PubMed Health⌕ Search

Biomedical subjects

R Cervera

Publications and source records attributed to R Cervera.

At least 127 records · Page 7Linked to original sources

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↗

Lack of relationship between human immunodeficiency virus infection and systemic lupus erythematosus.

The objective of this work was to determine whether HIV-1 and HIV-2 could be involved in the pathogenesis of systemic lupus erythematosus (SLE). Seventy-five consecutive Caucasian patients with SLE presenting at one institution over a 2-year period were studied. Serum samples were surveyed for anti-HIV-1 antibodies by a commercial ELISA coated with HIV-1-p24. For confirmation, conventional immunoblots were performed with the following antigens: HIV-1-gp41, p31, p24 and p17 (recombinant) and HIV-2-gp36 (synthetic peptide). Additionally, Western blots with HIV-1-gp160, gp120, gp41, p65, p51, p24 and p18 bands were applied. Seventeen (23%) patients exhibited reactivity with HIV-1-p24 in the ELISA, but in the immunoblots and Western blots these sera samples were negative. Patients with SLE may exhibit a reactivity with HIV-1-p24 in the ELISA for HIV infection screening but not in the confirmatory blots. This false-positive reactivity is probably due to molecular mimicry between autoantigens and retroviruses or a contaminant or artefacts in the antigen preparation procedure.

Acquired Immunodeficiency Syndrome↗

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↗

[Early diagnosis of systemic lupus erythematosus. A challenge for clinicians of different specialties].

This paper presents some of the results from a ten year prospective study (Eurolupus) analyzing the main clinical and immunological manifestations in 1,000 patients with systemic lupus erythematosus (SLE). The time lag from the first symptoms to diagnosis was much longer in patients from Norway compared with patients from Europe in general (6.6 years versus two years respectively). In order to improve the early diagnosis of SLE, the authors focus on early clinical symptoms and signs.

Female↗

[Primary antiphospholipid syndrome: clinical and biological study of 36 cases].

BACKGROUND: The aim of this study was to analyze the clinical and biological characteristics and follow up in a series of patients with the primary antiphospholipid syndrome. METHODS: Thirty-six patients were prospectively studied. The antiphospholipid antibodies were determined with the following techniques: IgG and IgM anticardiolipin antibodies by ELISA, lupic anticoagulant by coagulometric tests and serology of syphilis by a reaginic test. RESULTS: The mean age of the patients was of 29.9 years with a female/male relation of 4.1 to 1. The most frequently observed clinical manifestations were miscarriage and fetal death in a total of 22 patients (61%). Thrombotic phenomena were observed in 15 patients (42%) with predominance of venous thrombosis of the deep venous system of the limbs and pulmonary embolism. Thrombocytopenia was detected in 9 cases (25%), autoimmune hemolytic anemia in 3 (8%) and low or moderate titres of antinuclear antibodies in 12 (33%). Other infrequent clinical manifestations were cardiac valve lesions, livedo reticularis and epilepsy. Three patients (8%) had relatives with systemic lupus erythematosus. The antiphospholipid antibody study demonstrated the presence of anticardiolipin antibodies in 28 patients (78%), lupic anticoagulant in 22 (67%) (not determined in 3 patients) and falsely positive test for syphilis in 5 (14%). Patients with thrombosis received anticoagulant therapy, with all evolving favorably except two who died (multiple pulmonary thromboembolism in one case and cerebral hemorrhage in the other) mortality of the series thus being 5.6%. Fourteen women wished to become pregnant following fetal loss and were treated with acetylsalicylic acid in 3 cases together with prednisone (15 to 30 mg/day) with successful pregnancies being achieved in 12 cases (86%). CONCLUSIONS: The primary antiphospholipid syndrome is a well differentiated clinical entity and the presence of antiphospholipid antibodies should be investigated in young people presenting thrombosis or fetal loss for no apparent reason.

Abortion, Spontaneous↗

Comparison of the primary and secondary antiphospholipid syndrome: a European Multicenter Study of 114 patients.

