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
Biochemistry & Molecular Biology
Cecilia Nalli, Daniele Lini, Laura Andreoli, Francesca Crisafulli, Micaela Fredi, Maria Grazia Lazzaroni, Viktoria Bitsadze, Antonia Calligaro, Valentina Canti, Roberto Caporali, Francesco Carubbi, Cecilia Beatrice Chighizola, Paola Conigliaro, Fabrizio Conti, Caterina De Carolis, Teresa Del Ross, Maria Favaro, Maria Gerosa, Annamaria Iuliano, Jamilya Khizroeva, Alexander Makatsariya, Pier Luigi Meroni, Marta Mosca, Melissa Padovan, Roberto Perricone, Patrizia Rovere-Querini, Gian Domenico Sebastiani, Chiara Tani, Marta Tonello, Simona Truglia, Dina Zucchi, Franco Franceschini, Angela Tincani
Summary: The study found that decreased complement levels before pregnancy are associated with an increased risk of adverse pregnancy outcomes in women with triple antiphospholipid antibodies positivity, while this trend was not observed in those with single or double positivity. Complement levels are cost-effective and easy to measure, making them a potentially useful tool for identifying pregnant women at risk of pregnancy loss.
Review
Rheumatology
Amala Ambati, Yu Zuo, Jason S. Knight
Summary: This review provides an overview of the pathogenesis and clinical manifestations of APS, focusing on endothelial cell activation, complement activity, and neutrophil involvement. Recent studies have revealed the association between APS and increased circulating endothelial cells, extracellular vesicles, complement activity, and neutrophil extracellular traps. Potential treatment strategies for APS are discussed, as well as future directions in research, such as molecular stratification of APS patients.
CURRENT OPINION IN RHEUMATOLOGY
(2023)
Article
Immunology
Savino Sciascia, Rory Bloch, Tyler O'Malley, Anja Kammesheidt, Roberta Vezza Alexander
Summary: High titers of aPL often remain positive, allowing for earlier diagnosis and risk assessment at the initial screening. Low titers may require retesting. There is a high correlation between aCL and aB2GP1 measured using two different technologies. The correlation between PC4d and aPL is low to moderate, suggesting a possible additional value in evaluating the association with thrombosis in autoimmune diseases.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Hematology
Giorgia Grosso, Kerstin Sandholm, Aleksandra Antovic, Iva Gunnarsson, Agneta Zickert, Anna Vikerfors, Lennart Truedsson, Maria Bruzelius, Bo Nilsson, Kristina Nilsson-Ekdahl, Elisabet Svenungsson
Summary: The study found that C4BPt levels are decreased in aPL++ patients regardless of SLE, APS, clinical manifestations, or aPL profile, with positive associations with certain complement proteins. Warfarin treatment contributes to a reduction in C4BPt levels. Additional studies are needed to further explore the clinical implications of these findings.
THROMBOSIS AND HAEMOSTASIS
(2021)
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)
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
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)
Article
Developmental Biology
Enrique Esteve-Valverde, Jaume Alijotas-Reig, Cristina Belizna, Joana Marques-Soares, Ariadna Anunciacion-Llunell, Carlos Feijoo-Masso, Luis Saez-Comet, Arsene Mekinian, Raquel Ferrer-Oliveras, Elmina Lefkou, Stephanie Morales-Perez, Ariel Hoxha, Angela Tincani, Cecilia Nalli, Josep Pardos-Gea, Luca Marozio, Aldo Maina, Gerard Espinosa, Ricard Cervera, Sara De Carolis, Omar Latino, Sebastian Udry, Elisa Llurba, Carmen Garrido-Gimenez, Laura Trespidi, Maria Gerosa, Cecilia B. Chighizola, Patrizia Rovere-Querini, Valentina Canti, Karoline Mayer-Pickel, Sara Tabacco, Anna Arnau, Francesc Miro-Mur
Summary: This study analyzed a cohort of 1048 women with OAPS and found that 21.3% of women had low complement levels during pregnancy. Compared to women with normal complement levels, those with low complement levels had shorter pregnancy length, lower neonatal survival rate, higher fetal loss rate, and some placental vasculopathies. Therefore, proper treatment can improve adverse pregnancy outcomes related to low complement levels.
