First month prednisone dose predicts prednisone burden during the following 11†months: an observational study from the RELES cohort.

AIM: To study the influence of prednisone dose during the first month after systemic lupus erythematosus (SLE) diagnosis (prednisone-1) on glucocorticoid burden during the subsequent 11 months (prednisone-2-12). METHODS: 223 patients from the Registro Español de Lupus Eritematoso Sistémico inception cohort were studied. The cumulative dose of prednisone-1 and prednisone-2-12 were calculated and recoded into a four-level categorical variable: no prednisone, low dose (up to 7.5 mg/day), medium dose (up to 30 mg/day) and high dose (over 30 mg/day). The association between the cumulative prednisone-1 and prednisone-2-12 doses was tested. We analysed whether the four-level prednisone-1 categorical variable was an independent predictor of an average dose >7.5 mg/day of prednisone-2-12. Adjusting variables included age, immunosuppressives, antimalarials, methyl-prednisolone pulses, lupus nephritis and baseline SLE Disease Activity Index (SLEDAI). RESULTS: Within the first month, 113 patients (51%) did not receive any prednisone, 24 patients (11%) received average low doses, 46 patients (21%) received medium doses and 40 patients (18%) received high doses. There was a strong association between prednisone-1 and prednisone-2-12 dose categories (p<0.001). The cumulative prednisone-1 dose was directly associated with the cumulative prednisone-2-12 dose (p<0.001). Compared with patients on no prednisone, patients taking medium (adjusted OR 5.27, 95% CI 2.18 to 12.73) or high-dose prednisone-1 (adjusted OR 10.5, 95% CI 3.8 to 29.17) were more likely to receive prednisone-2-12 doses of >7.5 mg/day, while patients receiving low-dose prednisone-1 were not (adjusted OR 1.4, 95% CI 0. 0.38 to 5.2). If the analysis was restricted to the 158 patients with a baseline SLEDAI of ≥6, the model did not change. CONCLUSION: The dose of prednisone during the first month after the diagnosis of SLE is an independent predictor of prednisone burden during the following 11 months.

The effects of corticosteroids on cognitive flexibility and decision-making in women with lupus.

The aim of this study was to investigate the possible effects of corticosteroids in women with systemic lupus erythematosus (SLE) in two processes of executive function: cognitive flexibility and decision-making. To that end, we evaluated 121 women divided into three groups: 50 healthy women, 38 women with SLE not receiving corticosteroid treatment and 33 women with SLE receiving corticosteroid treatment. Cognitive flexibility was measured with the Trail Making Tests A and B; decision-making was measured with the Iowa Gambling Task. Additionally, demographic (age and education level), clinical (SLE Disease Activity Index (SLEDAI), Systemic Lupus International Collaborating Clinics (SLICC)/American College of Rheumatology (ACR) Damage Index (SDI) and disease duration) and psychological characteristics (stress vulnerability, perceived stress and psychopathic symptomatology) were evaluated. The results showed that both SLE groups displayed poorer decision-making than the healthy women ( p = 0.006) and also that the SLE group receiving corticosteroid treatment showed lower cognitive flexibility than the other two groups ( p = 0.030). Moreover, SLE patients showed poorer scores than healthy women on the following SCL-90-R subscales: somatisation ( p = 0.005), obsessions and compulsions ( p = 0.045), depression ( p = 0.004), hostility ( p = 0.013), phobic anxiety ( p = 0.005), psychoticism ( p = 0.016) and positive symptom total ( p = 0.001). In addition, both SLE groups were more vulnerable to stress ( p = 0.000). These findings help to understand the effects of corticosteroid treatment on cognitive flexibility and decision-making, in addition to the disease-specific effects suffered by women with SLE.

Cat scratch disease in an immunosuppressed patient with systemic lupus erythematosus.

Cat scratch disease is an infectious disorder transmitted by cats that typically affects children and young adults. Immunosuppression is a well-known risk factor for the development of severe and atypical forms of the disease; hence it is under-diagnosed in patients with compromised immunity. We are reporting the first case of cat scratch disease, which presented as fever and fatigue, in a patient with systemic lupus erythematosus while receiving immunosuppressant therapy after a kidney transplant.

