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)
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.
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
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
Francisco Javier Gil-Etayo, Sara Garcinuno, Antonio Lalueza, Raquel Diaz-Simon, Ana Garcia-Reyne, Daniel Enrique Pleguezuelo, Oscar Cabrera-Marante, Edgard Alfonso Rodriguez-Frias, Alfredo Perez-Rivilla, Manuel Serrano, Antonio Serrano
Summary: The presence of aPL in COVID-19 patients is significantly associated with thrombosis, with potential mechanisms including early immune dysregulation and delayed aPL-mediated thrombosis. Additionally, aPL found in COVID-19 patients may preexist before the infection.
Article
Hematology
Arne Vandevelde, Walid Chayoua, Bas de Laat, Gary W. Moore, Jacek Musial, Stephane Zuily, Denis Wahl, Katrien M. J. Devreese
Summary: The presence of antiphosphatidylserine/prothrombin antibodies (aPS/PT), especially with high antibody titer, is associated with the diagnosis of thrombotic antiphospholipid syndrome (TAPS).
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2022)
Article
Immunology
Simona Truglia, Antonella Capozzi, Silvia Mancuso, Valeria Manganelli, Luca Rapino, Gloria Riitano, Serena Recalchi, Serena Colafrancesco, Fulvia Ceccarelli, Tina Garofalo, Cristiano Alessandri, Agostina Longo, Roberta Misasi, Fabrizio Conti, Maurizio Sorice
Summary: Antiphospholipid syndrome (APS) is more common in women than in men, with male patients often exhibiting high levels of IgM anti-cardiolipin antibodies and a higher incidence of arterial thrombosis and myocardial infarction. Gender appears to influence the serological and clinical profiles of APS.
FRONTIERS IN IMMUNOLOGY
(2022)
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)
Review
Rheumatology
Savino Sciascia, Massimo Radin, Irene Cecchi, Maria Laura Bertolaccini, Maria Tiziana Bertero, Elena Rubini, Antonella Vaccarino, Mario Bazzan, Osvaldo Giachino, Simone Baldovino, Daniela Rossi, Giulio Mengozzi, Dario Roccatello
Summary: This study identified five clusters of patients with aPL, each exhibiting different clinical and laboratory characteristics. Clusters 1, 2, 3, and 5 corresponded to well-known entities, while cluster 4 may represent a bridging condition between primary APS and SLE. Cluster 4 showed lower thrombotic risk compared to primary APS but higher features such as ANA and cytopenia.
Article
Medicine, General & Internal
Chun Li, Yu Zuo, Song Zhang, Una E. Makris, David R. Karp, Zhanguo Li
Summary: This study aimed to identify additional clinical and epidemiological risks of arterial thrombosis, venous thrombosis, and pregnancy morbidities in individuals with persistent antiphospholipid antibodies (aPLs)-positive carriers. The study analyzed a large cohort of patients and found that smoking, hypertension, and the presence of underlying autoimmune diseases were additional risk factors for arterial thrombosis. Smoking and the presence of underlying autoimmune diseases were additional risk factors for venous thrombosis, and the presence of underlying autoimmune diseases was significantly associated with pregnancy morbidities. Furthermore, higher levels of cell-free DNA and citrullinated histone H3 were observed in APS patients and aPL patients with autoimmune diseases.
CHINESE MEDICAL JOURNAL
(2022)
Review
Medicine, General & Internal
Marco Capecchi, Maria Abbattista, Alessandro Ciavarella, Mario Uhr, Cristina Novembrino, Ida Martinelli
Summary: Antiphospholipid syndrome (APS) is an autoimmune disease characterized by the presence of antiphospholipid antibodies and thrombosis or obstetrical complications. Anticoagulant therapy is the mainstay of treatment, but its optimal intensity and risk stratification for thrombosis are still unclear.
JOURNAL OF CLINICAL MEDICINE
(2022)
Review
Medicine, General & Internal
Jin Zhang, Cheng Li, Xiaorong Han, Zhongbo Chen, Binay Kumar Adhikari, Yinghui Wang, Yonggang Wang, Jian Sun
Summary: Antiphospholipid syndrome (APS) is an autoimmune disease characterized by vascular thrombosis and pregnancy complications. Digestive system involvement in APS may result from autoimmune or thrombotic mechanisms, with the liver being the most commonly affected organ. This review discusses the clinical manifestations of APS in the digestive system, along with the epidemiology, pathophysiology, and treatment strategies for these patients.
