Article
Hematology
Romain Stammler, Yann Nguyen, Cecile Yelnik, Veronique Le Guern, Marc Lambert, Romain Paule, Eric Hachulla, Luc Mouthon, Anastasia Dupre, Felix Ackermann, Virginie Dufrost, Denis Wahl, Bertrand Godeau, Gaelle Leroux, Ygal Benhamou, Estibaliz Lazaro, Eric Daugas, Holy Bezanahary, Arsene Mekinian, Jean-Charles Piette, Nathalie Morel, Nathalie Costedoat-Chalumeau
Summary: This study analyzed the prevention of catastrophic antiphospholipid syndrome (CAPS), focusing on anticoagulation before CAPS episodes. The results indicated that the major precipitating factors of CAPS were infections, pregnancy, and surgery. Among patients with previous APS diagnosis, the proportion of using vitamin K antagonists (VKA) was low and many patients had a subtherapeutic international normalized ratio (INR) below 2, suggesting suboptimal anticoagulation management. These findings strongly suggest that inadequate anticoagulation can trigger CAPS.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2023)
Article
Immunology
Savino Sciascia, Massimo Radin, Mario Bazzan, Barbara Montaruli, Domenico Cosseddu, Claudio Norbiato, Maria Tiziana Bertero, Renato Carignola, Beatrice Bacco, Silvia Gallo Cassarino, Dario Roccatello
Summary: The presence of antiphospholipid antibodies in COVID-19 patients may contribute to a pro-coagulant state, but differs from patients with overt APS. Therefore, caution is needed in interpreting and generalizing the role of aPL in the management of COVID-19 patients. Additional well-designed clinical studies are necessary before introducing aPL testing as routine in COVID-19 patients.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Rheumatology
Aya Nawata, Rie Shirayama, Koichi Oshida, Tetsuji Sato, Takuma Ito, Eisuke Shiba, Koichi Kusuhara, Masanori Hisaoka
Summary: This case report highlights a challenging diagnosis and pathogenesis of catastrophic antiphospholipid syndrome and EBV-associated hemophagocytosis in a 14-year-old girl.
Review
Pharmacology & Pharmacy
Ariela Hoxha, Daniela Tormene, Elena Campello, Paolo Simioni
Summary: This study conducted a systematic review on the additional treatments used in refractory and high-risk antiphospholipid antibody syndrome (APS) pregnancies. The results showed that combination therapy with IVIG and PEX achieved a 100% live birth rate in refractory APS pregnancies, while Pravastatin, IA, and PEX showed higher live birth rates in high-risk APS pregnancies.
FRONTIERS IN PHARMACOLOGY
(2022)
Article
Rheumatology
Medha Barbhaiya, Stephane Zuily, Ray Naden, Alison Hendry, Florian Manneville, Mary-Carmen Amigo, Zahir Amoura, Danieli Andrade, Laura Andreoli, Bahar Artim-Esen, Tatsuya Atsumi, Tadej Avcin, Michael H. Belmont, Maria Laura Bertolaccini, D. Ware Branch, Graziela Carvalheiras, Alessandro Casini, Ricard Cervera, Hannah Cohen, Nathalie Costedoat-Chalumeau, Mark Crowther, Guilherme de Jesus, Aurelien Delluc, Sheetal Desai, Maria De Sancho, Katrien M. Devreese, Reyhan Diz-Kucukkaya, Ali Duarte-Garcia, Camille Frances, David Garcia, Jean-Christophe Gris, Natasha Jordan, Rebecca K. Leaf, Nina Kello, Jason S. Knight, Carl Laskin, Alfred I. Lee, Kimberly Legault, Steve R. Levine, Roger A. Levy, Maarten Limper, Michael D. Lockshin, Karoline Mayer-Pickel, Jack Musial, Pier Luigi Meroni, Giovanni Orsolini, Thomas L. Ortel, Vittorio Pengo, Michelle Petri, Guillermo Pons-Estel, Jose A. Gomez-Puerta, Quentin Raimboug, Robert Roubey, Giovanni Sanna, Surya V. Seshan, Savino Sciascia, Maria G. Tektonidou, Angela Tincani, Denis Wahl, Rohan Willis, Cecile Yelnik, Catherine Zuily, Francis Guillemin, Karen Costenbader, Doruk Erkan
Summary: This study developed new APS classification criteria with high specificity, including clinical and laboratory domains, and improved classification accuracy through weighting and stratification.
