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Biomedical subjects

R A Asherson

Publications and source records attributed to R A Asherson.

At least 55 records · Page 3Linked to original sources
PubMed1996

Libman-Sacks endocarditis in the antiphospholipid syndrome: immunopathologic findings in deformed heart valves.

OBJECTIVE: To examine the potential immunologic mechanism and involvement of antiphospholipid antibodies in the pathogenesis of heart valve lesions in patients with the antiphospholipid syndrome (APS). METHODS: Immunoperoxidase and immunofluorescence staining methods were used to evaluate 13 heart valve specimens derived from eight patients with the APS, either primary or secondary to systemic lupus erythematosus. Primary antibodies to human immunoglobulins, complement components, serum albumin and a monoclonal anti-idiotypic antibody to human anticardiolipin antibodies (aCL) were employed. Various tissue specimens from a patient with the APS as well as deformed and normal valves from subjects without the APS were used as controls. RESULTS: Linear subendothelial deposition consisting of immunoglobulins with complement components but not of a non-specific serum protein was found in deformed valves from patients with the APS. None of the control valves or tissues disclosed similar deposition. The same pattern and location of staining was obtained by the anti-idiotypic antibody to aCL. A significant amount of IgG immunoglobulins that bound to cardiolipin was eluted from a valve of a patient with secondary APS. CONCLUSION: Deposits of immunoglobulins including aCL, and of complement components, are common in affected valves of patients with primary and secondary APS. Such deposits may be involved in the pathogenesis of valvular lesions.

Adult↗
PubMed1996

The catastrophic antiphospholipid syndrome 1996: acute multi-organ failure associated with antiphospholipid antibodies: a review of 31 patients.

Thirty one patients with antiphospholipid antibodies who developed multi-organ failure ("Catastrophic Antiphospholipid Syndrome") are reviewed. Thirteen suffered from a 'Primary' antiphospholipid syndrome, 13 from defined SLE, 4 from 'lupus-like' disease and one from rheumatoid arthritis. In more than one third precipitating factors were evident (e.g. infections, major/minor surgical procedures, oral contraceptives). Death occurred in 60% of patients from a variety of causes (myocardial failure, ARDS, CNS causes or, often a combination). Disseminated Intravascular Coagulation was present in 8 of 31 patients. Plasmapheresis appeared to be useful in several who had not responded to conventional therapy (e.g. IV heparin, steroids, immunosuppression).

Adolescent↗
PubMed1996

Microstomia does not necessarily mean scleroderma.

Two patients with microstomia in the absence of any features of progressive systemic sclerosis are documented. Follow-up of almost 20 years did not reveal development of any features of this disease. The first patient had systemic lupus erythematosus and the second rheumatoid arthritis/Sjögren's syndrome with a complicating myositis.

Adult↗
PubMed1995

Clinicopathologic correlations of the antiphospholipid syndrome.

Since the original description and definition of the antiphospholipid syndrome (APS), a number of distinct clinical manifestations related to it have appeared in the literature. These include vascular obstruction of both veins and arteries, thrombus formation on the endocardium and its consequences, as well as a group of other conditions where vascular obstructive mechanisms are either incompletely understood or unproven, eg, chorea, avascular necrosis, and pulmonary hypertension. Single vessel (large/medium) involvement or multiple vascular occlusions may cause 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. Rapid chronological occlusive events occurring over days to weeks have been termed the "catastrophic" APS. Most of these complications may be ascribed to the hypercoagulable state of which antiphospholipid antibodies appear either to be "markers" or intimately connected with the highly complex coagulation mechanisms resulting in thrombotic occlusions.

Antiphospholipid Syndrome↗
PubMed1995

Review: antiphospholipid antibodies and the lung.

Pleuropulmonary complications of systemic lupus erythematosus (SLE) occur in 50-70% of patients and include pleuritis, pleural effusions, acute lupus pneumonitis, diffuse interstitial lung disease, atelectasis, diaphragmatic dysfunction and bronchiolitis obliterans. Additionally, a syndrome of acute reversible hypoxemia has recently been documented. This seems to occur in patients hospitalized for exacerbations of SLE and may be due to pulmonary leukoaggregation. It has become clear that other groups of pulmonary complications may be specifically associated with the antiphospholipid antibodies, both in patients with SLE and in those suffering from the "primary" antiphospholipid syndrome. These include pulmonary embolism and infarction, both thromboembolic and perhaps nonthromboembolic pulmonary hypertension, pulmonary arterial thrombosis, pulmonary microthrombosis, adult respiratory distress syndrome, intraalveolar pulmonary hemorrhage, as well as a postpartum syndrome.

