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Biomedical subjects

R Cervera

Publications and source records attributed to R Cervera.

At least 73 records · Page 4Linked to original sources
PubMed2000

The 4G/5G polymorphism of the type 1 plasminogen activator inhibitor gene and thrombosis in patients with antiphospholipid syndrome.

OBJECTIVE: To investigate the relationship between the 4G/5G polymorphism of the type 1 plasminogen activator inhibitor (PAI-1) gene and thrombotic manifestations in patients with antiphospholipid syndrome (APS). METHODS: We studied a total of 247 patients included in the following 4 groups: 70 patients with primary APS, 104 patients with systemic lupus erythematosus (40 with antiphospholipid antibodies [aPL] and clinical [secondary] APS, 13 with aPL but without clinical APS, and 51 with neither detectable aPL nor a history of thrombosis), 14 asymptomatic individuals with aPL, and 59 patients with thrombosis but without known thrombosis risk factors. A control group of 100 healthy individuals was also analyzed. PAI-1 4G/5G polymorphism was determined by polymerase chain reaction and endonuclease digestion. RESULTS: The allele frequency of 4G/5G in controls was 0.47/0.53. There were no differences in allele distribution among patient groups or between patients and controls. However, a higher frequency of the 4G allele was observed in APS patients with versus those without thrombosis (0.57 versus 0.39; P < 0.05) (odds ratio [OR] 2.83, 95% confidence interval [95% CI] 1.18-6.76). This higher frequency of the 4G allele was attributable to the higher frequency in patients with versus those without arterial thrombosis (0.64 versus 0.43; P < 0.01) (OR 5.96, 95% CI 1.67-21.32), while patients with venous thrombosis had an allele distribution similar to that of those without venous thrombosis (0.49 versus 0.50; P not significant). There was a trend toward higher PAI-1 antigen and activity levels in APS patients and controls with the 4G/4G genotype, but this did not reach statistical significance. CONCLUSION: The presence of the 4G allele of the 4G/5G polymorphism of the PAI-1 gene may be an additional risk factor for the development of arterial thrombosis in APS.

Adult↗
PubMed2000

Hepatitis C virus infection mimicking systemic lupus erythematosus: study of hepatitis C virus infection in a series of 134 Spanish patients with systemic lupus erythematosus.

OBJECTIVE: To determine the prevalence and clinical significance of hepatitis C virus (HCV) infection in patients with systemic lupus erythematosus (SLE). METHODS: We investigated 134 consecutive SLE patients (121 women and 13 men; mean age 35 years) who fulfilled the 1982 revised criteria for SLE of the American College of Rheumatology. Two hundred consecutive volunteer blood donors were also studied. Serum from all patients and controls was tested for antibodies to HCV (by third generation enzyme-linked immunosorbent assay and confirmed by third generation recombinant immunoblot assay [RIBA-3]). RESULTS: Antibodies to HCV were present in 18 patients with SLE (13%) and in 2 (1%) of the blood donors studied. Among the anti-HCV-positive group, HCV infection was confirmed (by RIBA-3 and polymerase chain reaction) in 15 SLE patients (11%) and in the 2 blood donors (1%) (P < 0.001). We observed a lower frequency of cutaneous SLE features (40% versus 76%; P = 0.01) and positivity for anti-double-stranded DNA (anti-dsDNA) (33% versus 81%; P < 0.001), and a higher frequency of hepatic involvement (93% versus 2%; P < 0.001), low C4 levels (73% versus 39%; P = 0.002), low CH50 levels (73% versus 44%; P = 0.03), and cryoglobulins (60% versus 22%; P = 0.02) in SLE patients with HCV infection compared with SLE patients without infection. CONCLUSION: The prevalence of HCV infection in SLE patients was higher than in blood donors from the same geographic area. SLE HCV-positive patients showed a lower frequency of cutaneous SLE features and anti-dsDNA antibodies, and a higher prevalence of liver involvement, hypocomplementemia, and cryoglobulinemia. HCV testing should be considered in the diagnosis of SLE, especially in patients who lack the typical cutaneous features of SLE or who have low titers of autoantibodies, cryoglobulinemia, or liver involvement.

