Search

Objectives: To compare the main characteristics of two inception cohorts (Italian [ITC] and Spanish [SPC]) cohorts of patients with systemic lupus erythematosus (SLE) at the time of diagnosis and at one year of follow-up., Methods: Demographic, clinical and immunological characteristics, and treatments at SLE diagnosis and at 12 months of follow-up of ITC and SPC were compared., Results: One hundred and sixty-four patients in the ITC and 231 patients in the SPC were compared. the patients from ITC were younger at SLE diagnosis (41.1±15.0 years vs. 46.4±15.6 years; p<0.001) and had a higher prevalence of arthritis (62.8% vs. 45.5%; p=0.001), serositis (25.6% vs. 16.0%; p=0.026), neurological involvement (7.9% vs. 1.7%; p=0.006), and immunological abnormalities (anti-dsDNA, anti-Sm, antiphospholipid antibodies) (93.9% vs. 77.8%; p<0.001). Conversely, photosensitivity (29.5% in ITC vs. 45.9% in SPC; p=0.001) and oral ulcers (12.4% vs. 30.3%; p<0.001) were more frequent at onset of SLE in the Spanish patients. At the first 12 months of follow-up, these differences were maintained. At SLE onset, more Italian patients received glucocorticoids (85.4% vs. 50.2%; p<0.001) and immunosuppressive agents. At 12 months of follow-up, more Spanish patients were treated with antimalarials (75.6% in ITC vs. 90.0% in SPC; p<0.001). Conversely, the use of glucocorticoids was lower in SPC (89.0% in ITC vs. 57.1% in SPC; p<0.001)., Conclusions: These cohorts presented different profiles in terms of pattern of organ/system involvement and disease treatment, possibly as a consequence of patient selection or different disease management approaches between Italy and Spain.

Objective: To compare the efficacy and safety of enteric-coated mycophenolate sodium (EC-MPS) versus azathioprine (AZA) in patients with active systemic lupus erythematosus (SLE) disease., Methods: A multicentre, 24-month, superiority, open-label, randomised controlled trial (NCT01112215) was conducted with 240 patients (120 per arm) receiving either EC-MPS (target dose: 1440 mg/day) or AZA (target dose: 2 mg/kg/day) in addition to prednisone and/or antimalarials. The primary endpoint was the proportion of patients achieving clinical remission, assessed by SLE Disease Activity Index 2000 (SLEDAI-2K) and British Isles Lupus Assessment Group (BILAG), at 3 and 24 months. Secondary endpoints included time to clinical remission, BILAG A and B flare rates, time to flare, corticosteroid reduction and adverse events (AEs)., Results: Proportion of patients achieving clinical remission (clinical SLEDAI=0) was higher in the EC-MPS group at 3 (32.5% vs 19.2%; treatment difference, 13.3 (CI 2.3 to 24), p=0.034) and 24 months (71.2% vs 48.3%; treatment difference, 22.9 (CI 10.4 to 34.4), p<0.001). EC-MPS was superior with respect to time to clinical remission (HR 1.43; 95% CI 1.07 to 1.91; p=0.017). BILAG A/B and B flares occurred more frequently in the AZA group (71.7% vs 50%, p=0.001 and 21.67% vs 8.3%, p=0.004, respectively). EC-MPS was superior with respect to time to first BILAG A/B (HR 1.81; 95% CI 1.3 to 2.56; p=0.0004) and BILAG A flare (HR 2.84; 95% CI 1.37 to 5.89; p=0.003). AEs were similar in both groups except for leucopenia that occurred more frequently with AZA., Conclusions: EC-MPS was superior to AZA in treating SLE and preventing further relapses., Trial Registration Number: NCT01112215; Results., Competing Interests: Competing interests: None declared., (© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Shrinking lung syndrome (SLS) is a rare and less known complication mainly associated with systemic lupus erythematosus (SLE). In this study, we analyze the clinical features, investigation findings, approaches to management, and outcome in a case series of 9 adult patients with SLE and SLS diagnosed during a 35-year period in 3 referral tertiary care hospitals in Spain. Additionally, we reviewed 80 additional cases previously reported (PubMed 1965-2015). These 80 cases, together with our 9 patients, form the basis of the present analysis.The overall SLS prevalence in our SLE population was 1.1% (9/829). SLS may complicate SLE at any time over its course, and it usually occurs in patients without previous or concomitant major organ involvement. More than half of the patients had inactive lupus according to SELENA-systemic lupus erythematosus disease activity index (SLEDAI) scores. Typically, it presents with progressive exertional dyspnea of variable severity, accompanied by pleuritic chest pain in 76% of the cases.An important diagnostic delay is common. The diagnostic tools that showed better yield for SLS detection are the imaging techniques (chest x-ray and high-resolution computed tomography) along with pulmonary and diaphragmatic function tests. Evaluation of diaphragm dome motion by M-mode ultrasonography and phrenic nerve conduction studies are less useful.There are no standardized guidelines for the treatment of SLS in SLE. The majority of patients were treated with medium or high doses of glucocorticoids. Several immunosuppressive agents have been used in conjunction with steroids either if the patient fails to improve or since the beginning of the treatment. Theophylline and beta-agonists, alone or in combination with glucocorticoids, have been suggested with the intent to increase diaphragmatic strength.The overall long-term prognosis was good. The great majority of patients had significant clinical improvement and stabilization, or mild to moderate improvement on pulmonary function tests. The mortality rate was very low.

