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JOURNAL ARTICLE
Sofia Romero-Santos, Daniela Parra-Tanoux, Carlos Cifuentes-González, Juliana Muñoz-Ortiz, Germán Mejía-Salgado, Alejandra de-la-Torre
PURPOSE: To present an atypical case of severe bilateral ocular toxoplasmosis with systemic involvement that initially mimicked an autoimmune etiology, posing challenges to its diagnosis and treatment. CASE REPORT: A 39-year-old immunocompetent male was admitted to the hospital due to a presumed pulmonary thromboembolism concomitant with an abrupt onset of vision loss. Initial differential diagnoses included antiphospholipid syndrome and systemic lupus erythematosus, prompting the administration of corticosteroid pulses and rituximab...
April 9, 2024: Ocular Immunology and Inflammation
#2
Laxman Wagle, Dhiraj R Regmi, Samaj Adhikari, Parmartha Basnyat, Abhishek Kalla
Splenic marginal zone lymphoma (SMZL) usually presents with splenomegaly or symptoms related to cytopenia. We report a case of a 56-year-old female with previously diagnosed antiphospholipid syndrome (APS) on warfarin therapy who initially presented with abdominal pain and was found to have massive splenomegaly and splenic infarction on CT imaging. Initial clinical presentations and imaging findings were attributed to the subtherapeutic coagulation profile. The patient was later diagnosed with SMZL following workup for pancytopenia including bone marrow biopsy, flow cytometry, and PET scan...
March 2024: Curēus
#3
REVIEW
Ignasi Rodriguez-Pintó, Gerard Espinosa, Ricard Cervera
Catastrophic antiphospholipid syndrome (CAPS) is a severe condition with high mortality. Since its description in 1992, an important effort has been made to improve and disseminate knowledge on CAPS. Most of our current knowledge comes from the studies performed using the CAPS Registry, a database created in 2000 to gather as many cases as possible in order to better define this disease. It has demonstrated that this condition has multiple faces and is often triggered by a precipitating factor that leads to a thrombotic microangiopathy and cytokine storm involving almost any organ of the body...
February 6, 2024: Rheumatology
#4
JOURNAL ARTICLE
Sushmita Mittal, Fizah S Chaudhary, Tun Tun Aung, Akram Babkir
BACKGROUND Catastrophic antiphospholipid syndrome (CAPS) is a rare, life-threatening form of antiphospholipid syndrome characterized by widespread thrombotic complications leading to multiorgan ischemia and failure. Although there are no standard treatment guidelines for CAPS, it often involves triple therapy with anticoagulation, corticosteroids, and plasma exchange. Recently, biologics such as rituximab and eculizumab have also shown promise as potential new therapies for CAPS, as observed in our case. CASE REPORT We describe a 59-year-old female patient who presented with altered mental status and diffuse weakness...
January 28, 2024: American Journal of Case Reports
#5
JOURNAL ARTICLE
Senem Tekeoglu, Duygu Temiz Karadag, Ozlem Ozdemir Isik, Ayten Yazici, Ayse Cefle
OBJECTIVE: This retrospective study aimed to conduct a comprehensive analysis of Turkish Systemic Lupus Erythematosus (SLE) patients of Caucasian ethnicity, focusing on their clinical, immunological, and therapeutic characteristics, damage accural and mortality. PATIENTS AND METHODS: We carried out a retrospective assessment of 300 SLE patients diagnosed between 2001 and 2017 at Kocaeli University Rheumatology Clinic. Demographic data, clinical manifestations, immunological profiles, treatment approaches, disease-related damage, and survival information were collected...
March 2024: Lupus
#6
REVIEW
Yunhong Ma, Shuangyi Wang, Fei Luo, Yuan Zhang, Juanjuan Diao
Lupus nephritis (LN) is a complication of systemic lupus erythematosus and a damaging disease of the kidney. The injury of LN in children is more serious than that in adults. However, the literature in this field is numerous and complex, which brings great challenges for researchers to extract information. The purpose of this study is to carry out bibliometric analysis and visualization of published literatures, and identify current research hotspots and future research trends in this field. Literature was retrieved from the Web Of Science database from 1999 to 2022...
January 5, 2024: Medicine (Baltimore)
#7
Denis Ruzdija, Aaron Bertolo, Shaunak K Pandya, Akshay Amaraneni
Diffuse alveolar hemorrhage is a rare complication of antiphospholipid syndrome with high mortality rates. Early diagnosis and treatment with steroids and immunosuppressive agents, along with the achievement of hemostasis and hemodynamic stability, is critical to improving outcomes. This case demonstrates the complexity of managing refractory diffuse alveolar hemorrhage in a 48-year-old woman with antiphospholipid syndrome requiring treatment with high-dose corticosteroids, rituximab, and cyclophosphamide.