Adult↗
PubMed2000

Increased serum levels of soluble L-selectin (CD62L) in patients with active systemic lupus erythematosus (SLE).

The adhesion molecule L-selectin (CD62L) mediates lymphocyte recirculation and leucocyte rolling on vascular endothelium at sites of inflammation. Serum levels of soluble L-selectin (sL-selectin) were measured in patients with SLE in order to relate these levels to clinical activity and immunological parameters. An ELISA was used to detect the soluble form of human L-selectin (CD62L) in 42 patients with SLE and in 33 healthy individuals. The mean +/- s.e.m. values of sL-selectin were 1285 +/- 121 ng/ml for patients with SLE and 986 +/- 180 ng/ml for healthy blood donors, but there was no significant difference. When patients with active SLE were analysed, higher levels of circulating sL-selectin were found when compared with patients without activity (1497 +/- 167 ng/ml versus 941 +/- 150 ng/ml; P = 0.028). We found a significant correlation between the levels of sL-selectin and of dsDNA antibodies (r = 0.36, P = 0. 044) and between levels of sL-selectin and SLE Disease Activity Index (SLEDAI) score (r = 0.42, P = 0.003). Patients with active SLE studied cross-sectionally showed significant elevations of sL-selectin (CD62L) compared with controls. Thus, the levels of this soluble adhesion molecule correlated with active disease and levels of anti-dsDNA antibodies.

Adolescent↗
PubMed2000

Cytopenia and past human parvovirus B19 infection in patients with primary Sjögren's syndrome.

OBJECTIVES: To determine the clinical significance of human parvovirus B19 infection in patients with primary Sjögren's syndrome (SS) and to investigate the immunologic and hematologic features related to B19 infection. METHODS: We included 80 consecutive patients with primary SS (74 women and 6 men), with a mean age of 62 years (range, 24 to 87 years) that were seen in our Unit. All patients fulfilled the European Community criteria for SS. As controls, we included 140 consecutive sera samples analyzed for B19 antibodies in our Microbiology Department and obtained from adult inpatients and outpatients of our Hospital. Serum from all patients and controls was tested for antibodies to B19 by enzyme-linked immunosorbent assay (ELISA). Additionally, the presence of B19 DNA in serum and in circulating leukocytes was investigated by nested polymerase chain reaction (PCR). RESULTS: Serological evidence of past B19 infection (positive IgG antibodies without IgM antibodies) was present in 28 (35%) patients with primary SS. None of these patients showed evidence for B19 viremia, and B19 virus DNA was not detected in the circulating leukocytes of IgG-B19(+) patients. Positivity for IgM antibodies to B19 was not detected in any patient. When compared with patients without evidence of past B19 infection, those with primary SS and past B19 infection showed a higher prevalence of cytopenia (57% v 15%; P < .001), and, specifically, of leukopenia (36% v 4%; P < .001). Additionally, when compared with controls positive for IgG-B19, SS patients with these antibodies had a higher prevalence of cytopenia (57% v 13%; P < .001), leukopenia (36% v 3%; P < .001) and thrombocytopenia (21% v 0%; P = .003). CONCLUSIONS: Serological evidence of past B19 infection is associated with the presence of cytopenia in our patients with primary SS. A possible relationship between B19 infection and the presence of cytopenia in primary SS may occur in some patients immunologically or genetically predisposed.

Adult↗
PubMed2000

Thyroid disease in primary Sjögren syndrome. Study in a series of 160 patients.

