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)
Review
Immunology
Marc Scheen, Amir Adedjouma, Emmanuel Esteve, David Buob, Noemie Abisror, Virginie Planche, Olivier Fain, Jean Jacques Boffa, Sophie De Seigneux, Arsene Mekinian, Fadi Haidar
Summary: Antiphospholipid antibody syndrome (APLS) is a rare autoimmune disease characterized by recurrent arterial and venous thromboembolic events, pregnancy-related complications, and persistent detection of antiphospholipid antibodies. Renal complications are less common, with renal artery stenosis being the most common kidney-related complication.
AUTOIMMUNITY REVIEWS
(2022)
Review
Immunology
Zelin Yun, Lizhi Duan, Xiangjun Liu, Qingmeng Cai, Chun Li
Summary: Antiphospholipid syndrome (APS) is an autoimmune disease characterized by thrombosis and pregnancy morbidity. Anticoagulation is the primary treatment, but is not always effective. This review explores the potential of biologics, such as rituximab and eculizumab, as targeted treatments for APS management.
FRONTIERS IN IMMUNOLOGY
(2023)
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
Rheumatology
Luca Moroni, Paolo Righini, Giuseppe A. Ramirez, Nicola Farina, Gaia Mancuso, Enrica Bozzolo, Ignasi Rodriguez-Pinto, Ricard Cervera, Giovanni Nano, Lorenzo Dagna
Summary: This case report described aortic occlusion as a debut manifestation of catastrophic antiphospholipid syndrome (CAPS) and discussed its clinical features with a review of the literature to identify risk factors and clues for clinical practice.
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.
Article
Medicine, General & Internal
Jessica Liang, Raai Mahmood, Ilyes Benchaala, Russel York, Housam Sarakbi
Summary: This case report emphasizes the importance of considering catastrophic antiphospholipid syndrome (CAPS) as a potential diagnosis in lupus patients presenting with digital ischemia. Diagnosis and treatment of digital ischemia can be complex in the setting of lupus, and a high index of suspicion for CAPS is essential for early management. CAPS is a life-threatening variant of antiphospholipid syndrome with increased intensity and extent of thrombotic outcomes.
CUREUS JOURNAL OF MEDICAL SCIENCE
(2023)
Article
Clinical Neurology
Federico Massa, Diego Franciotta, Stefano Grisanti, Luca Roccatagliata, Silvia Morbelli, Sabrina Beltramini, Antonio Uccelli, Angelo Schenone, Luana i Benedetti
Summary: In this study, a new immunotherapy protocol using intravenous immunoglobulin cycles was used to treat patients with NMDAR encephalitis. The results showed that this protocol led to significant improvements in patients' conditions without any adverse effects.
NEUROLOGICAL SCIENCES
(2022)
Article
Medicine, General & Internal
Daniela Alferes, Susana Pereira, Vitoria Paes de Faria, Ana Ventura, Maria Clara Almeida
Summary: Catastrophic antiphospholipid syndrome is a rare and life-threatening disorder characterized by multiple thrombotic events involving three or more organs in a short period of time, in the presence of persistent antiphospholipid antibodies. The optimal management of CAPS is unclear with a lack of consensus among experts. We report a case of a patient with primary antiphospholipid syndrome who experienced probable CAPS after receiving rivaroxaban, resulting in extensive cutaneous ulceration, acute coronary syndrome, and dialysis-dependent renal failure. Treatment with anticoagulation, glucocorticoids, and plasmapheresis, along with long-term vitamin K antagonist, resulted in the healing of skin lesions, regression of cardiac lesions, and recovery of renal function after three years on dialysis.