Objectives: This is the first Spanish multicentric inception lupus cohort, formed by SLE patients attending Spanish Internal Medicine Services since January 2009. We aimed to analyse drug therapy during the first year of follow-up according to disease severity., Methods: 223 patients who had at least one year of follow-up were enrolled upon diagnosis of SLE. Therapy with prednisone, pulse methyl-prednisolone, hydroxychloroquine, immunosuppressives and calcium/vitamin D was analysed., Results: Prednisone was given to 65% patients, at a mean (SD) daily dose of 11 (10) mg/d. 38% patients received average doses >7.5 mg/d during the first year. Patients with nephritis and with a SLEDAI ≥6 were treated with higher doses of prednisone. 81% of patients were treated with hydroxychloroquine, with higher frequency among those with a SLEDAI ≥6 (88% vs. 68%, p<0.001). The use of immunosuppressive drugs and methyl-prednisolone pulses was higher in patients with a baseline SLEDAI ≥6, however, differences were no longer significant when patients with lupus nephritis were excluded. The use of calcium/vitamin D increased with the dose of prednisone, however, 43% of patients on medium-high doses of prednisone did not take any calcium or vitamin D., Conclusions: This study gives a real-world view of the current therapeutic approach to early lupus in Spain. The generalised use of hydroxychloroquine is well consolidated. There is still a tendency to use prednisone at medium to high doses. Pulse methyl-prednisolone and immunosuppressive drugs were used in more severe cases, but not as steroid sparing agents. Vitamin D use was suboptimal.

Objective: To evaluate the utility of systemic lupus erythematosus (SLE) initial clinical manifestations and the SLE Disease Activity Index (SLEDAI) for identifying patients who will have a remission., Methods: We studied 100 SLE patients (85 females, 15 males) and identified all patients who had remission (defined as at least one continuous year during which lack of disease activity permitted withdrawal of all treatment to suppress general lupus activity of a particular clinical manifestation). Changes in laboratory parameters without clinical activity, thus not requiring treatment, did not invalidate remission. We did not include any patient who had never required treatment. We evaluated the SLEDAI values and the main SLE manifestations at the time of diagnosis of SLE, and also every 3 months during the first year of disease., Results: Twenty-four of the 100 SLE patients achieved remission that occurred a mean of 64 months after the diagnosis. They remained in remission for a mean of 55 months. There were no statistical differences in SLEDAI values and the initial manifestations (including renal and cerebral) between patients who reached remission and those who did not. The patients who have a higher SLEDAI score take longer to achieve remission., Conclusion: SLE patients with severe initial clinical manifestations and higher SLEDAI values may achieve clinical remission.

Objective: Systemic lupus erythematosus (SLE) predominantly affects young patients. SLE starting in later life has a clinical presentation different than in younger patients. We have used the SLE Disease Activity Index (SLEDAI) to explore the relationship between age of onset and disease activity., Methods: We selected all patients controlled in our hospital at the moment of clinical diagnosis of SLE (100 patients; 85 females and 15 males). They were classified in two groups: those with early onset (>50 y) and those with late onset (>50 y) based on their age at the moment of clinical diagnosis of SLE., Results: In 12 patients the onset of SLE was >50 y (10 females and two males; mean age 59 y). The early onset patients had significantly higher SLEDAI values at the presentation and during the first year of disease with respect to elderly patients. Antibodies to DNA and hypocomplementemia were detected more often in younger patients., Conclusion: Our results confirm using SLEDAI, that the lupus of the elderly patients is a distinct clinical subgroup with a milder course of disease.

We present three cases of patients with systemic lupus erythematosus (SLE) and osteonecrosis or avascular necrosis (AV). Although, the pathogenesis of osteonecrosis is controversial and multifactorial, the glucocorticoids therapy is the most important factor contributing to the lesion. We report the clinical presentation of the three patients. We comment the characteristics of AV, the diagnosis and the treatment of this uncommon complication in SLE patients.

