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Biomedical subjects

R Cervera

Publications and source records attributed to R Cervera.

At least 109 records · Page 6Linked to original sources
PubMed1998

Systemic lupus erythematosus (SLE) in childhood: analysis of clinical and immunological findings in 34 patients and comparison with SLE characteristics in adults.

OBJECTIVE: To define the pattern of disease expression in patients with childhood onset systemic lupus erythematosus (SLE). METHODS: Prospective analysis of clinical manifestations and immunological features of 34 patients in whom the first manifestations appeared in childhood from a series of 430 unselected patients with SLE. RESULTS: Thirty one (91%) patients from the childhood onset group were female and three male (9%) (ratio female/male, 10/1, with no difference compared with the adult onset group). Mean age of this group at disease onset was 11 years (range 5-14) compared with 32 years (15-48) for the remaining patients. The childhood onset patients more often had nephropathy (20% v 9% in adult onset SLE, p = 0.04; OR:2.7; 95% CI:1.1, 7), fever (41% v 21%, p = 0.006; OR:2.6, 95% CI:1.2, 5.7), and lymphadenopathy (6% v 0.5%, p = 0.03, OR: 12.3, 95% CI: 1.2, 127.6), as presenting clinical manifestations. During the evolution of the disease, the childhood onset patients had an increased prevalence of malar rash (79% v 51%, p = 0.002; OR:3.7; 95% CI:1.5, 9.5) and chorea (9% v 0%, p < 0.0001). This group exhibited a higher prevalence of anticardiolipin antibodies (aCL) of the IgG isotype when compared with the remaining patients (29% v 13%, p = 0.017; OR:2.9, 95% CI:1.2, 6.8). No significant differences were found among the other antibodies between the two groups. Childhood onset patients more often received azathioprine (15% v 6%, p = 0.00004; OR:11.2; 95% CI:2.8, 44.9) but no differences were detected between the groups concerning side effects or drug toxicity. CONCLUSIONS: The presentation and the clinical course of SLE varied in this series of 430 patients depending on their age at disease onset. Nephropathy, fever, and lymphadenopathy were more common in childhood onset patients as presenting clinical manifestations, while malar rash, chorea, and detection of IgG aCL were more common during the evolution of the disease.

Adolescent↗
PubMed1998

Young onset of primary Sjögren's syndrome: clinical and immunological characteristics.

The objective of our study was to determine the clinical and immunological characteristics of primary Sjögren's syndrome (SS) in patients with a young onset of the disease. We included 144 consecutive patients (134 female and 10 male; mean age at onset 53 y; range 20-87 y) visited in our Units. All patients were white and fulfilled four or more of the diagnostic criteria for SS, proposed by the European Community Study Group in 1993. Disease onset was determined on the basis of the appearance of symptoms strongly suggestive of SS. In 13 (9%) patients, disease onset occurred before the age of 35. All were female and the disease onset occurred between 20-34 y (mean, 28 y). When compared with patients with older onset, patients with a young onset of the primary SS presented a higher prevalence of lymphadenopathy (54% vs 6%, P < 0.001), rheumatoid factor (70% vs 39%, P=0.034), anti-Ro/SS-A antibodies (70% vs 28%, P=0.004) and monoclonal immunoglobulins (23% vs 4%, P=0.02) in their sera. From the initial diagnosis of SS, three patients with a young-onset of the primary SS have developed lymphoproliferative disease at the time of the study, compared with one patient of the older-onset group (23% vs 1%, P=0.002). Our study shows several differences between younger and older onset patients, including a higher incidence of lymphomas in the younger, thus conferring to the age at onset of the disease a prognostic value.

Adult↗
PubMed1997

[Primary Sjögren's syndrome: clinical and immunologic study of 80 patients].