PURPOSE: To determine whether the features of the antiphospholipid syndrome (APS) are in any way influenced by the presence or absence of systemic lupus erythematosus (SLE). We followed up patients with 'primary' APS (PAPS) and APS secondary to SLE (APS plus SLE) with the objective of comparing laboratory and clinical events and of determining whether patients with PAPS would have evolution to SLE. PATIENTS AND METHODS: A total of 114 patients from 3 European referral centers were included in this study. Fifty-six had APS plus SLE and 58 had PAPS. Laboratory and clinical data were collected during an average 2-year period. RESULTS: Patients with PAPS and patients with APS plus SLE had similar clinical and laboratory profiles, with the exceptions of autoimmune hemolytic anemia, endocardial valve disease, neutropenia, and low C4 levels, all of which occurred more frequently in patients with APS plus SLE (p values: < 0.05, < 0.005, < 0.01, and < 0.001, respectively). On follow-up, 10 thrombotic episodes occurred in 10 patients, 8 of whom were receiving anticoagulant therapy. No patient with PAPS had either anti-DNA or anti-extractable nuclear antigen antibodies, and these patients had a significantly lower prevalence of antinuclear antibodies (41%) than patients with APS plus SLE (89%). CONCLUSIONS: Patients with APS plus SLE and PAPS have similar clinical profiles, although heart valve disease, hemolytic anemia, low C4 levels, and neutropenia seem to be more common in patients with APS plus SLE. Patients with APS may develop further thrombotic events despite anticoagulation therapy.

Adult↗

Antibodies to endothelial cells in Behçet's disease: cell-binding heterogeneity and association with clinical activity.

OBJECTIVES: To investigate the prevalence and characteristics of antibodies to endothelial cells (aEC) from large vessel and from microvasculature in a group of patients with Behçet's disease (BD) to determine the relationship of these antibodies with clinical and laboratory features of the disease. METHODS: Thirty patients with BD were prospectively and consecutively studied. The aEC were determined by enzyme-linked immunosorbent assay (ELISA) using endothelial cells derived from human umbilical vein (large vessel) as well as from retroperitoneal adipose tissue (microvasculature). RESULTS: Fifteen patients (50%) had aEC, either directed to large vessel [8(26%) patients] or microvascular [13(43%) patients] endothelial cells. The percentage of active patients was significantly higher in the aEC-positive group [12(80%) patients] compared with the aEC-negative group [5(33%) patients] (p < 0.05). CONCLUSIONS: Patients with BD have a high prevalence of aEC when microvascular endothelial cells are used in the assay. These antibodies seem to be a marker of disease activity in this condition, previously considered as negative for autoantibodies.

Adipose Tissue↗

'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↗

[Systemic lupus erythematosus: a clinical and immunological study of 300 patients].

BACKGROUND: The aims of the present study were to analyze the clinical and immunologic characteristics of a wide group of patients with systemic lupus erythematosus (SLE) and define homogeneous subgroups with their own characteristics. METHODS: A prospective study of 300 patients diagnosed of SLE were studied. These patients were subdivided according to sex, age at the onset of the disease and immunologic profile. The statistical study was carried out by the chi (2), Fisher, Student's t and Mann-Whitney U tests. RESULTS: The series was made up of 266 (89%) females and 34 (11%) males. The mean age at onset of the disease was 31.8 +/- 14.6 years. In 48 (16%) patients the first manifestations appeared after the age of 50. Males were shown to present a lower prevalence of arthritis (59% vs 82% in woman, p < 0.005) and malar rash (29% vs 50%, p < 0.05), but had more cutaneous discoid lesions (18% vs 3% p < 0.001). In patients in whom the disease appeared after the age of 50 a lower prevalence of arthritis was presented (67% vs 82% in patients of less than 50 years of age, p < 0.005), malar rash (23% vs 53%, p < 0.001) and nephropathy (21% vs 41%, p < 0.05), but had greater myositis (17% vs 6%, p < 0.01). The absence of antinuclear antibodies (ANA) and the presence of anti-ds DNA and anti-ENA antibodies were associated with differences in the prevalence of different clinical manifestations. CONCLUSIONS: Sex, age and immunologic pattern in systemic lupus erythematosus permit the definition of homogeneous subgroups with their own characteristics: a) males present a lower prevalence of arthritis and malar rash, but a greater prevalence of cutaneous discoid lesions; b) patients over the age of 50 develop arthritis, malar rash and nephropathy with a lower prevalence but have a greater prevalence of myositis; c) patients without antinuclear antibodies and those with anti-ds DNA and anti-ENA antibodies present differences in the prevalences of different clinical manifestations.

Adolescent↗

Antibodies to endothelial cells in patients with Behçet's disease.

