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
Medicine, General & Internal
Toufic Tannous, Claudia Rosso, Jenna Iannuccilli, Karim Tannous, Matthew Keating
Summary: CAPS is a lethal disease with a high mortality rate, requiring anticoagulation and steroids for disease control. Additional treatments such as plasma exchange, IVIG, and monoclonal drugs like rituximab can also be beneficial in refractory cases. Eculizumab has shown disease control in certain case reports.
CUREUS JOURNAL OF MEDICAL SCIENCE
(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
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
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
C. Huang, Y. Zhao, X. Tian, Q. Wang, C. Hu, N. Jiang, S. Zhou, L. Zhang, J. Zhou, C. Wu, J. Li, D. Wu, J. Zhao, M. Li, X. Zeng
Summary: A predictive model for CAPS was developed using indicators such as hypertension, anaemia, elevated LDH, and proteinuria, which can aid in early recognition and intervention to improve prognosis.
CLINICAL AND EXPERIMENTAL RHEUMATOLOGY
(2023)
Article
Cell Biology
Tyler E. James, Leslie J. Martin, Theodore E. Warkentin, Mark A. Crowther
Summary: Catastrophic antiphospholipid syndrome (CAPS) is a rare condition characterized by sudden multiorgan dysfunction caused by thrombosis from antibodies. Treatment includes anticoagulation, corticosteroids, TPE, and IVIG. This case report is the first to document initial failure of IVIG treatment in a CAPS patient with subsequent success with TPE.
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)
Review
Hematology
Shruti Chaturvedi, Evan M. Braunstein, Robert A. Brodsky
Summary: Antiphospholipid syndrome (APS) is an acquired thromboinflammatory disorder characterized by the presence of antiphospholipid antibodies and an increased risk of venous or arterial thrombosis, with a severe form known as catastrophic APS (CAPS). Complement activation via antiphospholipid antibodies can cause cellular injury and promote coagulation, leading to a potential increase in risk for development of severe thrombotic APS and CAPS in a subset of patients with germline variants in genes crucial for complement regulation. Complement inhibition may be a promising therapy to reduce morbidity and mortality in these patients.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2021)
Article
Rheumatology
Jozelio Freire de Carvalho, Felipe Freire Silva, Yehuda Shoenfeld
Summary: This article describes a rare case of a young patient with cAPS who later developed SLE. Treatment included methylprednisolone, heparin, and immunoglobulin, leading to a good outcome and discharge from the hospital. The patient was later diagnosed with SLE and successfully treated with prednisone, azathioprine, and hydroxychloroquine, currently managing symptoms well with these medications.
Review
Ophthalmology
Nathalie Morel, Clemence Bonnet, Hanane Mehawej, Veronique Le Guern, Laurent Perard, Mathilde Roumier, Antoine Brezin, Bertrand Godeau, Julien Haroche, Ygal Benhamou, Marc Lambert, Cecile M. Yelnik, Nicolas Maillard, Bahram Bodaghi, Jean-Charles Piette, Nathalie Costedoat-Chalumeau
Summary: The study focused on posterior ophthalmic manifestations of catastrophic antiphospholipid syndrome, with bilateral retinal vascular occlusion being the main feature leading to poor visual prognosis. Choroidopathy and retinal vasculitis were also observed, but with better outcomes. Permanent visual loss was a common outcome in nearly half of the patients.
RETINA-THE JOURNAL OF RETINAL AND VITREOUS DISEASES
(2021)
Article
Ophthalmology
Nathalie Morel, Clemence Bonnet, Hanane Mehawej, Veronique Le Guern, Laurent Perard, Mathilde Roumier, Antoine Brezin, Bertrand Godeau, Julien Haroche, Ygal Benhamou, Marc Lambert, Cecile M. Yelnik, Nicolas Maillard, Bahram Bodaghi, Jean-Charles Piette, Nathalie Costedoat-Chalumeau
Summary: The study described the posterior ophthalmic manifestations of catastrophic antiphospholipid syndrome, which mainly included bilateral retinal vascular occlusion with poor visual prognosis, followed by choroidopathy and retinal vasculitis. Permanent visual loss was common, with nearly half of the patients experiencing it after a median follow-up of 14 months.