October 2023: Curēus
#8
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
A small subset of patients with antiphospholipid syndrome (APS) may develop widespread thrombotic disease with organ damage, referred to as catastrophic APS (CAPS) that is associated with a high mortality. Medical therapy typically involves a combination of anticoagulation, systemic glucocorticoids, plasmapheresis, and intravenous immune globulin (IVIG). There is currently no consensus for the management of refractory cases of CAPS. However, monoclonal antibodies such as rituximab and eculizumab have shown some benefits...
2023: Journal of Community Hospital Internal Medicine Perspectives
#9
JOURNAL ARTICLE
Antonis Fanouriakis, Myrto Kostopoulou, Jeanette Andersen, Martin Aringer, Laurent Arnaud, Sang-Cheol Bae, John Boletis, Ian N Bruce, Ricard Cervera, Andrea Doria, Thomas Dörner, Richard A Furie, Dafna D Gladman, Frederic A Houssiau, Luís Sousa Inês, David Jayne, Marios Kouloumas, László Kovács, Chi Chiu Mok, Eric F Morand, Gabriella Moroni, Marta Mosca, Johanna Mucke, Chetan B Mukhtyar, György Nagy, Sandra Navarra, Ioannis Parodis, José M Pego-Reigosa, Michelle Petri, Bernardo A Pons-Estel, Matthias Schneider, Josef S Smolen, Elisabet Svenungsson, Yoshiya Tanaka, Maria G Tektonidou, Yk Onno Teng, Angela Tincani, Edward M Vital, Ronald F van Vollenhoven, Chris Wincup, George Bertsias, Dimitrios T Boumpas
OBJECTIVES: To update the EULAR recommendations for the management of systemic lupus erythematosus (SLE) based on emerging new evidence. METHODS: An international Task Force formed the questions for the systematic literature reviews (January 2018-December 2022), followed by formulation and finalisation of the statements after a series of meetings. A predefined voting process was applied to each overarching principle and recommendation. Levels of evidence and strengths of recommendation were assigned, and participants finally provided their level of agreement with each item...
January 2, 2024: Annals of the Rheumatic Diseases
#10
Jessica Liang, Raai Mahmood, Ilyes Benchaala, Russel York, Housam Sarakbi
This case report aims to highlight the importance of keeping catastrophic antiphospholipid syndrome (CAPS) high on the list of differentials in patients with lupus who present with digital ischemia and to understand the workup and treatment of the disease. Catastrophic antiphospholipid syndrome is a life-threatening variant of antiphospholipid syndrome (APS), and it is distinguished on the APS spectrum by its increased intensity and extent of thrombotic outcomes. Less than 1% of patients with APS develop CAPS and the demographic of patients affected are primarily females, 37 ± 14 years old, and have underlying primary APS or systemic lupus erythematosus (SLE)...
August 2023: Curēus
#11
Tara L Gallant, Emily Zheng, Alicia M Hobbs, Alexander J Becka, Ruth A Bertsch
Thrombotic microangiopathy (TMA) is a range of diseases characterized by thrombocytopenia, microangiopathic hemolytic anemia, and organ injury. Complement-mediated TMA is a rare, life-threatening subtype of TMA that occurs due to the uncontrolled activation of the alternative complement pathway in the absence of normal regulation, often resulting from deficiencies of various regulatory proteins. Anti-glomerular basement membrane (anti-GBM) disease, previously known as Goodpasture syndrome, is a life-threatening form of vasculitis in which immunoglobulin G autoantibodies bind to the alpha-3 chain of type IV collagen in alveolar and glomerular basement membranes...
July 2023: Curēus
#12
JOURNAL ARTICLE
Stanley Niznik, Micha J Rapoport, Orly Avnery, Mona Kidon, Ronen Shavit, Martin H Ellis, Nancy Agmon-Levin
OBJECTIVES: Antiphospholipid syndrome (APS)-associated heart valve disease (HVD) is well described. Nonetheless, limited data exist on clinical parameters associated with the course of primary APS (pAPS) patients with HVD. The goal of this study was to assess clinical features and related outcomes in patients with APS associated HVD. METHODS: In this multicentre retrospective study, we identified 33 pAPS patients with HVD (pAPS-HVD group) and compared their clinical course with 128 pAPS patients with normal heart valves on echocardiography (pAPS-control group)...