We studied 160 consecutive patients (147 female and 13 male) with primary Sjögren syndrome (SS) to determine the prevalence and clinical significance of thyroid disease in a large series of patients with primary SS from our unit and to compare the prevalence and significance with those in 75 individuals without SS from a primary care center. Serum levels of thyroid hormones (free thyroxine, triiodothyronine, and thyroid-stimulating hormone) and autoantibodies against thyroglobulin (TgAb) and thyroid peroxidase (TPOAb) were measured in all SS patients and in 75 control patients. Fifty-eight (36%) of the 160 patients with primary SS had evidence of thyroid disease. Autoimmune thyroid disease (ATD) was diagnosed in 32 (20%) patients and nonautoimmune thyroid disease (NATD) in 26 (16%). No significant differences were found when these prevalences were compared with those in control patients. On the other hand, comparing those patients with altered hormonal profiles, patients with NATD showed mainly hyperthyroidism (10/17, 59% versus 2/20, 10% in patients with ATD, p = 0.001). Finally, when clinical and immunologic manifestations of SS were analyzed in patients with and without thyroid disease, respectively, we found that patients with thyroid disease had a higher prevalence of female gender (98% versus 88%, p = 0.03), antiparietal cell autoantibodies (33% versus 12%, p = 0.002), TgAb (30% versus 5%, p < 0.001), and TPOAb (40% versus 5%, p < 0.001). In conclusion, thyroid disease occurred in more than one-third of patients with primary SS; the main cause was ATD, which was present in 20% of the patients studied. We note that no significant differences were observed when the prevalence of thyroid disease (either ATD or NATD) was compared with that in a control group of similar age and gender. Our results indicate that middle-aged women (with or without SS) should be screened periodically for thyroid function.

Adult↗
PubMed2000

Catastrophic antiphospholipid syndrome.

In its classic presentation, the antiphospholipid syndrome manifests a combination of venous or arterial thrombosis and fetal loss, accompanied by elevations of antibodies directed toward negatively charged phospholipids, as measured by anticardiolipin antibody assays and/or positive lupus anticoagulant tests. The manifestations often include a moderate thrombocytopenia and, less commonly, hemolysis. In contrast, a less frequently encountered subset of the antiphospholipid syndrome, termed the "catastrophic" antiphospholipid syndrome, affects mainly small vessels predominantly supplying organs. The thrombocytopenia is usually marked, and a Coombs positive microangiopathic-type anemia may accompany the condition. Features of disseminated intravascular coagulation may be evident in some patients. It is fatal in approximately 50% of cases reported. Treatment should include not only adequate anticoagulation with intravenous heparin but also full doses of intravenous corticosteroids, to offset the systemic inflammatory response syndrome that occurs as a result of the extensive tissue damage, and plasmapheresis, using fresh frozen plasma. Parenteral antibiotics should be administered early if infection is suspected.

Antiphospholipid Syndrome↗
PubMed2000

Splenectomy for refractory Evans' syndrome associated with antiphospholipid antibodies: report of two cases.

The main haematological manifestations seen in patients with antiphospholipid antibodies (aPL) are thrombocytopenia, usually mild, and haemolytic anaemia with a positive Coombs test. Owing to the shared characteristics with idiopathic thrombocytopenic purpura, similar rules are followed in the treatment of these cytopenias. Two patients with severe aPL associated cytopenias, who required splenectomy after being refractory to steroids, immunosuppressive agents, and other treatments (intravenous gammaglobulin, danazol), are described, and previously reported cases are reviewed.

Adult↗
PubMed2000

Circulating concentrations of soluble L-selectin (CD62L) in patients with primary Sjögren's syndrome.