CUREUS JOURNAL OF MEDICAL SCIENCE
(2023)
Review
Medicine, General & Internal
Cristina Stanescu, Andreea Gabriella Andronesi, Ciprian Jurcut, Mihaela Gherghiceanu, Alexandra Vornicu, Florentina Andreea Burcea, Toader Danut Andronesi, Gabriela Elena Lupusoru, Luminita Iliuta, Bogdan Marian Sorohan, Bogdan Obrisca, Gener Ismail
Summary: This case report describes a 61-year-old male with SLE who was diagnosed with CAPS and successfully treated with a combination of low molecular weight heparin, intravenous methylprednisolone, plasmapheresis, and rituximab. Monitoring of CD19+B-lymphocytes may serve as a predictive tool for relapses and guide rituximab therapy in SLE patients with CAPS.
MEDICINA-LITHUANIA
(2021)
Article
Rheumatology
Kayoko Kaneko, Seiji Tsutsumi, Daisuke Fujita, Mayumi Sugiura-Ogasawara, Nobuaki Mitsuda, Keiichi Matsubara, Tatsuya Atsumi, Eisuke Inoue, Tetsuya Takimoto, Atsuko Murashima
Summary: The objective of the study was to compare the efficacy of IVIG therapy for obstetric APS refractory to conventional treatment. The study found that IVIG-only add-on therapy did not improve pregnancy outcomes, but the combination of IVIG with rituximab or statins along with conventional treatment resulted in improved outcomes and higher live birth rates.
MODERN RHEUMATOLOGY
(2023)
Article
Neurosciences
Fabian Boesl, Kristina Allers, Juliane Herm, Thomas Scheider, Christiana Franke
Summary: Progressive multifocal leukoencephalopathy (PML) is a rare demyelinating disease of the CNS caused by the human polyomavirus 2 (JCV) and primarily occurs in immunocompromised patients. There is currently no specific antiviral treatment available, but combined treatment with intravenous immunoglobulins, maraviroc, and pembrolizumab has shown some benefits in PML patients.
JOURNAL OF NEUROVIROLOGY
(2022)
Article
Medicine, General & Internal
Ayrton Bangolo, Sowmya Sagireddy, Sarah Mahamadeen, Felicia Hasta, Sadhu A. Reddy, Afshan Naz, Ravishankar Ranganatha, Cleveland Ricketts, Padmavathi Muppalla, Swathi Veliginti, Georgemar Arana, Dily T. Sathyarajan, Sachin Singh, Tanvi Shetty, Kshitij Bhardwaj, Sayed Hashemy, Roberto L. Duran, Sung H. Kim, Candice M. Hipolito, Kibo Yoon, Vrusha Patel, Aseel Alshimari, Pugazhendi Inban, Saaniya Yasmeen, Simcha Weissman
Summary: This article reports a case of refractory catastrophic antiphospholipid syndrome (CAPS) successfully treated with plasmapheresis and rituximab. It provides insights into the usage of rituximab in the management of CAPS.
JOURNAL OF COMMUNITY HOSPITAL INTERNAL MEDICINE PERSPECTIVES
(2023)
Article
Medicine, General & Internal
Mani Maheshwari, Hemanthkumar Athiraman
Summary: This case report describes a young female with no prior medical history who developed catastrophic antiphospholipid syndrome, a rare and severe disease characterized by small vessel occlusions and multi-organ involvement in the presence of antiphospholipid antibodies.
CUREUS JOURNAL OF MEDICAL SCIENCE
(2023)
Article
Immunology
Changgon Kim, Hyun-Sook Kim
Summary: This article presents a case of successful treatment of catastrophic antiphospholipid syndrome (CAPS) using the rituximab biosimilar CT-P10, which is cost-effective. The patient remained recurrence-free during the 2-year follow-up.