ANNALS OF THE RHEUMATIC DISEASES
(2023)
Review
Immunology
Xue Peng, Xi Tan, Aiyun Xing
Summary: Antiphospholipid syndrome (APS) is an acquired autoimmune disorder characterized by recurrent thrombosis and pregnancy complications. Obstetrical APS (OAPS) specifically refers to APS in pregnant women. The classification criteria for OAPS have generated discussion due to the possibility of excluding certain patients. This article presents two cases of non-criteria OAPS with severe complications and discusses the diagnosis, treatment, and prognosis of this unusual antenatal event. It also provides an overview of the pathogenetic mechanisms and clinical features of OAPS.
FRONTIERS IN IMMUNOLOGY
(2023)
Review
Medicine, General & Internal
Al-Ameen Oredegbe, Fabiana Maria Santelises Robledo, Krishnakumar Hongalgi, Geovani Faddoul, Swati Mehta
Summary: Catastrophic anti-phospholipid syndrome (CAPS) is a rare and fatal complication of antiphospholipid syndrome (APS) characterized by microvascular thrombosis in multiple sites leading to multi-organ damage. We present a challenging case of CAPS in a patient who initially presented with bilateral adrenal hemorrhage, making the diagnosis difficult. This case highlights the importance of considering APS in patients with multi-organ failure and multiple thromboses and hemorrhage.
AMERICAN JOURNAL OF THE MEDICAL SCIENCES
(2023)
Article
Biochemistry & Molecular Biology
Katja Perdan-Pirkmajer, Polona Zigon, Anja Boc, Eva Podovsovnik, Sasa Cucnik, Alenka Mavri, Ziga Rotar, Ales Ambrozic
Summary: This study suggests that negative aCL and/or anti-beta 2GPI at the time of acute DVT may not require further aPL testing, while LA should be checked after discontinuation of anticoagulant therapy. Positive aCL and/or anti-beta 2GPI at the time of acute DVT have a strong positive predictive value for APS and may impact therapeutic decisions.
Article
Immunology
Giuseppe Barilaro, Emmanuel Coloma-Bazan, Alejandro Chacur, Carlo Della Rocca, Albert Perez-Isidro, Estibaliz Ruiz-Ortiz, Odette Vinas, Dolors Tassies Penella, Joan Carles Reverter, Alicia Molina Andujar, Ricard Cervera, Gerard Espinosa
Summary: The purpose of this study was to analyze the persistence of antiphospholipid antibodies (aPL) over time in patients with antiphospholipid syndrome (APS) and its association with clinical recurrence. The results showed that more than half of the patients maintained persistent positive aPLs over a long-term follow-up, and these patients were more prone to experience recurrence of clinical manifestations.
AUTOIMMUNITY REVIEWS
(2022)
Review
Immunology
Brenda Lopez-Benjume, Ignasi Rodriguez-Pinto, Mary Carmen Amigo, Doruk Erkan, Yehuda Shoenfeld, Ricard Cervera, Gerard Espinosa
Summary: This study utilized data from the CAPS Registry to describe the real-world experience of eculizumab use in patients with catastrophic antiphospholipid syndrome (CAPS). Eculizumab can be considered as a first-line or rescue therapy for CAPS patients who have failed previous treatments.
AUTOIMMUNITY REVIEWS
(2022)
Article
Immunology
Michael A. Cole, Gloria F. Gerber, Shruti Chaturvedi
Summary: Complement is a major driver of antiphospholipid syndrome (APS) and a promising therapeutic target. Reliable, rapid-turnaround biomarkers are needed in APS to predict adverse clinical outcomes and determine who will benefit most from complement inhibition. Current studies highlight the inconsistencies in testing, suggesting that cell-based testing or multiplex panels may be most clinically relevant.
CLINICAL IMMUNOLOGY
(2023)
Article
Rheumatology
Ana Ponce, Ignasi Rodriguez-Pinto, Jose M. Basauli, Gerard Espinosa, Doruk Erkan, Yehuda Shoenfeld, Ricard Cervera
Summary: This study found that low levels of C3 and C4 plasma proteins are detected in 58% of episodes in patients with catastrophic antiphospholipid syndrome (CAPS). However, this decrease is not associated with clinical presentation, aPL positivity, or mortality.
Review
Biochemistry & Molecular Biology
Svetlana Vrzic Petronijevic, Aleksandra Vilotic, Zanka Bojic-Trbojevic, Sanja Kostic, Milos Petronijevic, Ljiljana Vicovac, Milica Jovanovic Krivokuca
Summary: Antiphospholipid syndrome (APS) is an autoimmune disease characterized by the presence of antiphospholipid antibodies (aPL). Women with APS are at high risk of recurrent early pregnancy loss and late obstetrical complications. In addition to vascular thrombosis, research has found that aPL has a direct negative effect on placental cells, which is a major mechanism of obstetric APS.