BACKGROUND: To determine the clinical and immunologic characteristics of a large cohort of patients with primary Sjörgen's syndrome (SS) and to asses if the sex, the age at onset, the time of evolution and the immunologic pattern define different subsets with specific characteristics. PATIENTS AND METHODS: We included 80 patients (76 female and 4 male) that were prospectively studied at our Unit. All patients fulfilled the European Community criteria proposed in 1993 for the diagnosis of SS. RESULTS: Mean age of patients was 62 years with a mean disease duration of 8 years. The most frequently observed clinical manifestations were xerostomia (96%), xerophthalmia (94%) and parotidomegaly (46%). The main extraglandular manifestations were arthritis (45%), Raynaud's phenomenon (20%) and liver involvement (19%). The immunologic study showed antinuclear antibodies in 82% of patients, rheumatoid factor in 45%, anti-Ro/SS-A in 40% and anti-La/SS-B in 20%. In patients with an onset of disease before the age of 40 years, a higher prevalence of parotidomegaly, peripheral neuropathy, cutaneous vasculitis, rheumatoid factor, anti-Ro/SS-A and anti-La/SS-B antibodies was observed. A disease duration longer than 10 years was associated with a higher prevalence of pulmonary involvement and more focus of mononuclear cells in the minor salivary glands. Clinical manifestations associated to any one or more immunologic marker (rheumatoid factor, anti-Ro/SS-A and/or anti-La/SS-B) were Raynaud's phenomenon, arthritis, thyroid disease, cutaneous vasculitis and peripheral neuropathy. CONCLUSIONS: Primary SS is an autoimmune disease characterized by a marked heterogeneity in the clinical presentation and evolution, thus allowing the definition of several subsets of patients with their own clinical and immunological characteristics.

Adult↗
PubMed1997

Antibodies to thromboplastin in systemic lupus erythematosus: isotype distribution and clinical significance in a series of 92 patients.

We determined the prevalence and relationship with clinical manifestations of antibodies to thromboplastin (aTP) in 92 patients with systemic lupus erythematosus (SLE). Thirty-two (35%) patients had aTP: 13 (14%) were positive for IgG aTP, 13 (14%) for IgM aTP, and 6 (7%) for both. Patients with aTP had an increased incidence of thrombosis (p = 0.01), thrombocytopenia (p < 0.001), hemolytic anemia (p < 0.001), and fetal losses (p = 0.03). When the IgG and IgM aTP isotypes were analysed separately, the IgG aTP were found to be associated with thrombosis (p < 0.001), thrombocytopenia (p < 0.001), and fetal losses (p = 0.02). The IgM aTP were associated with hemolytic anemia (p < 0.001). A correlation was found between the titers of aTP and those of anticardiolipin antibodies, in both IgG (p < 0.01, r = 0.6) and IgM (p < 0.01, r = 0.64) isotypes, and between the titers of IgG aTP and the diluted Russell's viper venom time used to detect the lupus anticoagulant (p < 0.001, r = 0.42). This test is a reliable, reproducible and sensitive assay for the detection of antiphospholipid antibodies, specially in those patients under anticoagulant therapy.

Adolescent↗
PubMed1997

Optic neuropathy in the "primary" antiphospholipid syndrome: report of a case and review of the literature.

Several visual disturbances have been associated with the presence of antiphospholipid antibodies, including vaso-occlusive retinopathy and ischaemic optic neuropathy. The latter has been rarely described in patients with "primary" antiphospholipid syndrome, and, when present, is generally associated with neurological disorders. We describe the case of a patient presenting with optic neuropathy in whom a "primary" antiphospholipid syndrome was diagnosed, and review all previously reported similar cases. The pathogenesis and treatment are also discussed.

Anticoagulants↗
PubMed1997

Anti-beta 2-glycoprotein I antibodies: a useful marker for the antiphospholipid syndrome.

We studied anti-beta 2-glycoprotein I antibodies (a beta 2GPI) in autoimmune disease patients to evaluate their relationship to clinical findings. Seventy-nine systemic lupus erythematosus (SLE) patients [44 with antiphospholipid antibodies (aPL)], 21 with primary antiphospholipid syndrome (APS), eight asymptomatic individuals with aPL and 60 controls were studied. Sixteen SLE patients (14 with aPL and two without aPL) and six with primary APS had a beta 2GPI. A significant relationship was found between a beta 2GPI and aPL (P < 0.01). In SLE, a significant correlation was found between previous thrombosis or thrombocytopenia and a beta 2GPI or a beta 2GPI + aPL, but not between fetal losses and a beta 2GPI. These data suggest that a beta 2GPI may be useful in the study of APS.

Adult↗
PubMed1997

Chorea in the antiphospholipid syndrome. Clinical, radiologic, and immunologic characteristics of 50 patients from our clinics and the recent literature.