RETINA-THE JOURNAL OF RETINAL AND VITREOUS DISEASES
(2021)
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)
Review
Obstetrics & Gynecology
Marta Fuentes Carrasco, Alejandra Mayoral Triana, Ignacio Cristobal Cristobal Garcia, Noelia Perez Perez, Nuria Izquierdo Mendez, Patricia Soler Ruiz, Virginia Gonzalez Gonzalez
Summary: Catastrophic antiphospholipid syndrome (CAPS) is a severe form of APS, with a case study involving a 33-week pregnant patient who underwent a cesarean section due to probable CAPS, and both the mother and baby were discharged within a week with clinical improvement. The current literature review and updated perspectives on CAPS during pregnancy were also provided.
EUROPEAN JOURNAL OF OBSTETRICS & GYNECOLOGY AND REPRODUCTIVE BIOLOGY
(2021)
Article
Clinical Neurology
Renato Mantegazza, Gil Wolfe, Srikanth Muppidi, Heinz Wiendl, Kenji P. Fujita, Fanny L. O'Brien, Heather D. E. Booth, James F. Howard
Summary: The study showed that eculizumab helped patients with anti-acetylcholine receptor-positive refractory generalized myasthenia gravis achieve rapid and sustained minimal manifestations (MM) status, supporting its use in this previously difficult-to-treat patient population.
Article
Rheumatology
C. M. Yelnik, G. Urbanski, E. Drumez, C. Caron, H. Maillard, S. Morell-Dubois, S. Dubucquoi, D. Launay, E. Hachulla, P. Y. Hatron, A. Duhamel, M. Lambert
Article
Hematology
Sebastien Miranda, Veronique Le Cam-Duchez, Jacques Benichou, Nathalie Donnadieu, Virginie Barbay, Maelle Le Besnerais, Francois-Xavier Delmas, Antoine Cuvelier, Herve Levesque, Ygal Benhamou, Guillaume Armengol
THROMBOSIS RESEARCH
(2017)
Article
Hematology
Paul Billoir, Sebastien Miranda, Louise Damian, Vincent Richard, Ygal Benhamou, Veronique Le Cam Duchez
THROMBOSIS RESEARCH
(2018)
Article
Multidisciplinary Sciences
Sebastien Miranda, Paul Billoir, Louise Damian, Pierre Alain Thiebaut, Damien Schapman, Maelle Le Besnerais, Fabienne Jouen, Ludovic Galas, Herve Levesque, Veronique Le Cam-Duchez, Robinson Joannides, Vincent Richard, Ygal Benhamou
Review
Hematology
Aurelien Delluc, Sebastien Miranda, Paul den Exter, Martha Louzada, Adriano Alatri, Shin Ahn, Manuel Monreal, Alok Khorana, Menno Huisman, Philip S. Wells, Marc Carrier
Review
Hematology
Marc A. Rodger, Sebastien Miranda, Aurelien Delluc, Marc Carrier
THROMBOSIS RESEARCH
(2019)
Letter
Hematology
Sebastien Miranda, Ygal Benhamou, Philip Wells, Marc Carrier
THROMBOSIS AND HAEMOSTASIS
(2019)
Letter
Medicine, General & Internal
Cecile M. Yelnik, Yann Nguyen, Veronique Le Guern, Eric Hachulla, Marc Lambert
EUROPEAN JOURNAL OF INTERNAL MEDICINE
(2020)
Article
Hematology
Sebastien Miranda, Junghyun Park, Gregoire Le Gal, Siavash Piran, Shemina Kherani, Marc A. Rodger, Aurelien Delluc
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2020)
Article
Rheumatology
S. Miranda, P. Billoir, M. Le Besnerais, R. Joannides, V Richard, H. Levesque, G. Armengol, J. Bellien, Y. Benhamou
Article
Peripheral Vascular Disease
Simon Soudet, Alexandre Le Joncour, Thomas Quemeneur, Helene Maillard, Fabien Koskas, Eric Hachulla, Patrice Cacoub, Cecile M. Yelnik, David Saadoun, Marc Lambert
Review
Critical Care Medicine
Thomas Moumneh, Philip S. Wells, Sebastien Miranda
CRITICAL CARE CLINICS
(2020)
Article
Rheumatology
Cecile M. Yelnik, Claire Martin, Emmanuel Ledoult, Sebastien Sanges, Vincent Sobanski, Meryem Farhat, Sandrine Morell-Dubois, Helene Maillard, Elodie Drumez, David Launay, Eric Hachulla, Marc Lambert
Summary: This study found that dyslipidemia is common in APS patients and is often insufficiently treated, especially in patients with a history of cardiovascular disease, who have the highest risk of future cardiovascular events.