EUROPEAN JOURNAL OF INFLAMMATION
(2022)
Article
Ophthalmology
Lucia Miguel-Escuder, Amanda Garcia Tirado, Maria Sainz-de-la-Maza, Gerard Espinosa, Aina Moll-Udina, Victor Llorenc, Alba Parrado-Carrillo, Alfredo Adan
Summary: This study retrospectively describes three cases of Anti-TNF-alpha-induced lupus (ATIL) in patients with non-infectious uveitis (NIU), who developed antinuclear antibody (ANA) and anti-dsDNA antibody positivity, arthritis, and in one case, skin lesions under adalimumab treatment. The condition resolved in all patients after adalimumab withdrawal, with one patient requiring corticosteroids, two patients requiring non-steroidal anti-inflammatory drugs, and one patient requiring hydroxychloroquine. None of the patients required another immunosuppressive drug. Two patients were able to continue follow-up without anti-TNF-alpha therapy, while the remaining patient switched to another anti-TNF-alpha treatment (golimumab). A review of the literature and comparison with previous ATIL cases was also conducted.
EUROPEAN JOURNAL OF OPHTHALMOLOGY
(2023)
Article
Rheumatology
Ana Matas-Garcia, Alfredo Guillen-Del-Castillo, Boris Kisluk, Albert Selva-O'Callaghan, Gerard Espinosa, Sergio Prieto-Gonzalez, Pedro Moreno Lozano, Gloria Garrabou, Josep Maria Grau-Junyent, Carmen Pilar Simeon-Aznar, Jose C. Milisenda
Summary: This study analyzed the clinico-serological and histological phenotypes of SSc patients with associated myopathy. The patients were divided into two subgroups based on histological findings: fibrosing and inflammatory. Significant differences were found in clinical data, antibody profile, electrophysiologic studies, treatment response, mortality, and survival between the two groups.
Article
Rheumatology
G. Pires da Rosa, E. Ferreira, B. Sousa-Pinto, P. Bettencourt, G. Espinosa, R. Cervera
Summary: This study analyzed a cohort of patients with laboratory criteria for anti-phospholipid syndrome (APS) but only presenting "non-criteria" clinical manifestations. The patients were compared with definite APS patients. The results showed that these "clinical non-criteria" patients had mainly hematological and neurological involvements, with significant treatment use. Some patients may progress to future systemic lupus erythematosus (SLE).
SCANDINAVIAN JOURNAL OF RHEUMATOLOGY
(2023)
Article
Rheumatology
Giuseppe Barilaro, Carlo Della Rocca, Alexandra Esteves, Ricard Cervera, Gerard Espinosa
Summary: This study analyzed the association between the average 'adjusted' Global APS Score (aGAPSS) and the change in Damage Index for APS (DIAPS) in patients with thrombotic and non-thrombotic APS. The results showed that a higher average aGAPSS value was associated with an increase in DIAPS during follow-up, suggesting that the average aGAPSS score can predict the increase in DIAPS.
Editorial Material
Medicine, General & Internal
Giuseppe Barilaro, Ricard Cervera
EUROPEAN JOURNAL OF INTERNAL MEDICINE
(2023)
Article
Rheumatology
Mohamed-Yacine Khitri, Alessandra Bartoli, Georgina Maalouf, Alban Deroux, Carlo Salvarani, Giacomo Emmi, Omer Karadag, Gerard Espinosa, Mathilde Leclercq, Gabriele Simonini, Mathieu Vautier, Patrice Cacoub, David Saadoun
Summary: This study evaluated the efficacy of tocilizumab in patients with refractory Behcet disease. The results showed that 83% of patients responded to tocilizumab, with 60% achieving complete response and 23% achieving partial response. Tocilizumab also significantly reduced steroid dosage and the need for concomitant disease-modifying antirheumatic drugs. However, a small number of patients experienced mild to moderate side effects and serious adverse events.
