RAYNAUDS-PHENOMENON IN SYSTEMIC LUPUS-ERYTHEMATOSUS

Objective: to determine the frequency and nature of clinical manifestations in Brazilian patients with systemic lupus erythematosus and Raynaud's phenomenon. Methods: One hundred twenty-three patients with systemic lupus erythematosus (1982 American Rheumatism Association criteria) and Raynaud's phenomenon (at least two-phase, bilateral color reaction reported by the patient or observed by a physician) referred to the Rheumatology Unit of the Campinas University Hospital were retrospectively compared with 149 systemic lupus erythematosus patients without Raynaud's phenomenon. For each patient, gender, race, age at disease onset, disease duration, follow-up duration, clinical manifestations, and laboratory test findings were recorded. Results: Patients with Raynaud's phenomenon were more likely to have skin lesions and/or myalgia/myopathy and less likely to have nephritis and nephrotic syndrome. All five patients with pulmonary hypertension also had Raynaud's phenomenon. Raynaud's phenomenon was less common in younger patients and more common in patients with alopecia. Conclusions: Raynaud's phenomenon was common in systemic lupus erythematosus patients from a tropical country. A better prognosis can be expected in lupus patients with Raynaud's phenomenon except when pulmonary hypertension occurs.62536737

Evaluation of the hypothalamic-pituitary-gonadal axis in males with systemic lupus erythematosus

Objective. To evaluate the hypothalamic-pituitary-gonadal axis in male patients with systemic lupus erythematosus (SLE). Methods. We studied 7 male patients with SLE and compared them with 10 age matched healthy controls. Clinical data, laboratory tests, drugs used, and disease activity for SLE (SLE Disease Activity Index) were determined. The basal serum levels of cortisol, total testosterone (T), free testosterone Fl, androstenedione, dehydroepiandrosterone sulfate (DHEAS), estradiol (E2), prolactin (PRL), luteinizing hormone (LH), and follicle stimulating hormone (FSH) were measured in all individuals. In addition, response of LH and FSH to stimulation with luteinizing hormone releasing hormone (LHRH, 100 mu g, intravenously) and response of T and FT to stimulation with human chorionic gonadotropins (HCG, 1500 u intramuscular for 3 days) were examined. Results. Patients with SLE had lower basal levels of T and FT than controls but this difference was not significant. DHEAS and A levels were significantly lower in patients than in controls. The low response of ET after stimulation with HCG indicated diminished testis function (mainly Leidyg cells). In contrast to other studies, the E2 level was significantly lower in patients than in controls. The groups did not differ in LH levels at baseline or after stimulation with LHRH. However, basal levels of FSH were significantly higher in patients. Conclusion. These results suggest that the hypothalamic-pituitary-axis function was normal in patients with SLE. The testis had diminished function, shown by reduced response of FT to stimulation with HCG, but possible inhibitory effects of glucocorticoid therapy must be considered.2561097110

Bone mineral density in systemic lupus erythematosus and its relation to age at disease onset, plasmatic estradiol and immunosuppressive therapy

Objective. - The aim of this paper was to evaluate bone mineral density (BMD) in patients with systemic lupus erythematosus (SLE), to determine the role of corticosteroids and cytotoxic drugs and to assess estrogen effect on BMD in SLE. Patients and methods. - BMD (DEXA) at lumbar vertebrae (L2-L4) and at femoral neck was performed in 60 pre-menopausal SLE patients and in 64 controls. Estradiol level was measured in all the individuals. Age, age at disease onset, body mass index (BMI), time of disease, disease activity (SLEDAI), prednisone dose at the evaluation, total cumulative and cumulative prednisone dose in the last year and cytotoxic drugs were assessed. Results. - The mean plasmatic estradiol was 175.9 pg/ml in patients and 149.9 in controls. BMD was inferior in patients than that in controls (P < 0.0001). The mean current, cumulative and previous year prednisone doses were, respectively, 19.17 mg/d, 28.78 g and 5.33 g. There was no association between corticosteroids or cytotoxic drug used and low bone mass. The serum concentration of estradiol did not influence the bone mass. The BMI and age at disease onset exhibited an influence on BMD at L2. Conclusions. - BMD was significantly lower in SLE patients but not related to CS (Corticosteroids)or other drugs; the estradiol in these patients had no effect on BMD. Low BMI interacting with early onset of disease might influence the probability of loss of bone mass. (C) 2002 Editions scientifiques,et medicales Elsevier SAS. All rights reserved.701404

Young age at onset, renal involvement, and arterial hypertension are of adverse prognostic significance in juvenile systemic lupus erythematosus