A loss in bone mass was reported in premenopausal systemic lupus erythematosus (SLE) women, but this problem has not been studied in SLE males. We evaluated bone mineral density (BMD) in SLE males and the relationship between prolactin (PRL) and testosterone with BMD. We also studied the controversial effect of steroid therapy on BMD in these patients. We measured BMD in the lumbar spine and at the hip in 20 SLE men (mean age 37 y) and in the controls (n = 40). We measured PRL and testosterone in serum and saliva. The mean dose of prednisone at the time of study was 11.6 mg; and cumulative dose was 17.6 g. No significative decrease in BMD was detected in SLE males vs controls; either in the lumbar spine (1.00 +/- 0.1 vs 1.05 +/- 0.1 g/cm2) or in the femoral neck (0.84 +/- 0.1 vs 0.87 +/- 0.1 g/cm2). No patient or control had osteoporosis or fractures. We did not find any relationship between BMD and cumulative dose and baseline dose of corticosteroids. The mean values of PRL and testosterone (serum and salivary) were in the normal range. We did not find any correlation between BMD, PRL and androgens. This study did not show a loss in bone mass in SLE men on corticosteroid therapy.

Objective: To verify the possible relationship between the presence of antiphospholipid antibodies (APLA) and the presence of epileptic seizures in patients with systemic lupus erythematosus (SLE)., Methods: A total of 168 patients with SLE were studied. Fifteen patients had antecedents of epilepsy which were non attributable to a cause other than SLE. Epilepsy was diagnosed on clinical and electroencephalographic grounds. Antibodies to cardiolipin (CLa) and lupus anticoagulant (LA) were measured., Results: Epileptic seizures were generalized in 13 and partial in two patients. The lupus anticoagulant was positive in 40% of patients with epilepsy compared to 32% in the control group; CLa IgG in 53.5% compared to 65.6%, and CLa IgM in 40% compared to 35.2%. Differences were never statistically significant. Neither when patients with moderate/high CLa levels were studied. Seven patients (40%) had some of the classical manifestations of antiphospholipid syndrome., Conclusions: No association was found between positive APLA and epilepsy in patients with SLE.

The prevalence of infection with VZV in 145 patients with SLE was investigated, with a mean follow-up of 7.6 years; its relationship with different variables, particularly with therapy of the underlying disease, was analyzed. Twenty episodes of VZV infection in 19 patients were diagnosed (13.1%). In no case was the therapeutic regime changed nor was worsening of SLE observed. There was neither dissemination of herpes nor superinfection. An increase in the number of VZV infections was observed in patients with SLE under corticosteroid therapy (p = 0.04) and particularly when drug administration was on a daily basis (p = 0.00006). Cytotoxic agents also favored the infection (p = 0.0014). VZV infection is of a benign nature in SLE and its emergence is favored by immunosuppressive agents. The risk is lower if corticosteroid administration is on alternate days. There is no need to decrease therapy for SLE.

Objective: To evaluate bone mineral density (BMD) in premenopausal patients with systemic lupus erythematosus (SLE)., Methods: We measured BMD by dual energy x ray absorptiometry at lumbar vertebrae L2-4 and at the right femoral neck in 74 premenopausal white patients (mean age 30.8 years) with SLE who were receiving glucocorticoid therapy, and in a control group., Results: The mean cumulative dose of prednisone was 32.5 (SD 28) g. The mean dose at the time of absorptiometry was 13.7 (6.9) mg. BMD was significantly reduced at the spine and at the femoral neck in SLE patients when compared with the control group: L2-4 = 0.943 (0.1) g/cm2 v 1.038 (0.1) g/cm2 (p < 0.001); femoral neck = 0.766 (0.09) g/cm2 v 0.864 (0.1) g/cm2 (p < 0.001). Nine patients (12.1%), but none of the control group, had a BMD less than the reference range., Conclusion: BMD in premenopausal patients with SLE was less than that in a control group and less than the reference range of values defining the presence of osteoporosis in 12.1%. We did not find a relationship between BMD and either cumulative or baseline dose of corticosteroid therapy.

Because doctors are reluctant to diagnose systemic lupus erythematosus (SLE) in elderly patients, the initial diagnosis in this age group is usually tardy. Furthermore, the presenting manifestations in these patients are commonly atypical. We describe a 72-year-old woman in whom chronic ascites with antiphospholipid antibodies was the initial predominant manifestation of subsequently fatal SLE. Only 13 cases of chronic lupus ascites have been reported in the English and French literature. Our patient represents the first case reported in an elderly person.