Review
Biochemistry & Molecular Biology
Alex A. Gandhi, Shanea K. Estes, Christine E. Rysenga, Jason S. Knight
Summary: Antiphospholipid syndrome (APS) is a leading cause of thrombosis, with pathogenic antibodies circulating at stable levels in blood, but thrombotic events often requiring a second hit. Investigators have turned to animal models to explore APS mechanisms, with each model having potential advantages and disadvantages.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Review
Immunology
Maria Eva Mingot-Castellano, Nora Butta, Mariana Canaro, Maria del Carmen Gomez del Castillo Solano, Blanca Sanchez-Gonzalez, Reyes Jimenez-Barcenas, Cristina Pascual-Izquierdo, Gonzalo Caballero-Navarro, Laura Entrena Urena, Tomas Jose Gonzalez-Lopez
Summary: Vaccination against SARS-CoV-2 can lead to hematologic autoimmune complications, including immune thrombocytopenia and immune thrombotic thrombocytopenic purpura. Although rare, prompt treatment is crucial to prevent life-threatening situations.
Article
Immunology
Oscar-Danilo Ortega-Hernandez, Yehuda Shoenfeld
CONTEMPORARY CHALLENGES IN AUTOIMMUNITY
(2009)
Article
Immunology
Oscar-Danilo Ortega-Hernandez, Mariaclara Cuccia, Sara Bozzini, Nicola Bassi, Samuel Moscavitch, Lina-Marcela Diaz-Gallo, Miri Blank, Nancy Agmon-Levin, Yehuda Shoenfeld
CONTEMPORARY CHALLENGES IN AUTOIMMUNITY
(2009)
Article
Immunology
Oscar-Danilo Ortega-Hernandez, Shaye Kivity, Yehuda Shoenfeld
Article
Rheumatology
Oscar-Danilo Ortega-Hernandez, Yehuda Shoenfeld
BEST PRACTICE & RESEARCH IN CLINICAL RHEUMATOLOGY
(2012)
Review
Allergy
Michal Benkler, Nancy Agmon-Levin, Sharon Hassin-Baer, Oren S. Cohen, Oscar-Danilo Ortega-Hernandez, Amalia Levy, Samuel-Datum Moscavitch, Martine Szyper-Kravitz, Maya Damianovich, Miri Blank, Joab Chapman, Yehuda Shoenfeld
CLINICAL REVIEWS IN ALLERGY & IMMUNOLOGY
(2012)
Article
Rheumatology
Oscar-Danilo Ortega-Hernandez, Ricardo Pineda-Tamayo, Aryce L. Pardo, Adriana Rojas-Villarraga, Juan-Manuel Anaya
CLINICAL RHEUMATOLOGY
(2009)
Article
Biotechnology & Applied Microbiology
Oscar-Danilo Ortega-Hernandez, Nancy-Agmon Levin, Arie Altman, Yehuda Shoenfeld
Article
Immunology
Nina Svetlicky, Oscar-Danilo Ortega-Hernandez, Luc Mouthon, Loic Guillevin, Hans-Jurgen Thiesen, Arie Altman, Martine Szyper Kravitz, Miri Blank, Yehuda Shoenfeld
JOURNAL OF CLINICAL IMMUNOLOGY
(2013)
Article
Rheumatology
M. Rinaldi, C. Perricone, O-D Ortega-Hernandez, R. Perricone, Y. Shoenfeld
Review
Rheumatology
Adriana Rojas-Villarraga, Oscar-Danilo Ortega-Hernandez, Luis F. Gomez, Aryce L. Pardo, Silvia Lopez-Guzman, Camila Arango-Ferreira, Maria-Eugenia Hincapie, Juan F. Betancur, Ricardo Pineda-Tamayo, Francisco J. Diaz, Juan-Manuel Anaya
SEMINARS IN ARTHRITIS AND RHEUMATISM
(2008)
Review
Rheumatology
Oscar-Danilo Ortega-Hernandez, Nicola Bassi, Yehuda Shoenfeld, Juan-Manuel Anaya
SEMINARS IN ARTHRITIS AND RHEUMATISM
(2009)
Review
Medicine, General & Internal
Shaye Kivity, Oscar D. Ortega-Hernandez, Yehuda Shoenfeld
ISRAEL MEDICAL ASSOCIATION JOURNAL
(2009)
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)