Review
Cardiac & Cardiovascular Systems
Cornelia M. Weyand, Joerg J. Goronzy
Summary: Giant cell arteritis is an autoimmune disease that affects medium and large arteries, causing inflammation, obstruction, and aneurysm formation. The disease progresses through various steps, involving both innate and adaptive immune responses, leading to the infiltration of immune cells into the arterial wall and the development of tissue-damaging macrophages. Understanding these immune mechanisms provides opportunities for prevention and management of this devastating disease.
CIRCULATION RESEARCH
(2023)
Review
Medicine, General & Internal
Kristie Pepper
Summary: Giant cell arteritis (GCA) affects large and medium sized blood vessels, including cranial vessels and the aorta. It is a rheumatic disease that rarely occurs in adults under 50 years old. GCA is the most common idiopathic systemic vasculitis. Systemic symptoms are common, and it can involve the muscles and extracranial branches of the carotid arteries. This article will review the epidemiology, pathogenesis, clinical manifestations, work up, and treatment options for GCA.
POSTGRADUATE MEDICINE
(2023)
Article
Rheumatology
Naomi J. Patel, Xiaoqing Fu, Yuqing Zhang, Sebastian H. Unizony, Zachary S. Wallace, Hyon K. Choi, John H. Stone
Summary: This study examined GCA patients enrolled in the GiACTA trial and found that cumulative prednisone exposure is associated with increased BMI, while effective disease control in newly diagnosed cases also contributes to BMI increase.
RHEUMATOLOGY AND THERAPY
(2022)
Article
Medicine, General & Internal
Anne Pankow, Sena Sinno, Thorsten Derlin, Marcus Hiss, Annette D. Wagner
Summary: This study retrospectively evaluated the efficacy of MMF in inducing remission in GCA. Results showed that MMF treatment effectively controlled disease activity in all patients and achieved disease remission.
FRONTIERS IN MEDICINE
(2023)
Review
Medicine, General & Internal
Andre Ramon, Helene Greigert, Paul Ornetti, Bernard Bonnotte, Maxime Samson
Summary: Giant cell arteritis (GCA) is a large-vessel granulomatous vasculitis that primarily affects patients over 50 years old. Diagnosis can be challenging due to the lack of specific biological tests. GCA can be divided into cranial and large-vessel types, with diagnosis confirmation typically requiring temporal artery biopsy. Large-vessel GCA presents with non-specific symptoms, and other potential diagnoses should be considered.
JOURNAL OF CLINICAL MEDICINE
(2022)
Review
Immunology
Mitsuhiro Akiyama, Shozo Ohtsuki, Gerald J. Berry, David H. Liang, Jorg J. Goronzy, Cornelia M. Weyand
Summary: Autoimmune diseases can affect every organ system, with giant cell arteritis being the most common autoimmune vasculitis. There are distinct features in GCA that set it apart from other diseases, requiring tailored diagnostic and therapeutic approaches. Recent studies are uncovering novel mechanisms for more precise pathogenic modeling and therapeutic targeting in GCA.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Immunology
Matheus Vieira, Paul Regnier, Anna Maciejewski-Duval, Alexandre Le Joncour, Guillaume Darasse-Jeze, Michelle Rosenzwajg, David Klatzmann, Patrice Cacoub, David Saadoun
Summary: This study investigates the molecular mechanisms of large-vessel involvement in giant cell arteritis (LV-GCA) by analyzing transcriptome and interferon gene signature in inflamed aortas from LV-GCA patients. The study reveals the critical involvement of pro-inflammatory signaling pathways, including interferon, JAK/STAT signaling, cytokines, and chemokines, in both aorta and T cells from LV-GCA patients. Furthermore, the study identifies a clear type I interferon signature in the aortas of LV-GCA patients and highlights the significant role of STAT3 in related gene networks. The findings suggest the potential for tailored therapeutic targeting in LV-GCA.