We analyzed the clinical, radiologic, and immunologic characteristics of 50 patients with chorea and the antiphospholipid syndrome (APS) (6 from our clinics and 44 from a MEDLINE computer-assisted review of the literature from 1985 through 1995). Forty-eight (96%) patients were female and 2 (4%) were male. Twenty-nine (58%) patients had defined systemic lupus erythematosus (SLE), 6 (12%) had "lupus-like" syndrome, and 15 (30%) patients had "primary" APS. Mean age of patients in this series was 23 +/- 12 years (range, 6-77 yr); mean age at presentation of chorea was 21 +/- 12 years (range, 6-77 yr). In 11 (22%) patients, the onset of chorea was in childhood (6-14 yr), and in 2 (4%) patients it presented at 60 years or more. Six (12%) patients developed chorea soon after they started taking estrogen-containing oral contraceptives, 3 (6%) developed chorea gravidarum, and 1 (2%) patient developed chorea shortly after delivery. Most patients (66%) presented only 1 episode of chorea. Chorea was bilateral in 55% of patients. Computed tomography and magnetic resonance imaging scans reported cerebral infarcts in 35% of patients. The following antibodies were detected: lupus anticoagulant (92%), anticardiolipin antibodies (91%), antinuclear antibodies (82%), anti-DNA (59%), anti-Ro (10%), anti-RNP (8%), anti-La (2%), and anti-Sm (2%). The chorea in these patients responded to a variety of medications, for example, steroids, haloperidol, antiaggregants, anticoagulants, or a combination of therapy, usually prescribed in the presence of other manifestations of APS or SLE. However, many patients responded well to haloperidol and to the discontinuation of oral contraceptives if this was the precipitating factor.

Adolescent↗
PubMed1997

Hepatitis C virus infection in 'primary' Sjögren's syndrome: prevalence and clinical significance in a series of 90 patients.

OBJECTIVES: To determine the prevalence and clinical significance of hepatitis C virus (HCV) infection in a large cohort of patients with "primary' Sjögren's syndrome (SS). METHODS: 90 consecutive patients (83 female and seven male) were included, with a mean age of 62 years (range 31-80) who prospectively visited our unit. All patients fulfilled the European Community criteria for SS and underwent a complete history, physical examination, as well as biochemical and immunological evaluation for liver disease. Serum from all patients was tested for antibodies to HCV by third generation enzyme linked immunoassay and positivity was confirmed by polymerase chain reaction. RESULTS: Antibodies to HCV were present in 13 (14%) patients with 'primary' SS. When compared with patients without HCV infection, patients with HCV infection presented a higher prevalence of hepatic involvement, (100% v 8%, p < 0.05). Transcutaneous liver biopsy was performed in five patients with HCV infection, and specimens obtained showed in all cases a chronic active hepatitis with varying degrees of portal inflammation. CONCLUSION: HCV infection is frequent in patients with "primary' SS and liver involvement is presented in all these patients. The possible pathogenic role of HCV infection in these patients is still unclear.

Adult↗
PubMed1997

Shrinking lungs syndrome in systemic lupus erythematosus: improvement with inhaled beta-agonist therapy.

The pathogenesis and therapy of shrinking lungs syndrome in patients with systemic lupus erythematosus remains controversial. It has been previously reported that corticosteroid treatment can be effective. We report a patient with shrinking lungs syndrome who presented a good response to inhaled beta-agonist therapy. Therapeutic approach to this syndrome is discussed.

Administration, Inhalation↗
PubMed1997

Anti-endothelial cell antibodies in systemic autoimmune diseases: prevalence and clinical significance.

