Review
Cardiac & Cardiovascular Systems
Martin Killian, Thijs E. van Mens
Summary: Antiphospholipid syndrome is an autoimmune disease characterized by thrombosis and pregnancy morbidity caused by antibodies targeting cell membrane phospholipids and/or associated proteins. Recent research suggests a possible link between the production of these antibodies and the peptides produced by the intestinal microbiome. Different mechanisms, including intracellular signaling, complement activation, neutrophil extracellular-traps, and the endothelial protein C receptor-lysobisphosphatidic acid complex, are implicated in the clinical manifestations caused by the autoantibodies. Pregnancy poses a high-risk situation for antiphospholipid syndrome patients due to the increased risk of thrombosis and obstetric complications, such as early pregnancy loss and late pregnancy loss.
FRONTIERS IN CARDIOVASCULAR MEDICINE
(2022)
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
Cardiac & Cardiovascular Systems
Vittorio Pavoni, Lara Gianesello, Andrew Horton
Summary: COVID-19 disease is associated with coagulation dysfunction, leading to an increased risk of arterial and venous thromboembolism, especially in critically ill patients. The mechanism of thrombosis in COVID-19 patients is complex and may be linked to the presence of antiphospholipid antibodies, but further research is needed to clarify their role.
JOURNAL OF THROMBOSIS AND THROMBOLYSIS
(2021)
Review
Cardiac & Cardiovascular Systems
Luca Depietri, Maria Rosaria Veropalumbo, Maria Cristina Leone, Angelo Ghirarduzzi
Summary: Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by recurrent thrombosis and/or pregnancy morbidity. Anticoagulation is the main treatment, but therapies targeting APS autoimmune pathogenesis have potential. Vitamin K antagonists (AVK) like warfarin are standard for venous thrombosis prevention. Direct oral anticoagulants (DOACs) aren't recommended for triple positive APS. Pregnancy complications may be improved with aspirin and heparin, prednisolone, hydroxychloroquine, IVIG. Catastrophic APS (CAPS) requires glucocorticoids, heparin, plasma exchange or IVIG, rituximab, or eculizumab. SARS COV2 infection may mimic APS, and anticoagulant therapy may be protective in this situation.
CARDIOVASCULAR DRUGS AND THERAPY
(2023)
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
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.
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)
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)
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
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
Biochemistry & Molecular Biology
Ariadna Anunciacion-Llunell, Candido Munoz, Dirk Roggenbuck, Stefano Frasca, Josep Pardos-Gea, Enrique Esteve-Valverde, Jaume Alijotas-Reig, Francesc Miro-Mur
Summary: Antiphospholipid syndrome (APS) is a systemic autoimmune condition characterized by the presence of antiphospholipid antibodies (aPL) associated with vascular thrombosis and/or pregnancy complications. The study found that OAPS and TAPS patients displayed different but overlapping clusters based on their aPL reactivities.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Hematology
Deepa J. Arachchillage, Indika Rajakaruna, Zain Odho, Christina Crossette-Thambiah, Phillip L. R. Nicolson, Lara N. Roberts, Caroline Allan, Sarah Lewis, Renu Riat, Philip Mounter, Ceri Lynch, Alexander Langridge, Roderick Oakes, Nini Aung, Anja Drebes, Tina Dutt, Priyanka Raheja, Alison Delaney, Sarah Essex, Gillian Lowe, David Sutton, Claire Lentaigne, Zara Sayar, Mari Kilner, Tamara Everington, Susie Shapiro, Raza Alikhan, Richard Szydlo, Michael Makris, Michael Laffan
Summary: Coagulation dysfunction and thrombosis are common complications in COVID-19 patients. this study found that prior use of oral anticoagulants may lead to better outcomes, including reducing the likelihood of admission to the ICU.