JOURNAL OF AUTOIMMUNITY
(2022)
Review
Immunology
Michelle L. Robinette, Deepak A. Rao, Paul A. Monach
Summary: Giant cell arteritis (GCA) is a granulomatous systemic vasculitis that affects the elderly, often co-occurring with inflammatory arthritis/bursitis and sharing features with other non-infectious granulomatous vasculitides. Research focuses on the immunopathology of GCA, comparing different variants of the disease and overlapping features with related conditions.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Cardiac & Cardiovascular Systems
Paul Regnier, Alexandre Le Joncour, Anna Maciejewski-Duval, Guillaume Darrasse-Jeze, Charles Dolladille, Wouter C. Meijers, Lisa Bastarache, Pierre Fouret, Patrick Bruneval, Floriane Arbaretaz, Celia Sayetta, Ana Marquez, Michelle Rosenzwajg, David Klatzmann, Patrice Cacoub, Javid J. Moslehi, Joe-Elie Salem, David Saadoun
Summary: By analyzing VigiBase database, we found that giant cell arteritis (GCA) is associated with anti-CTLA-4 treatment but not with anti-PD-1 or anti-PD-L1 treatment. We identified dysregulation of CTLA-4 pathway in CD4(+) T cells, particularly regulatory T cells, in GCA patients. This suggests the critical role of CTLA-4 immune checkpoint in GCA and provides a strong rationale for targeting this pathway in treatment.
CIRCULATION RESEARCH
(2023)
Editorial Material
Medicine, General & Internal
Alex Rebello, Prashant Joshi
Summary: A 70-year-old man presented with temporal headache and jaw pain with chewing, with thickened temporal arteries, but normal erythrocyte sedimentation rate.
NEW ENGLAND JOURNAL OF MEDICINE
(2022)
Article
Rheumatology
Eugenio De Miguel, Pierluigi Macchioni, Edoardo Conticini, Corrado Campochiaro, Rositsa Karalilova, Sara Monti, Cristina Ponte, Giulia Klinowski, Irene Monjo-Henry, Paolo Falsetti, Zguro Batalov, Alessandro Tomelleri, Alojzija Hocevar
Summary: The objective of this study was to analyze the prevalence and characteristics of subclinical giant cell arteritis (GCA) in patients with polymyalgia rheumatica (PMR). The researchers used ultrasound to examine multiple arteries of PMR patients and found some of them had subclinical GCA. Patients with subclinical GCA were older, had longer morning stiffness duration, and more frequently reported hip pain compared to those without subclinical GCA. The optimal screening strategy to detect vasculitis in PMR needs further investigation.
Review
Medicine, General & Internal
Colm Kirby, Rachael Flood, Ronan Mullan, Grainne Murphy, David Kane
Summary: Ultrasound is increasingly used for the diagnosis of GCA, with comparable sensitivity to MRI and PET/CT. It can predict disease severity and treatment response, and facilitate the implementation of fast-track clinics, reducing the risk of blindness. Ultrasound should be the first-line investigation for patients suspected of having GCA.
FRONTIERS IN MEDICINE
(2022)
Article
Rheumatology
Christian Dejaco, Cristina Ponte, Sara Monti, Davide Rozza, Carlo Alberto Scire, Lene Terslev, George A. W. Bruyn, Dennis Boumans, Wolfgang Hartung, Alojzija Hocevar, Marcin Milchert, Uffe Moller Dohn, Chetan B. Mukhtyar, Markus Aschwanden, Philipp Bosch, Dario Camellino, Stavros Chrysidis, Giovanni Ciancio, Maria Antonietta D'Agostino, Thomas Daikeler, Bhaskar Dasgupta, Eugenio De Miguel, Andreas P. Diamantopoulos, Christina Duftner, Ana Agueda, Ulrich Fredberg, Petra Hanova, Ib Tonder Hansen, Ellen-Margrethe Hauge, Annamaria Iagnocco, Nevsun Inanc, Aaron Juche, Rositsa Karalilova, Toshio Kawamoto, Kresten Krarup Keller, Helen Isobel Keen, Tanaz A. Kermani, Minna J. Kohler, Matthew Koster, Raashid Ahmed Luqmani, Pierluigi Macchioni, Sarah Louise Mackie, Esperanza Naredo, Berit Dalsgaard Nielsen, Michihiro Ogasawara, Carlos Pineda, Valentin Sebastian Schafer, Luca Seitz, Alessandro Tomelleri, Karina D. Torralba, Kornelis S. M. van der Geest, Kenneth J. Warrington, Wolfgang A. Schmidt
Summary: This study aimed to develop an OMERACT ultrasonography score for monitoring disease activity in giant cell arteritis (GCA) and evaluate its metric properties. The study followed the OMERACT Instrument Selection Algorithm and conducted Delphi rounds and online reliability exercise. The results showed that the OGUS had good reliability and sensitivity in assessing disease activity in GCA and could be used in clinical trials.