JOURNAL OF RHEUMATOLOGY
(2023)
Article
Medicine, General & Internal
Isaac Pons, Joana Louro, Marta Sitges, Barbara Vidal, Ricard Cervera, Gerard Espinosa
Summary: This study describes the prevalence, clinical and laboratory features, and evolution of heart valve involvement in patients with APS. The results showed that 50% of APS patients had valve involvement, with most of them being female and having arterial hypertension and arterial thrombosis. The valve involvement group had a higher mortality rate.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Medicine, General & Internal
Albert Perez-Isidro, Marc Xipell, Arturo Llobell, Noemi De Moner, Gema M. Lledo, Ricard Cervera, Sergio Prieto-Gonzalez, Luis F. Quintana, Gerard Espinosa, Mila Garcia-Ormaechea, Estibaliz Ruiz-Ortiz, Odette Vinas
Summary: This study investigated whether dsDNA antibody-secreting B-cells could be a complementary biomarker for monitoring disease activity and prognosis in SLE patients. The results showed that SLE-ELISpot had the best performance in identifying active patients. High SLE-ELISpot results were associated with haematological involvement and increased risk of disease flare-up, especially renal flare. Combining hypocomplementemia and high SLE-ELISpot results further increased these risks. Therefore, adding SLE-ELISpot to the current follow-up protocol can improve clinicians' personalized care decisions.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Rheumatology
Jose Manuel Mascaro, Ignasi Rodriguez-Pinto, Gabriela Poza, Anna Mensa-Vilaro, Julian Fernandez-Martin, Luis Caminal-Montero, Gerard Espinosa, Jose Hernandez-Rodriguez, Marina Diaz, Joana Rita-Marques, Raimon Sanmarti, Santos Castaneda, Dolores Colunga, Ruben Coto-Hernandez, Patricia Fanlo, Jose Ignacio Elejalde, Segundo Bujan, Ignasi Figueras, Francisco Manuel Marco, Mariano Andres, Silvia Suarez, Andres Gonzalez-Garcia, Xavier Fusta-Novell, Clara Garcia-Belando, Ana Granados, Maria Teresa Fernandez-Figueras, Neus Quilis, Maria Orriols-Caba, Ricardo Gomez de la Torre, Maria Cinta Cid, Georgina Espigol-Frigole, Alba Alvarez-Abella, Eztizen Labrador, Maria Rozman, Monica Lopez-Guerra, Paola Castillo, Jose R. Alamo-Moreno, Eva Gonzalez-Roca, Susana Plaza, Virginia Fabregat, Rocio Lara, Esther F. Vicente-Rabaneda, Sonia Tejedor-Vaquero, Giuliana Magri, Nuria Bonet, Manuel Solis-Moruno, Andrea Cerutti, Oscar Fornas, Ferran Casals, Jordi Yague, Juan Arostegui
Summary: Thirty patients with adult-onset autoinflammatory diseases were definitively diagnosed with VEXAS syndrome through genetic analyses. Patients presented with late-onset disease and symptoms including cutaneous lesions, fever, pulmonary manifestations, and arthritis. The UBA1 variants were detected in both hematopoietic and non-hematopoietic tissues.
ANNALS OF THE RHEUMATIC DISEASES
(2023)
Article
Dermatology
Bianca Bergamini, Julien Seneschal, Kristin J. Meyers, Frederick Durand, Ewa Haladyj, Gerard Espinosa, Jacob P. Thyssen
Summary: A total of 117 pregnancies with exposure to baricitinib were analyzed, and the clinical outcomes were found to be comparable to other targeted therapies for immune-mediated diseases or general population.
BRITISH JOURNAL OF DERMATOLOGY
(2023)
Editorial Material
Rheumatology
Gerard Espinosa, Ricard Cervera
Article
Medicine, General & Internal
O. Capdevila, F. Mitjavila, G. Espinosa, L. Caminal-Montero, A. Marin-Ballve, R. Gonzalez Leon, A. Castro, J. Canora, B. Pinilla, E. Fonseca, G. Ruiz-Irastorza, RELES, Autoimmune Dis Study Grp GEAS, Spanish Soc Internal Med
Summary: This study analyzed the characteristics and predictive factors of the use of rituximab and belimumab in patients with systemic lupus erythematosus. Rituximab was mainly used for hemolytic anemia or thrombocytopenia, while belimumab was mostly used for arthritis. Younger age, psychosis, and hemolytic anemia were identified as independent predictors of the use of these biological agents.
MEDICINA-LITHUANIA
(2023)
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)