Autoantibodies that bind to endothelial cells have been identified in patients with several forms of vasculitis. Behçet's disease--a multisystem inflammatory disorder of unknown etiology--is associated with thrombosis in addition to systemic manifestations resulting from small and large vessel vasculitis. We studied 72 Turkish patients (33 female, 39 male) with Behçet's disease in order to investigate the prevalence of antiendothelial cell antibodies (AECA) and to examine their possible relationship with clinical and laboratory features of the illness. Sera from 30 healthy Turkish people were used as controls. Human umbilical vein endothelial cells were cultured and used unfixed in a cellular ELISA to detect AECA. IgG and/or IgM AECA were found in 13 (18.1%) patients but not in healthy controls. Antiendothelial cell antibodies did not induce complement-mediated cytotoxicity as assessed by 51Cr release assay and the binding was not due to immune complexes. The prevalences of acute thrombotic events and retinal vasculitis at the time of the AECA assay among patients with AECA were significantly higher than those in patients without AECA. Laboratory parameters of active disease were higher in patients with AECA. There was no correlation between other clinical and laboratory features of Behçet's disease and AECA. Anticardiolipin and antineutrophil cytoplasmic antibodies were negative in our series, excluding a possibility of cross-reaction with AECA. Our results suggest a possible role of AECA in association with thrombosis and vasculitis in patients with Behçet's disease.

Adolescent↗

Systemic lupus erythematosus evolving into systemic vasculitis: a report of five cases.

Five patients with systemic lupus erythematosus are described who developed the disease in their second or third decade. The pattern of their disease then changed markedly and they developed the clinical features of a systemic vasculitis. The possible explanations for this transition of one connective tissue disease to another is discussed.

Adolescent↗

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↗

Calprotectin in patients with systemic lupus erythematosus: relation to clinical and laboratory parameters of disease activity.

Calprotectin (L1) is a granulocyte and monocyte cytosolic protein released during activation of these cells. The plasma level of L1 has been shown to be a good marker of disease activity in rheumatoid arthritis. In this cross-sectional study of 100 patients with systemic lupus erythematosus (SLE), the serum level of L1 was found to be higher in patients than in matched controls (3661 micrograms/l versus 1051 micrograms/l; P < 0.001). The serum level of L1 was the only laboratory parameter with significant association to the disease activity index SLEDAI (r = 0.28; P < 0.01). Furthermore, the serum level of L1 was significantly higher in SLE patients with anti-DNA antibodies compared to patients without anti-DNA antibodies (4501 micrograms/l versus 3279 micrograms/l; P = 0.01). SLE patients with arthritis had higher serum levels of L1 than patients without arthritis (7652 micrograms/l versus 2811 micrograms/l; P < 0.01), indicating that the serum level of L1 also reflects arthritis activity in SLE.

Adult↗

Systemic lupus erythematosus: clinical and immunologic patterns of disease expression in a cohort of 1,000 patients. The European Working Party on Systemic Lupus Erythematosus.

In the present study we have analyzed the prevalence and characteristics of the most relevant clinical and immunologic features in 1,000 patients with SLE. Several differences in the expression of the disease have been observed in relation to the patients' age at onset, sex, and autoantibody serology. The childhood-onset patients more often had malar rashes (55% vs 39%) and nephropathy (28% vs 15%) as presenting manifestations. During the evolution of the disease, these patients had an increased prevalence only of malar rash (79% vs 56%) and a lower prevalence of rheumatoid factor (6% vs 19%). The older-onset patients (age 50 or older) less often showed malar rash (21% vs 42%), arthritis (52% vs 71%), and nephropathy (3% vs 17%) as the first symptom. During the evolution of their disease, these patients had a decreased prevalence of malar rash (33% vs 60%), photosensitivity (29% vs 47%), arthritis (73% vs 85%), nephropathy (22% vs 41%), thrombosis (4% vs 15%), and anti-La antibodies (6% vs 20%), but an increased prevalence of sicca syndrome (33% vs 15%). Males more often had serositis (28% vs 16%) as a first symptom, but they presented with a lower prevalence of arthritis (74% vs 85%) during the evolution of the disease. The presence of ANA, a high titer of anti-dsDNA, rheumatoid factor, anti-ENA, and antiphospholipid antibodies also distinguished additional homogeneous SLE subsets of clinical significance.

Adolescent↗