Registry of the Spanish Network for Systemic Sclerosis: Survival, Prognostic Factors, and Causes of Death.

Systemic sclerosis (SSc) is a rare, multisystem disease showing a large individual variability in disease progression and prognosis. In the present study, we assess survival, causes of death, and risk factors of mortality in a large series of Spanish SSc patients. Consecutive SSc patients fulfilling criteria of the classification by LeRoy were recruited in the survey. Kaplan-Meier and Cox proportional-hazards models were used to analyze survival and to identify predictors of mortality. Among 879 consecutive patients, 138 (15.7%) deaths were registered. Seventy-six out of 138 (55%) deceased patients were due to causes attributed to SSc, and pulmonary hypertension (PH) was the leading cause in 23 (16.6%) patients. Survival rates were 96%, 93%, 83%, and 73% at 5, 10, 20, and 30 years after the first symptom, respectively. Survival rates for diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc were 91%, 86%, 64%, and 39%; and 97%, 95%, 85%, and 81% at 5, 10, 20, and 30 years, respectively (log-rank: 67.63, P < 0.0001). The dcSSc subset, male sex, age at disease onset older than 65 years, digital ulcers, interstitial lung disease (ILD), PH, heart involvement, scleroderma renal crisis (SRC), presence of antitopoisomerase I and absence of anticentromere antibodies, and active capillaroscopic pattern showed reduced survival rate. In a multivariate analysis, older age at disease onset, dcSSc, ILD, PH, and SRC were independent risk factors for mortality. In the present study involving a large cohort of SSc patients, a high prevalence of disease-related causes of death was demonstrated. Older age at disease onset, dcSSc, ILD, PH, and SRC were identified as independent prognostic factors.

A man with unilateral endophthalmitis: a case of disseminated nocardiosis.

We present the case of a patient with an infection by Nocardia which manifested itself with monocular endophthalmitis. Nocardia infection is not common and ocular involvement is one of the most uncommon presentations. In these cases it is very important to make an early diagnosis and intensive treatment to prevent the visual prognosis.

Association between low 25-hydroxyvitamin D, insulin resistance and arterial stiffness in nondiabetic women with systemic lupus erythematosus.

OBJECTIVE: The objective of this paper is to examine if there is an association between low levels of 25-hydroxyvitamin D (25(OH)D) and insulin resistance (IR) in nondiabetic women with systemic lupus erythematosus (SLE) and to evaluate its impact on arterial stiffness. PATIENTS AND METHODS: In this cross-sectional study 25(OH)D, insulin, insulin resistance measured by the homeostatic model assessment (HOMA-IR), homocysteine, fibrinogen, characteristics of SLE, medications and pulse-wave velocity (PWV) were measured in 106 nondiabetic women with SLE and 101 matched controls. RESULTS: Women with SLE tended to have lower 25(OH)D levels (p = 0.078) and a higher frequency of 25(OH)D deficiency (defined as < 10 ng/ml) than controls (p = 0.058). Patients from the lowest quartile of the 25(OH)D range had higher PWV (p = 0.043), fasting glucose (p = 0.035), insulinemia (p ≤ 0.001), HOMA-IR (p = 0.006), C4 (p = 0.012), as well as more frequent IR (p = 0.002) and metabolic syndrome (p = 0.052) than those in the upper quartile, and no differences were found in age, body mass index (BMI), blood pressure, lipid levels and renal function. In women with SLE, 25(OH)D inversely correlated with insulin (p = 0.006), HOMA-IR (p = 0.008) and C4 (p = 0.048) and tended to correlate with fibrinogen (p = 0.060) after adjustment for BMI, age, SLEDAI, prednisone dose, renal function, inflammation markers and seasonal variation, but not with PWV. In controls, 25(OH)D correlated only with homocysteine after the same adjustment, and the correlation with PWV tended to be significant after adjustment for BMI and age (r = -0.190, p = 0.10). CONCLUSION: Low 25(OH)D levels were found to be associated with increased IR in nondiabetic women with SLE independently of BMI. Low 25(OH)D levels, but not IR, could be associated with increased arterial stiffness in these patients.