ANNALS OF THE RHEUMATIC DISEASES
(2023)
Review
Rheumatology
Peter M. Andel, Stavros Chrysidis, Julia Geiger, Anne C. Bull Haaversen, Glenn Haugeberg, Geirmund Myklebust, Berit D. Nielsen, Andreas P. Diamantopoulos
Summary: GCA is the most common large vessel vasculitis in the elderly population. Advanced imaging techniques have improved the diagnosis of GCA in many regions, especially when combined with US examinations in fast-track clinics. However, diagnostic modalities are operator dependent and expertise in advanced imaging may not be widely available in all locations. This paper reviews the current evidence on GCA diagnostics and proposes a simple algorithm for diagnosing GCA for rheumatologists not in specialist centers.
Article
Immunology
Mahmut S. Kaymakci, Nicholas A. Boire, Melanie C. Bois, Mohanad M. Elfishawi, Hannah E. Langenfeld, Andrew C. Hanson, Cynthia S. Crowson, Matthew J. Koster, Yuki Sato, Cornelia M. Weyand, Kenneth J. Warrington
Summary: This study investigated the clinicopathologic features of patients with giant cell arteritis (GCA) who had thoracic aorta aneurysm or dissection surgery. Histopathologic evaluation of the thoracic aorta obtained during surgery revealed active aortitis in most patients with GCA despite being considered in clinical remission several years after GCA diagnosis. The overall mortality compared to age and sex-matched general population was significantly increased.
AUTOIMMUNITY REVIEWS
(2023)
Article
Nutrition & Dietetics
L. E. T. Vissers, I Sluijs, S. Burgess, N. G. Forouhi, H. Freisling, F. Imamura, T. K. Nilsson, F. Renstroem, E. Weiderpass, K. Aleksandrova, C. C. Dahm, A. Perez-Cornago, M. B. Schulze, T. Y. N. Tong, D. Aune, C. Bonet, J. M. A. Boer, H. Boeing, M. D. Chirlaque, M. Conchi, L. Imaz, S. Jaeger, V Krogh, C. Kyro, G. Masala, O. Melander, K. Overvad, S. Panico, M. J. Sanches, E. Sonestedt, A. Tjonneland, I Tzoulaki, W. M. M. Verschuren, E. Riboli, N. J. Wareham, J. Danesh, A. S. Butterworth, Y. T. van der Schouw
Summary: Instrumental variable and gene-outcome analyses showed no significant causal relationship between milk consumption and the risk of stroke or CHD.
BRITISH JOURNAL OF NUTRITION
(2022)
Article
Peripheral Vascular Disease
Martina Chiriaco, Domenico Trico, Simone Leonetti, John R. Petrie, Beverley Balkau, Kurt Hojlund, Zoltan Pataky, Peter M. Nilsson, Andrea Natali
Summary: The relationship between adiposity and blood pressure is influenced by sex and ACE genotype, with a stronger effect observed in women and individuals carrying the II variant of the ACE gene.
Article
Endocrinology & Metabolism
Ayesha Fawad, Andreas Bergmann, Janin Schulte, Zahra A. Butt, Peter M. Nilsson, Louise Bennet, Marju Orho-Melander, Olle Melander
Summary: This study found that high levels of proneurotensin are associated with increased mortality risk in middle-aged individuals, particularly for deaths related to cardiovascular disease, digestive tract disease, mental and behavioral disease, and unspecific causes.
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM
(2022)
Article
Hematology
Eric Manderstedt, Christer Hallden, Christina Lind-Hallden, Johan Elf, Peter J. Svensson, Gunnar Engstrom, Olle Melander, Aris Baras, Luca A. Lotta, Bengt Zoller, Regeneron Genetics Center
Summary: This study aimed to investigate the relationship between THBD gene variations and VTE. The results showed that rare qualifying THBD variants were associated with VTE, suggesting their contribution to the development of VTE.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2022)
Article
Endocrinology & Metabolism
Filip Ottosson, Einar Smith, Ulrika Ericson, Louise Brunkwall, Marju Orho-Melander, Salvatore Di Somma, Paola Antonini, Peter M. Nilsson, Celine Fernandez, Olle Melander
Summary: Lean individuals with an obesity-related metabolome have an increased risk for type 2 diabetes and all-cause mortality compared with lean individuals with a healthy metabolome.
Article
Medicine, General & Internal
Sofia Enhorning, Olle Melander, Gunnar Engstrom, Solve Elmstahl, Lars Lind, Peter M. Nilsson, Mats Pihlsgard, Simon Timpka
Summary: A study in Malmo, Sweden found that copeptin, a surrogate marker of vasopressin, exhibits a distinct seasonal variation with a peak in winter and a nadir in late summer. This seasonal variation may be related to an increased risk of cardiometabolic disease.