Objective. To look for associations between mortality, clinical or laboratory data, and age at disease onset in systemic lupus erythematosus patients aged 16 years or younger at disease onset. Patients and methods. The medical records of patients seen at the Clinics Hospital, State University of Campinas, Sao Paulo, Brazil. between 1979 and 1995 were reviewed retrospectively. All 59 included patients (48F/11M) fulfilled four or more American College of Rheumatology criteria for systemic lupus erythematosus, Patients with discoid, drug-induced or neonatal lupus, or other systemic connective tissue diseases were excluded. Patients were studied individually then classified in three groups based on age at disease onset, as follows: Group I, less than or equal to 9 years of age; Group II, 10-14 years of age; and Group III, 15-16 years of age. Clinical and laboratory abnormalities and mortality were compared in the three groups. Results. The most frequent clinical manifestations were joint symptoms (91.5%), renal involvement (71.1%), malar rash (61%), alopecia (61%), fever (59.3%) and photosensitivity (52.5%). Laboratory findings included antinuclear antibody in 94.9% of cases, LE cells in 71.1%, low serum complement in 65.3%, anti-DNA in 63.4%, hematuria in 62.7%, and proteinuria in 61%, The mortality rate was 23.7% (9F/5M) overall, 18.7% in females, and 45.4% in males (P=0.07). The cause of death was infection in eight patients (57.1% of decedents), central nervous system disease in five (35.7%), and renal insufficiency in one (7.2%), Disease onset before 15 years of age (P=0.026), renal involvement (P=0.03), and arterial hypertension (P=0.002) were predictive of mortality. Mortality was not influenced by gender or race.66630330

Evans syndrome and Systemic Lupus Erythematosus: Clinical presentation and outcome

Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)Objective: To review the clinical, laboratory and outcome features of Evans syndrome (ES) in systemic lupus erythematosus (SLE) patients. Methods: We reviewed the charts of 953 SLE patients followed up regularly at our service. ES was defined as the presence of hemolytic anemia and thrombocytopenia concomitantly or sequentially. Clinical and laboratory manifestations occurring during the disease course, as well as concomitant diseases and survival was carefully reviewed. Results: We identified ES in 26 of 953 (2.7%) SLE patients. Twenty-three were women with mean age at SLE diagnosis of 25.7 years. Four (15%) patients had disease onset before the age of 16. In the majority of patients (92%), immune thrombocytopenia and AIHA appeared simultaneously at the beginning of SLE. Active features of SLE were a frequent finding concomitant to ES, especially arthritis (77%), malar rash (61.5%), photosensitivity (57.6%), oral ulcers (34.6%), nephritis (73%), serositis (54%), neuropsychiatric (19%) and pulmonary (15%) manifestations. In addition to this multisystemic disease, 34.6% of our patients had an association with another autoimmune disease such as antiphospholipid syndrome. Recurrence of ES was observed in only four (15%) patients. After follow-up time of 8.72 years, 19 patients (73%) were in remission and seven (27%) patients died. Discussion: ES is a rare manifestation in SLE, occurring in patients with severe multisystemic SLE manifestations. Treatment strategies frequently used in SLE contribute to longer disease remission and less frequent exacerbation than observed in the general population with ES. (C) 2011 Societe francaise de rhumatologie. Published by Elsevier Masson SAS. All rights reserved.794362364Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)FAPESP [FAPESP 08/020917-0, 09/11076-2]CNPq [301112-2007-, 0300447/2009-4

Clinical implications of migraine in systemic lupus erythematosus: relation to cumulative organ damage

The aim of this study was to determine the clinical implications of migraine in systemic lupus erythernatosus (SLE) using the cumulative organ damage scores (SLICC-DI). Eighty SLE, 40 rheumatoid arthritis (RA) patients and 40 controls (non SLE, nor RA out-patients), all women, were included. Migraine was defined according to the International Headache Society (IHS) criteria for neuropsychiatric SLE. Disease activity was measured by MEX-SLEDAI and cumulative organ damage by SLICC-DI. Statistics were obtained by Chi-square and Fischer's exact tests. ANOVA was used for comparing means. Migraine was identified in 42.5% of SLE patients, compared to 12.5% of RA patients (P < 0.05) and 10.0% (P < 0.05) in the control group. In the SLE group, a significant association between migraine and Raynaud's phenomenon (P = 0.003, OR = 10.1; 95%Cl 2.9-35) and antiphospholipid antibodies (P = 0.0012; OR = 7.5; 95%Cl 2.5-22.9) was noted. SLE patients with active migraine had higher MEX-SLEDAI scores than SLE patients without migraine. SLE patients with past history of migraine had significantly higher SLICC scores than SLE patients without migraine. History of migraine was associated with greater organ damage. Active migraine was associated with higher disease activity, antiphospholipid antibodies and worsening of Raynaud's phenomenon. The increased cumulative organ damage in SLE patients with past history of migraine justifies the routine evaluation of migraine in clinical practice.24121024103

SYSTEMIC LUPUS-ERYTHEMATOSUS IN 18 BRAZILIAN MALES - CLINICAL AND LABORATORY ANALYSIS

The clinical and laboratory features of 18 males were compared with 254 female patients with systemic lupus erythematosus (SLE). At disease onset male patients were younger than female. Nephropathy, nephrotic syndrome and thrombocytopenia were significantly more frequent in male patients (p<0.05). Pleuritis occurred as the initial symptom at a significantly higher frequency in males (p<0.05). No significant immunological difference was found between two groups, except for anti-Sm antibodies which were more frequent in males than in females, but were measured in few patients. We concluded that male patients with SLE have a more severe disease with higher morbidity, specially related to renal disease.12452252