BRITISH JOURNAL OF HAEMATOLOGY
(2022)
Article
Hematology
Deepa J. Arachchillage, Indika Rajakaruna, Ian Scott, Mihaela Gaspar, Zain Odho, Winston Banya, Aikaterini Vlachou, Graziella Isgro, Lenka Cagova, Joshua Wade, Lucy Fleming, Mike Laffan, Richard Szydlo, Stephane Ledot, Rachel Jooste, Alain Vuylsteke, Hakeem Yusuff
Summary: Bleeding and thrombosis are common complications in patients supported with ECMO, associated with increased mortality risk. Major bleeding and intracranial bleeding increase the risk of mortality by nearly 4-fold, while pulmonary embolism increases mortality risk by 2-fold.
BRITISH JOURNAL OF HAEMATOLOGY
(2022)
Letter
Clinical Neurology
Christina Crossette-Thambiah, Charis Pericleous, Namir Asmar, Joshua Bomsztyk, Amita Ranger, Abdul Shlebak, Saipriya Ramji, Soma Banerjee, Mike Laffan, Deepa Arachchillage
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY
(2022)
Article
Hematology
Deepa J. Arachchillage, Lucy Mackillop, Arvind Chandratheva, Jayashree Motawani, Peter MacCallum, Mike Laffan
BRITISH JOURNAL OF HAEMATOLOGY
(2022)
Review
Cardiac & Cardiovascular Systems
Christophe Vandenbriele, Deepa J. Arachchillage, Pascal Frederiks, Gennaro Giustino, Diana A. Gorog, Mario Gramegna, Stefan Janssens, Bart Meyns, Amin Polzin, Mara Scandroglio, Benedikt Schrage, Gregg W. Stone, Guido Tavazzi, Thomas Vanassche, Pascal Vranckx, Dirk Westermann, Susanna Price, Alaide Chieffo
Summary: There is growing interest in the use of mechanical circulatory support for patients with cardiogenic shock, but the delicate balance between bleeding and thrombosis poses challenges for patient outcomes. This article presents an anti-Factor Xa/activated partial thromboplastin time-guided anticoagulation algorithm and discusses the monitoring pitfalls of heparin in critically ill patients, aiming to guide physicians in standardizing anticoagulation treatment to reduce device-related morbidity and mortality.
JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY
(2022)
Article
Rheumatology
Deepa J. Arachchillage, Indika Rajakaruna, Charis Pericleous, Philip L. R. Nicolson, Mike Makris, Mike Laffan
Summary: Patients with autoimmune disease (AD) admitted with COVID-19 showed differences in laboratory findings and clinical outcomes compared to patients without AD. Those with severe rheumatologic AD had higher mortality rates and elevated levels of anemia, renal impairment, LDH, CRP, which are associated with increased mortality in COVID-19 patients.
Article
Cardiac & Cardiovascular Systems
Ingrid Bekono-Nessah, Alex Rosenburg, Christopher T. Bowles, Fernando Riesgo-Gil, Ulrich Stock, Richard R. Szydlo, Mike Laffan, Deepa J. Arachchillage
Summary: Major bleeding and thrombosis significantly increase the 1-year mortality in HVAD patients. Optimal perioperative hemostasis and anticoagulation remain crucial for HVAD patients.
Review
Biochemistry & Molecular Biology
Deepa J. Arachchillage, Mike Laffan, Charis Pericleous
Summary: Antiphospholipid syndrome (APS) is a highly prothrombotic disorder caused by thrombo-inflammatory antiphospholipid antibodies (aPL) that lead to thrombosis through various mechanisms. Obstetric complications in APS are a result of placental thrombosis, inflammation, and complement activation. While anticoagulation is the main treatment, it may not effectively prevent thrombosis recurrence in some patients, especially those with triple positive aPL.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Hematology
Deepa J. Arachchillage, Charis Pericleous
Summary: Antiphospholipid syndrome (APS) is an autoimmune disease characterized by the presence of antiphospholipid antibodies, leading to thrombosis and/or pregnancy complications. APS has a long history dating back to the mid-20th century, when it was first identified during syphilis screening programs. While anticoagulation with vitamin K antagonists remains the main treatment for thrombotic APS, newer options like direct acting oral anticoagulants have shown inferiority, particularly in patients with triple-positive APS and arterial thrombosis. Immunomodulatory agents such as hydroxychloroquine are increasingly used to treat refractory cases of APS.