August 12, 2023: Rheumatology
#13
REVIEW
Luca Depietri, Maria Rosaria Veropalumbo, Maria Cristina Leone, Angelo Ghirarduzzi
Antiphospholipid syndrome (APS) is a systemic autoimmune disorder clinically characterized by recurrent arterial and venous thrombosis and/or pregnancy morbidity in the presence of antiphospholipid antibodies. Currently, treatment is mainly focused on anticoagulation, but therapies targeting mechanisms involved in APS autoimmune pathogenesis could play an important role in specific settings. An evidence-based therapeutic approach is limited by the broad clinical spectrum of the syndrome and the nature of a "rare disease" that makes it difficult to carry out well-designed prospective studies...
August 12, 2023: Cardiovascular Drugs and Therapy
#14
JOURNAL ARTICLE
Lauren J He, Laarni C Quimson, Oluwakemi Onajin, Kimberly C Trotter
BACKGROUND: Systemic lupus erythematosus is an autoimmune disease that can have cutaneous and systemic manifestations. Lupus panniculitis, also known as lupus mastitis, is a subset of chronic cutaneous lupus erythematosus that involves inflammation of the subcutaneous fat. The pathogenesis of lupus mastitis is not fully understood. Diagnosis involves a combination of skin manifestations, imaging, and pathologic confirmation. Treatment typically includes steroids and antimalarials, with more severe disease requiring additional immunosuppressive medications...
August 9, 2023: Journal of Medical Case Reports
#15
Mani Maheshwari, Hemanthkumar Athiraman
A very rare and severe disease catastrophic antiphospholipid syndrome is defined by small vessel occlusions resulting in multi-organ involvement in the presence of antiphospholipid antibodies. This case report presents a case of catastrophic antiphospholipid syndrome in a young female without past medical history.
July 2023: Curēus
#16
JOURNAL ARTICLE
Sanober Nusrat, Sayani Tewari, Osman Khan
Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) is a rare acquired bleeding disorder secondary to development of antibodies against prothrombin protein, in the presence of antiphospholipid antibodies. We describe the case of a 13-year-old girl who presented with severe menorrhagia and symptomatic anemia. Labs indicated anemia, thrombocytopenia, elevated PT and aPTT, high-titer inhibitor on mixing studies, positive ANA and anti-dsDNA antibodies, along with a triple-positive antiphospholipid antibody panel...
July 17, 2023: Thrombosis Journal
#17
JOURNAL ARTICLE
Kimberley Youkhana, Hilary Heiling, Allison Deal, Stephan Moll
No abstract text is available yet for this article.
July 2023: TH Open: Companion Journal to Thrombosis and Haemostasis
#18
JOURNAL ARTICLE
Youao Zhang, Zixuan Jia, Xu Xia, Jieyan Wang
BACKGROUND: Many studies have shown an association between COVID-19 and autoimmune diseases (ADs). Studies on COVID-19 and ADs have also increased significantly, but there is no bibliometric analysis to summarize the association between COVID-19 and ADs. The purpose of this study was to perform a bibliometric and visual analysis of published studies related to COVID-19 and ADs. METHODS: Based on the Web of Science Core Collection SCI-Expanded database, we utilize Excel 2019 and visualization analysis tools Co-Occurrence13...
July 3, 2023: Clinical and Experimental Medicine
#19
JOURNAL ARTICLE
Kayoko Kaneko, Seiji Tsutsumi, Daisuke Fujita, Mayumi Sugiura-Ogasawara, Nobuaki Mitsuda, Keiichi Matsubara, Tatsuya Atsumi, Eisuke Inoue, Tetsuya Takimoto, Atsuko Murashima
OBJECTIVES: To compare the efficacy of intravenous immunoglobulin (IVIG) therapy for obstetric antiphospholipid syndrome (APS) refractory to conventional treatment. METHODS: We conducted a single-arm and open-label multicentre clinical intervention trial. The enrolled criteria were patients with refractory APS who had a history of still or premature birth before 30 weeks of gestational age, even though they had been treated with conventional treatment, i.e., heparin and low-dose aspirin...
June 21, 2023: Modern Rheumatology
#20
REVIEW
Zelin Yun, Lizhi Duan, Xiangjun Liu, Qingmeng Cai, Chun Li
Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by thrombosis and pregnancy morbidity with the persistent presence of antiphospholipid antibodies (aPLs). Although anticoagulation is the primary treatment for APS, it fails in approximately 20-30% of obstetric APS cases and more than 30% of thrombotic APS cases. Therefore, there is a need for new, targeted treatments beyond anticoagulants. Biologics, such as rituximab and eculizumab, have been recommended for refractory catastrophic APS...
2023: Frontiers in Immunology
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