SYSTEMIC LUPUS-ERYTHEMATOSUS - CLINICAL AND LABORATORY ASPECTS RELATED TO AGE AT DISEASE ONSET

Objective:To analyse the clinical and laboratory parameters in patients with SLE according to their age at disease onset a retrospective study was undertaken of 272 Brazilian patients fulfilling the 1982 ARA criteria for SLE who were referred to the University Hospital of Campinas between 1973 - 1992. Methods: The patients were divided into three groups according to their age at disease onset: Group A. under the age of 16 (39 patients); Group B age 17 to 49 (223 patients), Group C over the age of 50 (10 patients). Various clinical and laboratorial parameters were analysed and compared among these groups. Results: There were no significant differences in terms of race, time of disease onset of time of follow-up. Group A had more male patients than Groups B (p < 0.05) of C. Alopecia as an early manifestation, seizures and gastrointestinal involvement were more frequent in Group A (p < 0.05). Raynaud's phenomenon was lower in Group A than in Groups B and C (p < 0.05). Pericarditis was higher in Group C than in Groups A or B (p < 0.05). Nephrotic syndrome was lower in Group C than in Group A (p < 0.05). Positive LE cells were higher in Groups A and C than in Group B (p < 0.05). Anti-DNA antibodies were more prevalent in Group A than in B (p < 0.05). Anti-cardiolipin antibodies were more frequent in adult patients (p < 0.05) (Group B). The mortality rate was higher in Group A than in B or C (p < 0.05). Conclusion: The clinical presentation and course of SLE may be influenced by the age at disease onset. Younger patients showed a poorer prognosis with more seizures, gastrointestinal involvement, nephrotic syndrome, and a higher rate of mortality than the other groups. Group A included more male patients and also exhibited more positive LE cells and anti-DNA antibodies. Raynaud's phenomenon was lower in these young patients. Elderly patients (C) presented more pericarditis and showed mild disease.12660360

Ovarian failure in SLE patients using pulse cyclophosphamide: comparison of different regimes

The objective of this study was to determine the frequency and risk factors of early ovarian failure in systemic lupus erythematosus (SLE) women treated with cyclophosphamide (CY). We further tried to determine if there was a reduction of ovarian failure in recent years, due to reduction in the CY dose. We reviewed the charts of all women below 40 years of age who received intravenous CY pulse therapy. In order to be included, the patients must have finished CY treatment before completing 40 years. Patients were divided into two groups: Group A (57 patients), patients who were treated with 0.75 mg/body surface; Group B (50 patients), patients treated with 0.5 mg/body surface. Fifty patients with similar age distribution who never received CY were selected from the database as a control group (Group C). The Chi-square test was applied to compare the categorical variables of the groups and whenever needed, the Fisher's Exact test was used. We observed similar age distribution and disease duration at disease onset between groups. Also, no differences regarding the age at menarche, total prednisone dose, and SLICC-ACR/DI scores were observed at disease onset between the three groups. In group A, ten (17.5%) patients refereed sustained amenorrhea, independently associated with treatment duration (P = 0.001), total intravenous cyclophosphamide (IV-CF) dose (P = 0.02), older age at disease onset (P = 0.04). Seven (12.3%) patients referred transient amenorrhea. Transient amenorrhea was related to CY treatment duration (P = 0.017). In group B, no patient reported sustained amenorrhea and 10 of 50 (20%) patients referred transient amenorrhea, related to CY treatment duration (P = 0.017). The most important risk factors for menstrual abnormalities were duration of treatment and cumulative dose of CY. Lower CY dose reduced the number of premature ovarian failures significantly in this cohort.28656757

Neuropsychiatric Manifestations in Systemic Lupus Erythematosus Epidemiology, Pathophysiology and Management

Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)Systemic lupus erythematosus (SLE) is a relapsing-remitting autoimmune disease with CNS involvement occurring in up to 75% of patients. However, the frequency of neuropsychiatric manifestations in SLE studies varies widely, depending on the type of manifestations included and the method used for evaluation. CNS involvement may be considered primary if directly related to SLE activity in the CNS or secondary when related to treatment, infections, metabolic abnormalities or other systemic manifestations such as uraemia and hypertension. The pathogenesis of neuropsychiatric SLE is as yet unknown, though numerous autoantibodies and cytokines have been suggested as possible mediators. However, independent of the aetiology of the insult, the final common pathway in neuropsychiatric SLE is the involvement of the cerebral microvasculature. The diagnosis of primary CNS involvement by SLE is often difficult, as both focal and diffuse manifestations may occur and there is no gold standard for diagnosis. A high index of clinical suspicion, in addition to laboratory and neuroimaging findings may support the diagnosis. Treatment is mostly empirical, although one randomized controlled trial has shown that cyclophosphamide in addition to methylprednisolone is superior to methylprednisolone alone in severe neuropsychiatric SLE.o TEXTO COMPLETO DESTE ARTIGO, ESTARÁ DISPONÍVEL À PARTIR DE AGOSTO DE 2015.259721736Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)FAPESP [2008/020917-0, 2009/06049-6, 09/11076-2]CNPq [300447/2009-4