Antiphospholipid syndrome (APS) is a rare autoimmune disease characterized by recurrent arterial and venous thromboembolic events. Renal complications occur in 3 % of patients. Renal artery stenosis is the most common, and APS-related nephropathy is the predominant microvascular complication. APS nephropathy has heterogeneous manifestations ranging from hematuria and non-nephrotic range proteinuria to hypertension and multi-organ failure caused by catastrophic antiphospholipid antibody syndrome. Anticoagulation and thromboprophylaxis are key to management. Immunosuppression has been used with some success but lacks randomized controlled trial validation for their use.

Download full-text PDF

Source
http://dx.doi.org/10.53738/REVMED.2024.20.863.430DOI Listing

Publication Analysis

Top Keywords

antiphospholipid antibody
8
[kidney disease
4
disease antiphospholipid
4
antibody syndrome]
4
syndrome] antiphospholipid
4
antiphospholipid syndrome
4
syndrome aps
4
aps rare
4
rare autoimmune
4
autoimmune disease
4

Similar Publications

Antibodies to β2-glycoprotein I (β2GPI) cause thrombosis in antiphospholipid syndrome, however the role of β2GPI in coagulation in vivo is not understood. To address this issue, we developed β2GPI-deficient mice (Apoh-/-) by deleting exon 2 and 3 of Apoh using CRISPR/Cas9 and compared the development of thrombosis in wild-type (WT) and Apoh-/- mice using rose bengal and FeCl3-induced carotid thrombosis, laser-induced cremaster arteriolar injury, and inferior vena cava (IVC) stasis models. We also compared tail bleeding times and activation of platelets from WT and Apoh-/- mice in the absence and presence of β2GPI.

View Article and Find Full Text PDF

To identify clusters of autoantibodies in a large cSLE population and to verify possible associations between different autoantibody clusters and the following variables: demographic data, cumulative clinical and laboratory manifestations, disease activity, cumulative damage and mortality. A cross-sectional study was performed in 27 Pediatric Rheumatology University centers, including 912 cSLE patients. The frequencies of seven selected autoantibodies (anti-dsDNA, anti-Ro/SSA, anti-La/SSB, anti-Sm, anti-RNP, aCL IgM and/or IgG and LA) were used for cluster analysis using the K-means method.

View Article and Find Full Text PDF

Little is known about how patients with antiphospholipid syndrome (APS) or antiphospholipid antibodies (aPL) access and trust health information. This research aimed to: describe the sources of information most frequently accessed/trusted by patients with APS/aPL; identify if individuals with APS/aPL perceived their health had been negatively impacted by various sources and document obstacles to accessing health information. Patients meeting Revised Sapporo Criteria for APS or with ≥1 positive aPL on ≥2 occasions were recruited to an online survey regarding their health information use at diagnosis and within 6 months preceding survey completion.

View Article and Find Full Text PDF

Systemic lupus erythematosus (SLE) is an inflammatory autoimmune disease with an important course due to systemic compromise. SLE is frequently associated with antiphospholipid syndrome, and pulmonary thromboembolism (PE) is particularly common. It is extremely rare for PE to be the initial clinical presentation and even more uncommon for it to coincide with cardiac tamponade, representing a challenge in diagnosis and management.

View Article and Find Full Text PDF

Inflammation and Coagulation in Neurologic and Psychiatric Disorders.

Semin Thromb Hemost

January 2025

Department of Neurology, Sheba Medical Center, Tel Ha'Shomer, Israel.

Coagulation factors are intrinsically expressed in various brain cells, including astrocytes and microglia. Their interaction with the inflammatory system is important for the well-being of the brain, but they are also crucial in the development of many diseases in the brain such as stroke and traumatic brain injury. The cellular effects of coagulation are mediated mainly by protease-activated receptors.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!