Unusual manifestations of the antiphospholipid syndrome.

Clin Rev Allergy Immunol

Rheumatic Diseases Unit, Department of Medicine, University of Cape Town School of Medicine, Cape Town, South Africa.

Published: August 2003

AI Article Synopsis

  • The antiphospholipid syndrome (APS) typically presents with various forms of thrombosis, fetal losses, and low platelet counts, all marked by the presence of antiphospholipid antibodies.
  • Deep vein thrombosis and cerebrovascular accidents are common manifestations, with a range of additional symptoms affecting different organ systems.
  • The article also discusses less common clinical features of APS, including conditions like Sneddon's syndrome and Budd-Chiari syndrome, which occur in a small percentage of patients.

Article Abstract

The classical clinical picture of the antiphospholipid syndrome (APS) is characterized by venous and arterial thromboses, fetal losses and thrombocytopenia, in the presence of antiphospholipid antibodies (aPL), namely lupus anticoagulant (LA), anticardiolipin antibodies (aCL), or antibodies to the protein "cofactor" b2 glycoprotein I. Single vessel involvement or multiple vascular occlusions may give rise to a wide variety of presentations. Any combination of vascular occlusive events may occur in the same individual and the time interval between them also varies considerably from weeks to months or even years. Deep vein thrombosis, sometimes accompanied by pulmonary embolism, is the most frequently reported manifestation in this syndrome. Cerebrovascular accidents-either stroke or transient ischemic attacks-are the most common arterial thrombotic manifestations. Early and late fetal losses, premature births and pre-eclampsia are the most frequent fetal and obstetric manifestations. Additionally, several other clinical features are relatively common in these patients, i.e., thrombocytopenia, livedo reticularis, heart valve lesions, hemolytic anemia, epilepsy, myocardial infarction, leg ulcers, and amaurosis fugax. However, a large variety of other clinical manifestations have been less frequently described in patients with the APS, with prevalences lower than 5%. These include, among others, large peripheral or aortic artery occlusions, Sneddon's syndrome, chorea, transverse myelopathy, intracardiac thrombus, adult respiratory distress syndrome, renal thrombotic microangiopathy, Addison's syndrome, Budd-Chiari syndrome, nodular regenerative hyperplasia of the liver, avascular necrosis of the bone, cutaneous necrosis or subungual splinter hemorrhages. In this article, some of these "unusual" manifestations are reviewed.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7101991PMC
http://dx.doi.org/10.1385/CRIAI:25:1:61DOI Listing

Publication Analysis

Top Keywords

antiphospholipid syndrome
8
fetal losses
8
syndrome
7
unusual manifestations
4
manifestations antiphospholipid
4
syndrome classical
4
classical clinical
4
clinical picture
4
picture antiphospholipid
4
syndrome aps
4

Similar Publications

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with a complex etiology primarily linked to abnormalities in B lymphocytes within the human body, resulting in the production of numerous pathogenic autoantibodies. Telitacicept is a relatively novel humanized, recombinant transmembrane activator, calcium modulator and cyclophilin ligand interactor fused with the Fc portion (TACI-Fc). It works by competitively inhibiting the TACI site, neutralizing the activity of B-cell lymphocyte stimulator and A proliferation-inducing ligand.

View Article and Find Full Text PDF

Chronic subdural hematoma is a common condition in neurosurgical practice. It is usually treated by burr-hole surgery. Patients with coagulopathies such as antiphospholipid syndrome, are at increased risk of complications, and careful consideration of the patient's specific risk of both bleeding and thromboembolic complications must guide medical management.

View Article and Find Full Text PDF

Impact of Lupus Anticoagulant on INR Using Recombinant Prothrombin Time Reagent.

Am J Case Rep

January 2025

Department of Clinical Diagnostic Laboratories, Hospital Al-Sultan Abdullah, Puncak Alam, Selangor, Malaysia.

BACKGROUND Lupus anticoagulants (LA) can interfere with routine coagulation tests such as the activated partial thromboplastin time (aPTT) and prothrombin time (PT). The international normalized ratio (INR) is derived from PT and is used to monitor warfarin therapy. A positive LA result is one of the laboratory criteria of the 2023 ACR/EULAR antiphospholipid syndrome (APS) classification criteria.

View Article and Find Full Text PDF

Introduction: The incidence of cognitive compromise in systemic lupus erythematosus is variable; it presents early and is usually asymptomatic. Our study evaluated the frequency of cognitive impairment in patients without a previous diagnosis of neuropsychiatric lupus and compared the differences in intracerebral size in subgroups with cognitive alterations and positive autoantibodies.

Methods: This is a cross-sectional study.

View Article and Find Full Text PDF

Infective endocarditis causing acute aortic occlusion in a patient with systemic lupus erythematosus: A rare case report.

Int J Surg Case Rep

January 2025

Department of Vascular Surgery, Royal Perth Hospital, Perth 6000, Australia; University of Western Australia, School of Surgery, Perth 6000, Australia. Electronic address:

Introduction: We present a unique case of acute aortic occlusion secondary to infective endocarditis (IE).

Presentation Of Case: An Aboriginal Australian woman with systemic lupus erythematosus presented with fever, confusion, tachycardia, and tachypnoea and had cold, pulseless, insensate, and paralysed lower limbs. Computed tomography angiography revealed multifocal occlusion of the distal aorta and lower limb vessels.

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!