinmann-Menke, J. Petersen, C. Iking-Konert, P. Lamprecht
ANNALS OF THE RHEUMATIC DISEASES
(2022)
Meeting Abstract
Rheumatology
S. Arnold, J. Mahrhold, A. Kerstein-Staehle, E. Csernok, B. Hellmich, N. Venhoff, J. Thiel, K. Affeldt, A. Jahnke, G. Riemekasten, P. Lamprecht
ANNALS OF THE RHEUMATIC DISEASES
(2022)
Meeting Abstract
Rheumatology
E. B. Adjailia, H. Grasshoff, J. Y. Humrich, P. Lamprecht, G. Riemekasten
ANNALS OF THE RHEUMATIC DISEASES
(2022)
Article
Rheumatology
Sebastian Klapa, Sabrina Arnold, Peter Lamprecht
Summary: Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are two types of ANCA-associated vasculitis (AAV) characterized by systemic necrotizing small-vessel vasculitis affecting any organ. GPA also involves extravascular necrotizing granulomatous inflammation. It typically presents as a pulmonary-renal syndrome with alveolar hemorrhage and rapidly progressive glomerulonephritis.
AKTUELLE RHEUMATOLOGIE
(2023)
Review
Immunology
Katja Bieber, Jennifer E. Hundt, Xinhua Yu, Marc Ehlers, Frank Petersen, Christian M. Karsten, Jorg Koehl, Khalaf Kridin, Kathrin Kalies, Anika Kasprick, Stephanie Goletz, Jens Y. Humrich, Rudolf A. Manz, Axel Kuenstner, Christoph M. Hammers, Reza Akbarzadeh, Hauke Busch, Christian D. Sadik, Tanja Lange, Hanna Grasshoff, Alexander M. Hackel, Jeanette Erdmann, Inke Koenig, Walter Raasch, Mareike Becker, Anja Kerstein-Staehle, Peter Lamprecht, Gabriela Riemekasten, Enno Schmidt, Ralf J. Ludwig
Summary: Approximately 5% of the world-wide population is affected by autoimmune diseases, which are still difficult to treat and have a significant economic impact. The progression from harmless to inflammatory autoimmune disease conditions is a key factor. Biomarkers that can predict this progression would be highly impactful. Factors such as genetics, environment, and lifestyle choices may influence the progression from benign to inflammatory autoimmune conditions. Research is needed to define and modulate autoimmune predisease.
AUTOIMMUNITY REVIEWS
(2023)
Biographical-Item
Dermatology
Christoph M. Hammers
BRITISH JOURNAL OF DERMATOLOGY
(2023)
Review
Immunology
Karen Aymonnier, Jennifer Amsler, Peter Lamprecht, Alan Salama, Veronique Witko-Sarsat
Summary: Vasculitis is a group of rare immune-mediated diseases characterized by the attack of blood vessels by the immune system. Neutrophils play an important role in these diseases by releasing cytotoxic mediators that can harm the vascular system.
IMMUNOLOGICAL REVIEWS
(2023)
Editorial Material
Rheumatology
Ole Hudowenz, Peter Lamprecht
ARTHRITIS & RHEUMATOLOGY
(2023)
Article
Rheumatology
Sebastian Klapa, Antje Mueller, Andreas Koch, Anja Kerstein-Staehle, Wataru Kaehler, Harald Heidecke, Susanne Schinke, Markus Huber-Lang, Martin Nitschke, Silke Pitann, Solveig Augustin, Christian M. Karsten, Gabriela Riemekasten, Peter Lamprecht
Summary: This study examined the concentrations of circulating antibodies targeting C3a and C5a complement receptors in AAV and analyzed their association with disease activity. The results showed that the concentrations of anti-C3aR and anti-C5aR antibodies were decreased in AAV patients. Low concentrations of anti-C5aR antibodies were associated with disease activity and an increased risk for relapse in AAV.
ARTHRITIS & RHEUMATOLOGY
(2023)
Article
Medicine, General & Internal
Anna Kernder, Tim Filla, Kirsten de Groot, Bernhard Hellmich, Julia Holle, Peter Lamprecht, Frank Moosig, Nikolas Ruffer, Christof Specker, Stefan Vordenbaeumen, Matthias Schneider, Gamal Chehab
Summary: This study analyzed the impact of the COVID-19 pandemic on medical care and vaccination acceptance of vasculitis patients in Germany. The findings showed that many patients had their scheduled rheumatology appointments canceled or switched to digital services due to the COVID-19 pandemic. Changes in therapeutic regimens were also observed. Overall, vasculitis patients in Germany had a high acceptance rate for vaccination, especially for COVID-19 vaccination.
FRONTIERS IN MEDICINE
(2023)
Article
Immunology
Anna Sophie Wesselmann, Axel Kuenstner, Anke Faehnrich, Christian Rose, Peter Lamprecht, Hauke Busch, Ralf J. Ludwig, Andreas Recke
Summary: Schnitzler syndrome is a rare autoinflammatory disorder characterized by urticarial rash, joint pain, recurrent fever, leucocytosis, elevated CRP and SAA, and monoclonal gammopathy. This case report highlights that the existing Strasbourg criteria for diagnosis may be too strict, as a Schnitzler-like syndrome was diagnosed despite the absence of gammopathy. The patient showed neutrophilic dermal inflammation, excessive increase in inflammatory parameters, prompt response to anakinra, and neutrophil epitheliotropism, suggesting an autoinflammatory disease.
FRONTIERS IN IMMUNOLOGY
(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)