ANNALS OF MEDICINE
(2021)
Article
Immunology
Manuel Serrano, Gerard Espinosa, Antonio Serrano, Ricard Cervera
Summary: COVID-19 not only affects the respiratory system, but also has an impact on the cardiovascular system, causing coagulation abnormalities and potentially increasing the risk of thrombotic events and mortality. This multiorgan vascular disease overlaps with other known microangiopathies and may be associated with inflammatory response and coagulopathy.
AUTOIMMUNITY REVIEWS
(2022)
Article
Multidisciplinary Sciences
Nadine Mueller-Calleja, Anne Hollerbach, Jennifer Royce, Svenja Ritter, Denise Pedrosa, Thati Madhusudhan, Sina Teifel, Myriam Meineck, Friederike Haeuser, Antje Canisius, T. Son Nguyen, Johannes Braun, Kai Bruns, Anna Etzold, Ulrich Zechner, Susanne Strand, Markus Radsak, Dennis Strand, Jian-Ming Gu, Julia Weinmann-Menke, Charles T. Esmon, Luc Teyton, Karl J. Lackner, Wolfram Ruf
Summary: The study revealed that antiphospholipid antibodies (aPLs) recognize endosomal lysobisphosphatidic acid (LBPA) presented by the endothelial protein C receptor (EPCR), leading to thrombosis induction and inflammatory signaling, contributing to autoimmune pathologies and autoantibody production. Pharmacological intervention targeting the EPCR-LBPA signaling pathway can attenuate these pathologies.
Article
Clinical Neurology
Liqi Shu, Ekaterina Bakradze, Setareh Salehi Omran, James Giles, Jordan Amar, Nils Henninger, Marwa Elnazeir, Ava Liberman, Khadean Moncrieffe, Jenny Rotblat, Richa Sharma, Yee Cheng, Adeel S. Zubair, Alexis Simpkins, Grace Li, Justin Kung, Dezaray Perez, Mirjam R. Heldner, Adrian Scutelnic, Rascha Von Martial, Bernhard Siepen, Aaron Rothstein, Ossama Khazaal, David Do, Sami Al Kasab, Line Abdul Rahman, Eva A. Mistry, Deborah Kerrigan, Hayden Lafever, Thanh N. Nguyen, Piers Klein, Hugo J. Aparicio, Jennifer A. Frontera, Lindsey Kuohn, Shashank Agarwal, Christoph Stretz, Narendra Kala, Sleiman ElJamal, Allison Chang, Shawna Cutting, Fransisca Indraswari, Adam de Havenon, Varsha Muddasani, Teddy Wu, Duncan Wilson, Amre Nouh, Daniyal Asad, Abid Qureshi, Justin Moore, Pooja Khatri, Yasmin Aziz, Bryce Casteigne, Muhib Khan, Yao Cheng, Brian MacGrory, Martin Weiss, Dylan Ryan, Maria Cristina Vedovati, Maurizio Paciaroni, James Siegler, Scott Kamen, Siyuan Yu, Christopher Leon Guerrero, Eugenie Atallah, Gian Marco De Marchis, Alex Brehm, Tolga Dittrich, Marios Psychogios, Ronald Alvarado-Dyer, Tareq Kass-Hout, Shyam Prabhakaran, Tristan Honda, David Liebeskind, Karen Furie, Shadi Yaghi
Summary: This study aimed to identify predictors of recurrent venous thrombosis in patients with CVT. The study found that Black race, history of VTE, and the presence of one or more positive antiphospholipid antibodies were associated with recurrent venous thrombosis among patients with CVT.