Impaired sexual function in women with systemic lupus erythematosus: a cross-sectional study.

OBJECTIVE: The objectives of this paper are to compare sexual function and distress in women with systemic lupus erythematosus (SLE) and in healthy controls; to determine the association between disease characteristics, quality of life, psychopathology and sexual function; and to compare sexual function and distress of women according to age (reproductive and nonreproductive-age women). METHODS: We conducted a cross-sectional study of 120 participants; 65 women had SLE (aged 18-65), and 55 were healthy, age-matched controls. The assessment included the Female Sexual Function Index (FSFI), Symptom Checklist-90-Revised (SCL-90-R), Short Form 36 health survey (SF-36), socio-demographic characteristics and the Systemic Lupus International Collaborating Clinics (SLICC) and SLE Disease Activity Index (SLEDAI) in SLE patients only. RESULTS: Of 65 eligible patients with SLE, 61 (94%) responded; of 55 control subjects, 53 (96%) responded. The FSFI total score and subscale scores for desire, arousal, lubrication, orgasm and pain were significantly lower in patients with SLE. More somatization, obsessive-compulsive symptoms, interpersonal sensitivity, depression, anxiety, hostility, phobia, paranoid ideation, psychoticism, the Positive Symptom Total (PST), Positive Score Discomfort Index (PSDI), the use of psychotropic drugs, general health, vitality, social function, emotional role and mental health were significantly associated with changes in the patient group's sexuality. Multivariate analysis indicated that depression, PSDI and vitality were the variables significantly associated with low sexual function in patients with SLE. CONCLUSIONS: Women with SLE reported significantly impaired sexual function compared with healthy controls. Impaired sexual function was associated with somatization, obsessive-compulsive behavior, interpersonal sensitivity, depression, hostility, paranoid ideation, psychoticism, PST, higher scores in the PSDI subscale, vitality, social functioning and mental health. These results indicate that, in daily practice, inquiring about sexuality and quality of life and screening for psychopathology are important for every patient with SLE, irrespective of their clinical characteristics.

Follow-up of patients with systemic lupus erythematosus: what is not found in the guidelines.

A series of measures in the management of patients with systemic lupus erythematosus (SLE) which usually are not found in the lupus guidelines are discussed. In the lupus patient who has been well-controlled in the long term, the dose of hydroxychloroquine should be progressively reduced, without decreasing more than approximately 600 mg per week. We recommend taking this drug in the morning in patients with insomnia, at night in those with dyspepsia and to separate the intake of the drug from the shower (and the water should be as cool as possible) in those patients with aquagenic pruritus. We do not use prednisone on alternate days and exceptionally divide the dose into ¾ before breakfast and » before dinner. Twenty to 30 min should be used per patient in every scheduled visit to assure a good clinical and human practice. We analyzed the follow-up of 112 consecutive patients from our systemic disease unit and found that 71.4% of them had symptoms that were unexplained by lupus and we only referred 8.9% of them to other specialists, probably because of our general training as internal medicine doctors. We suggest that knowing the views of SLE specialists might be of interest since, well-designed studies that would allow to progress in the understanding of this disease could be performed based on their experience.

Quality-of-life predictor factors in patients with SLE and their modification after cognitive behavioural therapy.