Adolescent↗
PubMed1995

Autoantibodies in systemic lupus erythematosus and urticarial vasculitis.

OBJECTIVE: Urticarial vasculitis (UV) is both a primary disorder and a cutaneous vasculitic manifestation in patients with connective tissue diseases. We examined the prevalence of autoantibodies to vascular endothelial cells (aECA) and anti-Clq antibodies in patients with UV. METHODS: ELISA were used to detect aECA and anti-Clq autoantibodies, and we tested for correlation with UV in 4 patient groups: healthy controls, patients with systemic lupus erythematosus (SLE) with and without UV, patients with primary systemic vasculitides with UV, and patients with hypocomplementemic urticarial vasculitis syndrome (HUVS). RESULTS: aECA were detected in 82% of patients with SLE with UV and 70% of patients with HUVS. In contrast, aECA were found in 32% of patients with SLE without UV and 14% of patients with primary UV. Anti-Clq antibodies were present in all patients with HUVS, but in < 20% in the other 3 groups. Antineutrophil cytoplasmic antibodies were detected in only one patient. CONCLUSION: Although the development of UV is likely to be multifactorial, the high prevalence of aECA in HUVS and SLE with UV suggests that this antibody is associated with vasculitis and may have a role in the pathogenesis of UV in these patients.

Adult↗
PubMed1994

'Primary', 'secondary' and other variants of the antiphospholipid syndrome.

Although the APS seen as a PAPS or accompanying SLE essentially manifests the same clinical features, there appears to be distinct differences in the two groups of patients which have been summarized in this article. Additionally, the fact that some patients with Sneddon's syndrome, Trousseau's syndrome, or even Addison's disease, may in reality be examples of aPL-related vascular occlusive events has opened new avenues, not only for identification of these patients, but also for more systematic therapeutic regimens.

Antiphospholipid Syndrome↗
PubMed1993

Antiphospholipid syndrome.

The antiphospholipid antibodies (aPL), namely, the lupus anticoagulant and the anticardiolipin antibodies, are a family of autoantibodies directed predominantly against negatively charged phospholipids. Many studies have confirmed that patients with these antibodies are prone to repeated episodes of thrombosis, fetal losses, and thrombocytopenia. The association of aPL with these clinical events has been termed the antiphospholipid syndrome. Several skin lesions have been found in patients with this syndrome, including livedo reticularis, livedoid vasculitis, thrombophlebitis, cutaneous infarctions and gangrene of digits, ulcerations, lesions resembling vasculitis (nodules, macules), cutaneous necrosis/infarctions, subungual splinter hemorrhages, and, less commonly, discoid lupus and Degos' disease (malignant atrophic papulosis). In this article, we review the main immunologic and clinical aspects of this syndrome with special emphasis on the dermatologic features.

Antiphospholipid Syndrome↗
PubMed1993

Postpartum bilateral renal vein thrombosis in the primary antiphospholipid syndrome.

A young Caucasian woman developed bilateral deep vein thromboses (DVT) while taking the oral contraceptive (OC) in 1984. After a 9-month period of longterm anticoagulation therapy with warfarin, she experienced recurrent DVT 2 weeks following discontinuation of anticoagulation. She was then maintained with warfarin for the following 6 years. Elevated antibodies to cardiolipin (IgG aCL) were first detected in 1988. In 1990, warfarin was switched to aspirin and low molecular weight heparin as she wished to become pregnant. At 24 weeks she developed eclampsia and labor was induced. During the first postpartum week she experienced loin pain, associated with rapidly rising creatinine levels and features of a nephrotic syndrome. An abdominal ultrasound showed bilateral renal vein thrombosis with enlarged kidneys. She was treated with continuous ambulatory peritoneal dialysis (CAPD) for 4 months and her renal function is now recovering. Our patient is another example of the primary antiphospholipid syndrome.

Adult↗