Letter
Rheumatology
Nadine Mueller-Calleja, Karl J. Lackner
ANNALS OF THE RHEUMATIC DISEASES
(2019)
Review
Immunology
Karl J. Lackner, Nadine Mueller-Calleja
EXPERT REVIEW OF CLINICAL IMMUNOLOGY
(2019)
Article
Biology
Klytaimnistra Kiouptsi, Stefanie Finger, Venkata S. Garlapati, Maike Knorr, Moritz Brandt, Ulrich Walter, Philip Wenze, Christoph Reinhardt
Article
Hematology
Anne Hollerbach, Nadine Mueller-Calleja, Svenja Ritter, Friederike Haeuser, Antje Canisius, Carolin Orning, Kerstin Jurk, Karl J. Lackner
THROMBOSIS AND HAEMOSTASIS
(2019)
Meeting Abstract
Medical Laboratory Technology
N. Mueller-Calleja, K. J. Lackner
CLINICA CHIMICA ACTA
(2019)
Article
Hematology
Nadine Mueller-Calleja, Anne Hollerbach, Svenja Ritter, Denise G. Pedrosa, Dennis Strand, Claudine Graf, Christoph Reinhardt, Susanne Strand, Philippe Poncelet, John H. Griffin, Karl J. Lackner, Wolfram Ruf
Article
Cardiac & Cardiovascular Systems
Anne Hollerbach, Nadine Mueller-Calleja, Antje Canisius, Carolin Orning, Karl J. Lackner
JOURNAL OF THROMBOSIS AND THROMBOLYSIS
(2020)
Article
Biochemistry & Molecular Biology
Stephanie Makhoul, Stephanie Dorschel, Stepan Gambaryan, Ulrich Walter, Kerstin Jurk
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2020)
Article
Cell Biology
Elena J. Kumm, Oliver Pagel, Stepan Gambaryan, Ulrich Walter, Rene P. Zahedi, Albert Smolenski, Kerstin Jurk
Article
Hematology
Shane Comer, Zoltan Nagy, Alfonso Bolado, Alexander von Kriegsheim, Stepan Gambaryan, Ulrich Walter, Oliver Pagel, Rene P. Zahedi, Kerstin Jurk, Albert Smolenski
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2020)
Article
Biochemistry & Molecular Biology
Stephanie Makhoul, Elena Kumm, Pengyu Zhang, Ulrich Walter, Kerstin Jurk
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2020)
Article
Multidisciplinary Sciences
Nadine Mueller-Calleja, Anne Hollerbach, Jennifer Royce, Svenja Ritter, Denise Pedrosa, Thati Madhusudhan, Sina Teifel, Myriam Meineck, Friederike Haeuser, Antje Canisius, T. Son Nguyen, Johannes Braun, Kai Bruns, Anna Etzold, Ulrich Zechner, Susanne Strand, Markus Radsak, Dennis Strand, Jian-Ming Gu, Julia Weinmann-Menke, Charles T. Esmon, Luc Teyton, Karl J. Lackner, Wolfram Ruf
Summary: The study revealed that antiphospholipid antibodies (aPLs) recognize endosomal lysobisphosphatidic acid (LBPA) presented by the endothelial protein C receptor (EPCR), leading to thrombosis induction and inflammatory signaling, contributing to autoimmune pathologies and autoantibody production. Pharmacological intervention targeting the EPCR-LBPA signaling pathway can attenuate these pathologies.
Article
Hematology
Anne Hollerbach, Nadine Mueller-Calleja, Denise Pedrosa, Antje Canisius, Martin F. Sprinzl, Tanja Falter, Heidi Rossmann, Marc Bodenstein, Christian Werner, Ingo Sagoschen, Thomas Muenzel, Oliver Schreiner, Visvakanth Sivanathan, Michael Reuter, Johannes Niermann, Peter R. Galle, Luc Teyton, Wolfram Ruf, Karl J. Lackner
Summary: The study found that lipid-binding aPL are common in COVID-19 patients, with higher concentrations of inflammatory markers and increased likelihood of critical clinical course and fatal outcome. B1a cells producing lipid-reactive aPL of the IgG isotype circulate in the blood of COVID-19 patients, leading to thrombotic and inflammatory effects. In vivo, COVID-19 aPL accelerate thrombus formation in an experimental mouse model, indicating a causal role in COVID-19-associated coagulopathy.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2021)
Article
Immunology
Nadine Mueller-Calleja, Anne Hollerbach, Antje Canisius, Carolin Orning, Susanne Strand, Karl J. Lackner
Summary: In order to activate cellular signals, endosomal toll-like receptors (TLRs) need to be translocated from the endoplasmic reticulum (ER) to the endosome and proteolytically cleaved within the endosome. Antiphospholipid antibodies induce endosomal NADPH-oxidase (NOX) and the translocation of TLR7/8 to the endosome. The deficiency of NOX2 or the inhibition of endosomal NOX impedes the translocation of TLR3, TLR7/8, and TLR9, leading to delayed mRNA synthesis and secretion of TNF-α.
EUROPEAN JOURNAL OF IMMUNOLOGY
(2023)
Review
Hematology
Karl J. Lackner, Nadine Mueller-Calleja