OBJECTIVE: Serum concentrations of soluble (s) L-selectin (CD62L) were measured in patients with primary Sjögren's syndrome (SS) to relate these concentrations to clinical and immunological features of SS. METHODS: The study included 40 consecutive patients (38 women and two men) with a mean age of 61 years (range 24-78) who fulfilled four or more of the preliminary diagnostic criteria for SS proposed by the European Community Study Group in 1993, and 33 healthy blood donors from the hospital blood bank. A sandwich enzyme linked immunosorbent assay (ELISA) was used to detect the soluble form of human sL-selectin (CD62L). RESULTS: The mean (SEM) values of sL-selectin (CD62L) were 861 (66) microg/ml for patients with SS and 986 (180) microg/ml for healthy blood donors, but there was no significant difference. In patients with primary SS, serum sL-selectin (CD62L) concentrations were significantly higher in patients with Raynaud's phenomenon (1275 (112) microg/ml versus 789 (69) microg/ml, p=0.007), autoimmune thyroiditis (1162 (113) microg/ml versus 787 (69) microg/ml, p=0.02) and rheumatoid factor (993 (95) microg/ml versus 684 (70) microg/ml, p=0.01) when compared with patients without these features. CONCLUSION: The presence of Raynaud's phenomenon, autoimmune thyroiditis and rheumatoid factor is associated with higher concentrations of circulating sL-selectin (CD62L) in the sera of patients with primary SS.

Adult↗
PubMed2000

Primary Sjögren's syndrome in men: clinical and immunological characteristics.

OBJECTIVE: To determine the clinical and immunological characteristics of primary Sjögren's syndrome (SS) in men from a large series of unselected patients with this condition. METHODS: We studied 223 consecutive patients (204 women and 19 men; mean age at onset 53 y, range 15-87 y, mean disease duration 77 months) with primary SS visited in our units. All these patients fulfilled 4 or more of the diagnostic criteria for SS proposed by the European Community Study Group in 1993. RESULTS: Nineteen (9%) patients were men and they represent the male group described in this paper. Extraglandular manifestations during the course of their disease were present in 10 (53%) of our male patients with primary SS: articular involvement in 4 (21%) patients, interstitial pneumopathy in 3 (16%) and peripheral neuropathy in 2 (11%). ANA were positive in 13 (68%) patients, RF in 5 (31%), anti-Ro/SS-A in 3 (16%) and cryoglobulins in 1/14 (7%). When compared with women, men with primary SS presented a lower prevalence of articular involvement (21 percent; vs 46%, P=0.03, OR 0.32, CI 0.07-0.97). CONCLUSION: Although primary SS is typically a disease of middle-aged women, clinicians should note that it may be diagnosed in male patients. Except for a lower prevalence of articular involvement, we could no find any notable differences in clinical and immunological characteristics between male and female patients with primary SS.

Adolescent↗
PubMed2000

Mixed cryoglobulinemia: new concepts.

The most documented extrahepatic manifestation of hepatitis C virus (HCV) infection is mixed cryoglobulinemia (MC). MC is characterised by the presence of temperature-sensitive protein complexes: in type II MC, cryoglobulins are composed of a monoclonal rheumatoid factor (usually, IgMkappa) against polyclonal IgG. In type III MC, all components are polyclonal. The presence of microheterogeneity and other new types of cryoglobulins is a novel and recent observation. The production of different autoantibodies and circulating immune complexes, including the cryoglobulins, are responsible for systemic vasculitis and various organ damage. In a limited number of MC patients, a malignancy, that is B-cell non-Hodgkin's lymphoma or hepatocellular carcinoma, may also develop. Finally, results of interferon and/or ribavirin treatments in MC patients represent an indirect proof for the pathogenetic link between MC and HVC infection. The discovery of the relation between HCV infection and MC shows the striking association between a viral infection and an autoimmune disease and, thus, a potential link between the systemic autoimmune and lymphoproliferative disorders.

Antiviral Agents↗
PubMed2000

Psychiatric and psychosocial disorders in patients with systemic lupus erythematosus: a longitudinal study of active and inactive stages of the disease.