OBJECTIVE: To investigate the prevalence and characteristics of anti-endothelial cell antibodies (AECA) in a large cohort of patients with several well defined systemic autoimmune diseases, in order to determine their relationship with the clinical and laboratory features of these diseases. METHODS: Clinical and laboratory features of 216 consecutive Caucasian patients were prospectively studied. One hundred and seven patients had been diagnosed as having a primary systemic vasculitis-specifically, 39 had temporal arteritis (TA), 25 polyarteritis nodosa (PAN), 9 Wegener's granulomatosis (WG), and 34 Behcet's disease (BD)-, 90 patients had systemic lupus erythematosus (SLE), and 19 had a primary Sjogren's syndrome (SS). The AECA were determined by ELISA. RESULTS: One hundred and four (48%) patients with systemic autoimmune diseases were found to have a positive titre of AECA. Specifically, AECA were detected in 41 (38%) patients with a primary systemic vasculitis (13 (33%) with TA, 14 (56%) with PAN, 5 (56%) with WG and 9 (26%) with BD), in 58 (63%) patients with SLE, and in 5 (26%) patients with a primary SS. In patients with a primary systemic vasculitis, those with AECA were found to have an increased prevalence of disease activity (P < 0.05). In SLE patients, those with AECA were found to have an increased prevalence of vascular lesions (P < 0.05), lupus nephropathy (P < 0.05), and anticardiolipin antibodies (aCL) (P < 0.001). CONCLUSIONS: Patients with systemic autoimmune diseases have a high prevalence of AECA and they are associated with the presence of vascular lesions, nephropathy, and aCL in SLE, as well as with disease activity in several primary systemic vasculitis (TA, PAN, WG and BD).

Adult↗
PubMed1996

Antibodies to endothelial cells and to beta 2-glycoprotein I in the antiphospholipid syndrome: prevalence and isotype distribution.

The aim of this study was to analyse the prevalence and isotype distribution of antibodies to endothelial cells (aEC) and to beta 2-glycoprotein I (a beta 2GPI) in the antiphospholipid syndrome (APS). Fifteen patients with an APS [nine associated with systemic lupus erythematosus (SLE) and six "primary'] and 15 with SLE without an APS were prospectively studied. The aEC were determined by an enzyme-linked immunosorbent assay (ELISA) using endothelial cells derived from human umbilical vein and the a beta 2GPI by ELISA using highly purified beta 2GPI. A positive titre of aEC was detected in 20 out of 30 patients (67%), but in none of the control group. Ten patients had both IgG and IgM isotypes, five had IgG only and five had only IgM. Thirteen patients with the APS (87%) were found to have a positive titre of aEC, while only seven with SLE but without a history of APS (47%) had aEC (P < 0.05). Nine patients with the APS (60%) had a positive titre of a beta 2GPI (four had both IgG and IgM isotypes, one had IgG only and four had only IgM), while none of the patients without an APS (0%) had these antibodies (P < 0.001). A significant association was also found between the presence of aPL and aEC (P < 0.05), as well as between aPL and a beta 2GPI (P < 0.001). Both aEC and a beta 2GPI can be found in the APS. This reinforces the theory that APS represents a complex autoimmune disorder in which several autoantibodies co-exist with aPL.

Adult↗
PubMed1996

Von Willebrand factor in the outcome of temporal arteritis.

OBJECTIVE: To determine fluctuation in circulating von Willebrand factor (vWF) in the outcome of patients with temporal arteritis. METHODS: Plasma vWF antigen concentrations were measured in 65 patients with biopsy proven temporal arteritis at different disease activity stages, in 12 with isolated polymyalgia rheumatica, and in 16 controls. Fourteen temporal arteritis patients underwent serial determinations during the course of their disease. RESULTS: vWF concentrations were significantly raised in temporal arteritis (mean 220 [arbitrary units], range 96 to 720) and in polymyalgia rheumatica (mean 196, range 103 to 266) compared with healthy controls (mean 98, range 75 to 137) (P < 0.05). Although vWF values tended to be higher in temporal arteritis, no significant differences were found between temporal arteritis and polymyalgia rheumatica patients nor between temporal arteritis patients with ischaemic complications (mean 269, range 130 to 720) and those who presented with polymyalgia rheumatica or constitutional symptoms only (mean 179, range 140 to 220). The highest levels were obtained in patients with associated, mainly infectious, diseases (mean 631, range 240 to 1680). Raised vWF values found in active temporal arteritis patients (mean 220, range 96 to 720) persisted within the first two years after the beginning of treatment (mean 244, range 102 to 510) but tended to normalise in patients in long term remission (mean 143, range 50 to 260). CONCLUSIONS: Persistent elevation of vWF during early remission of temporal arteritis might represent an endothelial activation status induced by a remaining inflammatory microenvironment rather than a marker of endothelial cell injury. In long term remission, decreasing vWF concentrations might reflect progression of inflammatory lesions to a healing stage.

Acute Disease↗