Article
Rheumatology
Elena Gkrouzman, Ecem Sevim, Jackie Finik, Danieli Andrade, Vittorio Pengo, Savino Sciascia, Maria G. Tektonidou, Amaia Ugarte, Cecilia B. Chighizola, H. Michael Belmont, Chary Lopez-Pedrera, Lanlan Ji, Paul Fortin, Maria Efthymiou, Guilherme Ramires de Jesus, D. Ware Branch, Cecilia Nalli, Michelle Petri, Esther Rodriguez, Ricard Cervera, Jason S. Knight, Tatsuya Atsumi, Rohan Willis, Maria Laura Bertolaccini, Hannah Cohen, Jacob Rand, Doruk Erkan
Summary: The study revealed that approximately 80% of patients with persistently antiphospholipid antibody (aPL) positive remained stable in their aPL profiles over a 5-year follow-up period. Additionally, the decrease in triple aPL positivity at baseline was associated with increased odds of stable aPL profile.
JOURNAL OF RHEUMATOLOGY
(2021)
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
Obstetrics & Gynecology
Fergus P. McCarthy, Adedamola Adetoba, Carolyn Gill, Kate Bramham, Maria Bertolaccini, Graham J. Burton, Guillermina Girardi, Paul T. Seed, Lucilla Poston, Lucy C. Chappell
AMERICAN JOURNAL OF OBSTETRICS AND GYNECOLOGY
(2016)
Review
Immunology
Maria Laura Bertolaccini, Gregorio Contento, Ross Lennen, Giovanni Sanna, Philip J. Blower, Michelle T. Ma, Kavitha Sunassee, Guillermina Girardi
JOURNAL OF AUTOIMMUNITY
(2016)
Article
Medicine, Research & Experimental
Eleftheria Lefkou, Apostolos Mamopoulos, Themistoklis Dagklis, Christos Vosnakis, David Rousso, Guillermina Girardi
JOURNAL OF CLINICAL INVESTIGATION
(2016)
Article
Obstetrics & Gynecology
Rozalia Pataky, Forbes A. Howie, Guillermina Girardi, James P. Boardman
JOURNAL OF MATERNAL-FETAL & NEONATAL MEDICINE
(2017)
Article
Immunology
Guillermina Girardi
JOURNAL OF REPRODUCTIVE IMMUNOLOGY
(2016)
Article
Biochemistry & Molecular Biology
Silvia M. A. Pedroni, Juan M. Gonzalez, Jean Wade, Maurits A. Jansen, Andrea Serio, Ian Marshall, Ross J. Lennen, Guillermina Girardi
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE
(2014)
Article
Peripheral Vascular Disease
Jameel Singh, Abdulwahab Ahmed, Guillermina Girardi
Letter
Peripheral Vascular Disease
Eleftheria Lefkou, Apostolos Mamopoulos, Nikolaos Fragakis
Article
Medicine, Research & Experimental
Sangeetha Ramesh, Craig N. Morrell, Cristina Tarango, Gail D. Thomas, Ivan S. Yuhanna, Guillermina Girardi, Joachim Herz, Rolf T. Urbanus, Philip G. de Groot, Philip E. Thorpe, Jane E. Salmon, Philip W. Shaul, Chieko Mineo
JOURNAL OF CLINICAL INVESTIGATION
(2011)
Article
Oncology
Danielle Cohen, Aletta Buurma, Natascha N. Goemaere, Guillermina Girardi, Saskia le Cessie, Sicco Scherjon, Kitty W. M. Bloemenkamp, Emile de Heer, Jan A. Bruijn, Ingeborg M. Bajema
JOURNAL OF PATHOLOGY
(2011)
Article
Immunology
Juan M. Gonzalez, Roberto Romero, Guillermina Girardi
JOURNAL OF REPRODUCTIVE IMMUNOLOGY
(2013)
Article
Immunology
Guillermina Girardi
JOURNAL OF REPRODUCTIVE IMMUNOLOGY
(2014)
Review
Hematology
G. Girardi
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2011)
Article
Developmental Biology
Juan M. Gonzalez, Silvia M. A. Pedroni, Guillermina Girardi
MOLECULAR HUMAN REPRODUCTION
(2014)
Article
Biochemistry & Molecular Biology
G. Girardi, J. Fraser, R. Lennen, R. Vontell, M. Jansen, G. Hutchison
MOLECULAR PSYCHIATRY
(2015)