The objective was to analyze psychiatric disorders and psychosocial dysfunction in patients with systemic lupus erythematosus (SLE), studied longitudinally during active and subsequent inactive stage of their disease. During a 6 month period of study, we selected 20 consecutive patients with SLE who presented with a SLE flare. All patients fulfilled the 1982 revised criteria of the American College of Rheumatology for the classification of SLE. When patients entered the study, we performed psychiatric (CIS, RDC, STAI, HD, BDI, GHQ and MMS) psychosocial (GAS and VAS-P) scores assessment. One year later, we repeated the psychiatric and psychosocial assessment when patients showed inactive disease. The 20 patients evaluated were women, with a mean age of 34 y (SE 14.4, range 20-57). According to CIS evaluation, we diagnosed 8 (40%) psychiatric cases in the acute episode of SLE. The RDC diagnosis showed generalized anxiety in 5 patients, panic disorders in 2 patients and generalized anxiety plus depressive symptoms in one patient. One year later, when patients did not show disease activity, we diagnosed 2 (10%) psychiatric cases (P<0.05). When SLE patients were clinically inactive, they showed lower levels of psychological distress (GHQ scale, 1.8 vs 5.6, P<0.001), with a lower grade of anxiety measured by both HA (3.2 vs 8.2, P<0.01) and STAI-S (7.95 vs 20.90, P<0.001) scales. We also found a lower score in pain perception (VAS-P) (2.80 vs 4.25, P<0. 01) and higher occupational activity (VAS-P) (83.9 vs 66.2, P<0.01) and general functioning (GAS) (93.75 vs 83.50, P<0.05) during the inactive stage. No significant differences were found when we compared cognitive impairment, grade of depression and physical disability between inactive and active stages. We conclude that in SLE patients, psychiatric and psychosocial disorders during acute episodes are usually mild and seem to be related to the psychological impact of disease activity on patients. This type of psychiatric pathology is similar to that which would be expected in other groups coping with a stressful event, indicating that our patients did not react in a way specifically determined by their systemic disease.

Adult↗
PubMed2000

Catastrophic antiphospholipid syndrome: clinical description and triggering factors in 8 patients.

In 1992, an attempt to single out a different and important group of patients was adopted by introducing the term "catastrophic" antiphospholipid syndrome (APS) to describe their potentially life threatening clinical course, characterized by acute multiorgan failure (3 or more organ systems damaged). Patients previously described in the literature showed certain differences that appear to distinguish this minority group of patients with catastrophic APS from the majority of APS patients. We describe 8 patients with catastrophic APS, focusing especially on the possible extrinsic factors (mainly infections) that might trigger this "catastrophic" situation.

Adolescent↗
PubMed2000

Prevalence and clinical significance of antiprothrombin antibodies in patients with systemic lupus erythematosus or with primary antiphospholipid syndrome.

BACKGROUND AND OBJECTIVE: Antibodies to prothrombin (aII) have been identified in patients with antiphospholipid antibodies, but their clinical significance is not well known. The aim of our study was to investigate their prevalence and association with clinical manifestations of the antiphospholipid syndrome (APS) in patients with primary APS or with systemic lupus erythematosus (SLE). DESIGN AND METHODS: A series of 177 patients with autoimmune diseases was studied: 70 with primary APS and 107 with systemic lupus erythematosus. A control group of 87 healthy volunteers were included in the study. aII were investigated in sera by an ELISA, using human prothrombin as antigen fixed in irradiated polystyrene plates. RESULTS: aII prevalence in patients with autoimmune disease was 47% (57% and 40% in patients with primary APS or with SLE, respectively) significantly higher than in controls (5%) (p<0.0001). In the whole series, thrombotic events were more prevalent in patients with aII (45% vs 28%; p=0.02). Moreover, aII was found to be an independent risk factor for arterial thrombosis (OR=2.4; p=0. 04). Similarly, in patients with SLE, aII were associated with both arterial and venous thrombosis (35% vs 14%; p=0.01), although only IgG-aII (OR=3.7; p=0.01) had an independent value as risk factor for thrombosis. However, a relationship between aII and thrombosis was not found in primary APS. aII were associated with thrombocytopenia only in patients with primary APS (OR=6.7; p=0.007). INTERPRETATION AND CONCLUSIONS: aII seem to be a serological marker of thrombosis in autoimmune diseases, mainly in SLE patients and/or in the arterial territory.

Abortion, Spontaneous↗