JOURNAL OF INTERNAL MEDICINE
(2022)
Editorial Material
Rheumatology
Matthew J. Koster, Kenneth J. Warrington
Summary: The new classification criteria for ANCA-associated vasculitis endorsed by ACR and EULAR reflect important developments in the field and highlight the significance of ANCA testing.
NATURE REVIEWS RHEUMATOLOGY
(2022)
Editorial Material
Rheumatology
Bradly A. Kimbrough, Elie Naddaf, Kenneth J. Warrington, Rafael E. Jimenez, Matthew J. Koster
Article
Rheumatology
Karin Wadstrom, Lennart T. H. Jacobsson, Aladdin J. Mohammad, Kenneth J. Warrington, Eric L. Matteson, Magnus E. Jakobsson, Carl Turesson
Summary: Elevated levels of IFN-gamma were found years prior to the diagnosis of GCA, suggesting that T cell activation may precede the clinical onset of the disease.
Review
Rheumatology
Baptiste Chevet, Divi Cornec, Marta Casal Moura, Emilie Cornec-Le Gall, Fernando C. Fervenza, Kenneth J. Warrington, Ulrich Specks, Alvise Berti
Summary: ANCA-associated vasculitides (AAV) are rare, primary, systemic necrotizing small-vessel vasculitides. The most common types are granulomatosis with polyangiitis and microscopic polyangiitis. Risk factors for developing AAV include exposure to silica dust, farming, and chronic nasal Staphylococcus aureus infection. Diagnosis of AAV requires further investigation, including ANCA testing, and a structured clinical assessment. Treatment options vary depending on the severity of AAV and the patient's characteristics, with rituximab being favored as an effective option for both induction and maintenance of remission.
Review
Urology & Nephrology
Marta Casal Moura, Cynthia S. Crowson, Ulrich Specks, Kenneth J. Warrington, Ladan Zand, Sanjeev Sethi, Fernando C. Fervenza
Summary: Patients with ANCA-associated vasculitis (AAV) are at high risk of progressing to end-stage kidney disease (ESKD). Adding plasma exchange (PLEX) to standard treatment may reduce the risk of ESKD. However, there is still debate on which patients benefit from PLEX.
CLINICAL KIDNEY JOURNAL
(2023)
Article
Rheumatology
Alexander S. Hines, Matthew J. Koster, Allison R. Bock, Ronald S. Go, Kenneth J. Warrington, Horatiu Olteanu, Terra L. Lasho, Mrinal M. Patnaik, Kaaren K. Reichard
Summary: In this retrospective study, a clinically applicable targeted-screening approach was used to identify VEXAS syndrome among male patients with bone marrow vacuolization. A total of 292 patients were included, and after exclusion criteria, 21 patients were rated with moderate to high suspicion of VEXAS syndrome. Somatic UBA1 variants associated with VEXAS syndrome were detected in 87.5% of the tested patients. The utilization of this approach resulted in a positive detection rate of 87.5%.
Review
Immunology
Matthew J. Koster, Max Guarda, Umar Ghaffar, Kenneth J. Warrington
Summary: The article introduces mimics of vasculitis, including non-inflammatory syndromes and secondary vasculitis. The clinical, radiographic, and histological features that differentiate these cases from primary vasculitis are highlighted.
EXPERT REVIEW OF CLINICAL IMMUNOLOGY
(2023)
Article
Rheumatology
Mahmut S. Kaymakci, Mohanad M. Elfishawi, Hannah E. Langenfeld, Andrew C. Hanson, Cynthia S. Crowson, Melanie C. Bois, Umar Ghaffar, Matthew J. Koster, Ulrich Specks, Kenneth J. Warrington
Summary: This study aimed to characterize patients with L-AAV, where L-AAV can involve temporal arteries, aorta, and periaortic soft tissue.
Article
Rheumatology
Stephanie Q. Duong, Cynthia S. Crowson, Arjun Athreya, Elizabeth J. Atkinson, John M. Davis, Kenneth J. Warrington, Eric L. Matteson, Richard Weinshilboum, Liewei Wang, Elena Myasoedova
Summary: Using machine learning methods, we identified clinical predictors of response to methotrexate treatment in patients with RA, including baseline DAS28-ESR, ACPA, and HAQ score. Our prediction model for methotrexate response was externally validated in DMARD-naive patients with RA, providing guidance for clinical decision-making.
ARTHRITIS RESEARCH & THERAPY
(2022)