OBJECTIVE: The aim of the study was to determine the clinical and psychological factors linked to health-related quality of life (HRQOL) in patients with systemic lupus erythematosus (SLE) and test the effectiveness of cognitive behavioural therapy in changing these factors. METHODS: We evaluated 34 patients with SLE over a period of 15 months. In order to study the variables related to items of QOL and the physical (PCS) and mental (MCS) component summaries of the SF-36, several multiple linear regression models were constructed. Patients were randomized and distributed into two similar groups; one of them received cognitive behavioural therapy and the other received the usual controlled care. The psychological aspects as well as the related-disease factors were evaluated four times during the study. RESULTS: Self-perceived stress (R² corrected: 0.314, t: -2.476, p < 0.021), vulnerability to stress (R² corrected: 0.448, T: -2.166, p < 0.04) and anxiety (R² corrected: 0.689, T: -7.294, p < 0.00) were predictor variables of MCS. The group of patients who received the therapy improved their level of physical role functioning, vitality, general health perceptions and mental health, compared with the group of patients who only received conventional care. CONCLUSION: QOL usually depends on multiple factors, some of which are stress and anxiety, which can be modified by a cognitive behavioural therapy, in order to obtain a significant improvement in the HRQOL, irrespective of the activity level of the disease. Frequent evaluations of the quality of life in patients with SLE and psychological treatment should also be considered.

Correlation of asymptomatic hyperuricaemia and serum uric acid levels with arterial stiffness in women with systemic lupus erythematosus without clinically evident atherosclerotic cardiovascular disease.

The objective of this article was to evaluate whether serum uric acid (SUA) correlates with arterial stiffness and inflammation markers in a cohort of women with systemic lupus erythematosus (SLE) without overt atherosclerotic cardiovascular diseases, who attended a community hospital. One hundred and two women with SLE were assessed as part of this cross-sectional study. Carotid-femoral pulse wave velocity (PWV) was measured using an automatic device (Complior). C-reactive protein (CRP), fibrinogen and homocysteine levels as well as other metabolic results were recorded. Duration and activity of SLE, damage accrual and treatments were recorded. SLE women were categorized as having or not having hyperuricaemia (HU) according to SUA levels (greater than or up to 6.2 mg/dl, respectively). A multiple linear regression analysis was used to determine the independent link between SUA levels and other variables. Women with SLE and HU (n = 15, 15%) had a worse cardiovascular risk profile that included ageing, hypertension, obesity, higher total cholesterol levels, renal failure and presence of metabolic syndrome. Also, the duration of SLE was increased and damage accrual was greater. In the unadjusted analysis, SUA levels correlated with PWV, CRP, fibrinogen and homocysteine. However, in a multivariate linear regression analysis, SUA levels independently correlated with the duration of SLE, creatinine, total cholesterol and homocysteine levels but did not correlate with PWV. In conclusion, SUA was associated with arterial stiffness, but not independently of age and homocysteine levels. Nevertheless, SUA might be an ancillary indicator of subclinical atherosclerosis in SLE women without clinically evident atherosclerotic cardiovascular disease.

Efficacy of cognitive behavioural therapy for the treatment of chronic stress in patients with lupus erythematosus: a randomized controlled trial.

BACKGROUND: Chronic stress worsens the quality of life (QOL) of lupus patients by affecting their physical and psychological status. The effectiveness of a cognitive-behavioural intervention in a group of patients with lupus and high levels of daily stress was investigated. METHODS: Forty-five patients with lupus and high levels of daily stress were randomly assigned to a control group (CG) or a therapy group (TG); they received cognitive behavioural therapy (CBT) which consisted of ten consecutive weekly sessions. The following variables were evaluated at baseline and at 3, 9 and 15 months: (1) stress, anxiety, depression, (2) Systemic Lupus Erythematosus Disease Activity Index, somatic symptoms, number of flares, (3) anti-nDNA antibodies, complement fractions C3 and C4 and (4) QOL. A multivariate analysis of repeated measures and various analyses of variance were carried out. RESULTS: We found a significant reduction in the level of depression, anxiety and daily stress in the TG compared to the CG and a significant improvement in QOL and somatic symptoms in the TG throughout the entire follow-up period. We did not find any significant changes in the immunological parameters. CONCLUSIONS: CBT is effective in dealing with patients suffering from lupus and high levels of daily stress as it significantly reduces the incidence of psychological disorders associated with lupus and improves and maintains patients' QOL, despite there being no significant reduction in the disease activity index.