SEMINARS IN THROMBOSIS AND HEMOSTASIS
(2023)
Article
Hematology
Deepa Arachchillage, Indika Rajakaruna, Zain Odho, Mike Makris, Mike Laffan
Summary: The controversy of post-discharge thromboprophylaxis in COVID-19 patients remains. This observational study across 26 NHS Trusts aimed to determine the impact of thromboprophylaxis on hospital acquired thrombosis (HAT) in discharged patients. The study found no difference in HAT between patients discharged with and without thromboprophylaxis, but increasing age and smoking significantly increased the risk of HAT.
BRITISH JOURNAL OF HAEMATOLOGY
(2023)
Article
Critical Care Medicine
Benjamin E. E. Garfield, Paolo Bianchi, Deepa J. J. Arachchillage, Francisca Caetano, Sujal Desai, James Doyle, Clara Hernandez Caballero, Anne-Marie Doyle, Sachin Mehta, Alexander Law, Sian Jaggar, Maria Kokosi, Philip L. Molyneaux, Maurizio Passariello, Meena Naja, Carole Ridge, Joana Alcada, Brijesh Patel, Suveer Singh, Stephane Ledot
Summary: This study aimed to compare patients supported on venovenous extracorporeal membrane oxygenation (ECMO) between the first and second waves of COVID-19 in terms of mortality, decannulation, ECMO duration, complications, lung function, and quality of life. The results showed that the second wave had lower survival rate and decannulation rate, longer ECMO duration, longer noninvasive ventilation application, and higher frequency of barotrauma. However, lung function and quality of life at follow-up were similar across the waves.
CRITICAL CARE MEDICINE
(2023)
Review
Immunology
Daniel M. Altmann, Emily M. Whettlock, Siyi Liu, Deepa J. Arachchillage, Rosemary J. Boyton
Summary: Long COVID is a condition where persistent symptoms occur in a significant number of individuals who have had COVID-19, regardless of the severity of their initial infection. It is estimated that at least 10% of global COVID-19 patients have long COVID. The symptoms of long COVID can range from mild to severe, affecting multiple organs and systems, and can relapse and remit over time. There are radiological abnormalities observed in various body sites of individuals with long COVID, suggesting possible endothelial activation and clotting abnormalities. Autoantibodies and immune subset changes are also found in individuals with long COVID. The current understanding suggests an immunopathogenic etiology of long COVID, but more research is needed for a mechanistic synthesis and effective therapeutic approaches.
NATURE REVIEWS IMMUNOLOGY
(2023)
Review
Hematology
Anna M. Randi, Daisy Jones, Claire Peghaire, Deepa J. Arachchillage
Summary: This review discusses the organotypic differences in the hemostatic profile of endothelial cells, focusing on two major endothelial regulators, von Willebrand factor and thrombomodulin, as examples of transcriptional mechanisms that control heterogeneity.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2023)
Article
Hematology
Deepa J. Arachchillage, Anna Weatherill, Indika Rajakaruna, Mihaela Gaspar, Zain Odho, Graziella Isgro, Lenka Cagova, Lucy Fleming, Stephane Ledot, Mike Laffan, Richard Szydlo, Rachel Jooste, Ian Scott, Alain Vuylsteke, Hakeem Yusuff
Summary: Thrombosis and major bleeding are common complications in V-V ECMO patients and significantly increase mortality. Age and elevated creatinine level are associated with increased mortality, while the type of thrombosis has different effects on mortality.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2023)
Correction
Immunology
Daniel M. Altmann, Emily M. Whettlock, Siyi Liu, Deepa J. Arachchillage, Rosemary J. Boyton
NATURE REVIEWS IMMUNOLOGY
(2023)
