Search

Abstract This article reviews the evolution of the concept of Hughes syndrome starting with the discovery of beef heart extract-reactive antibodies in sera of patients with syphilis, presence of such antibodies in some normal individuals who went on to develop systemic lupus erythematosus (SLE), identification of the antigen as anionic phospholipids and some of the cofactors of the blood coagulation cascade, development of more refined techniques for detecting such antibodies and finally its implication in the causation of an acquired hypercoagulable state. The review then discusses the results of extensive international collaborative efforts of the major centres with interest and expertise in Hughes syndrome. It summarizes the recently enunciated classification criteria for this disease. The results and the implications of some of the recent seminal papers, especially on the frequency of different clinical manifestations in a large cohort of patients followed over long-periods, and recommendations for long-term high-intensity anticoagulation for the optimum management of this disease, are also discussed. [ABSTRACT FROM AUTHOR]

Presents views on progress in rheumatology. Techniques to better diagnosis of vasculitis and antiphospholipid syndrome; Mechanism of action of nonsteroidal anti-inflammatory drugs; Discovery of glucocorticoids.

To describe the risk factors for stroke among Filipino patients with systemic lupus erythematosus (SLE). This study retrospectively reviewed the medical records of Filipino patients with SLE seen at the Section of Rheumatology, Clinical Immunology and Osteoporosis of the University of Santo Tomas Hospital from 1998 to 2003. Assessment was made on the presence of known risk factors for stroke including age, hypertension, diabetes mellitus, dyslipidemia, personal or family history of coronary artery disease, family history of stroke, and SLE-related factors such as antiphospholipid syndrome (APS) and nephrotic syndrome. Of a total of 449 patients, 25 recorded 26 stroke events during the course of their SLE. Twenty-four female patients and one male had a mean age at stroke occurrence of 38.08 ± 15.45 SD years, with a mean duration from SLE diagnosis to stroke event of 62.48 ± 54.58 SD months. Infarction was implicated in 19 of 26 (73%) events, four (16%) had transient ischemic attack, two (8%) had intracerebral haemorrhage, and one had amaurosis fugax. Eighteen (72%) had systemic arterial hypertension, three (12%) had diabetes mellitus, and nine (36%) had dyslipidemia. A significant family or personal history was present in eight (32%), and there were no smokers. Twenty-one patients were on corticosteroid treatment with an average prednisone dose of 13.02 ± 10.95 SD mg/day. Nine (36%) were on immunosuppressive therapy prior to stroke, and two were on hydroxychloroquine. APS was diagnosed in three (12%), four (16%) each had nephrotic syndrome and extra-CNS vasculitis. Hypocomplementemia was present in six (24%). Hypertension, dyslipidemia, and family or personal history were the predominant risk factors in this series, although SLE-related factors also possibly contributed to the occurrence of stroke, particularly in the younger patients. [ABSTRACT FROM AUTHOR]

Systemic lupus erythematosus (SLE) is the autoimmune disease that most commonly compromises pregnancy. Moreover, the relationship between SLE and pregnancy is in both directions. However, the current experience indicates that pregnancy in patients with SLE should not be regarded as an unacceptable high risk condition for the mother or her baby provided that careful planning of conception and multidisciplinary monitoring and treatment are carried out. [ABSTRACT FROM AUTHOR]

The term‘catastrophic’ antiphospholipid syndrome (CAPS) is defined as an accelerated form of antiphospholipid syndrome (APS) usually resulting in multiorgan failure. These patients have in common: (i) clinical evidence of multiple organ involvement developing in a very short time period; (ii) histopathological evidence of multiple small vessel occlusions (a minority also have large vessel thrombosis); and (iii) laboratory confirmation of the presence of antiphospholipid antibodies (aPL), usually in high titres. Although less than 1% of patients with the APS develop this complication, its potentially lethal outcome emphasizes its importance in clinical medicine. [ABSTRACT FROM AUTHOR]

Discusses the autoimmune aspects of accelerated atherosclerosis in rheumatology. Information on atherosclerosis in rheumatoid arthritis; Role of atherosclerosis in systemic lupus erythematosus; Involvement of autoimmune rheumatic diseases with enhanced expression of atherosclerosis.

Abstract Background: There is a paucity of studies on antiphospholipid syndrome (APS) in Asian patients. Aim: This is the first study in Singapore to describe the clinical features, serological markers and outcomes of one of the largest cohort of APS patients in South-east Asia. Method: One hundred and forty-six patients were studied. Results: Within the study group, 89 were primary APS and 57 secondary APS. Age range: 16–84 years (mean Age: 50.1 years). Male : female ratio: 1 : 2.1. Racial distribution: Chinese, 69.2%; Malays, 18.5%; Indians, 11.7%. Systemic lupus erythematosus was the commonest disease associated in 48 patients (32.9%), while 17 (11.6%) had lupus-like disease. Eighty-two (56.2%) patients had arterial thrombosis, 37 (25.3%) patients had venous thrombosis, 14 (9.6%) had arterial and venous thrombosis while 17 (11.6%) had obstetric manifestations; 28.8% had thrombocytopenia. Clinical manifestations were diverse with neurological (49.3%), cardiac (40%), renal (23.6%) involvement. There were also several unusual presentations. Recurrent thrombosis occurred in 24.6%. Two had catastrophic APS and there were 12 deaths (9.6%). Antiphospholipid antibody testing included lupus anticoagulant, serum aCL IgG, IgM and IgA, anti-β2 glycoprotein I (aβ2-GPI) IgG, IgM, and IgA. The serological positivity rates of the various antiphospholipid antibodies were reported. aβ2-GPI was positive in 67% of those tested, of which aβ2-GPI IgA was the most common subtype. The presence of the aβ2-GPI was associated with recurrent venous thrombosis. Conclusion: The clinical manifestations of APS in Asian patients were diverse and multisystemic. Recurrent thrombosis rate was high with significant morbidity and mortality. [ABSTRACT FROM AUTHOR]