Actigraphic and self-reported characterization of sleep in systemic lupus erythematosus patients.

OBJECTIVES: In a cross-sectional study, we explored possible differences in sleep parameters between SLE patients and age- and gender-matched healthy controls through actigraphic and self-reported measures. Furthermore, we aimed to identify possible predictors of such disturbances in the patient cohort. METHODS: Participants' sociodemographic data and sleep parameters were collected. Sleep parameters were evaluated through the Pittsburgh Sleep Quality Index, the Insomnia Severity Index and 7-day actigraphic monitoring. The 10-item Perceived Stress Scale was used to investigate stress. Disease activity and daily glucocorticoid dose were assessed in SLE patients. Possible predictors of the SLE group were explored through two binomial logistic models. Within the SLE group, possible predictors of sleep parameters were tested estimating multiple linear regression models. RESULTS: A total of 40 SLE patients and 33 controls were included in the study. The SLE group showed worse sleep maintenance actigraphic parameters (i.e. sleep efficiency and wake after sleep onset), higher total sleep time and higher perceived stress. Within the SLE cohort, the daily glucocorticoids dose was associated with an impairment in sleep maintenance despite no reduction in sleep duration, typical of normal sleep duration insomnia, whereas perceived stress was associated with short sleep duration insomnia. CONCLUSION: Compared with healthy controls, SLE patients showed worse sleep quality and greater perceived stress severity. As glucocorticoids and perceived stress are associated with different types of insomnia in these patients, a multidimensional approach to both sleep characterization and therapy might be preferred.

How do systemic lupus erythematosus patients with very-long disease duration present? Analysis of a monocentric cohort.

OBJECTIVE: to describe the disease path and the very long-term outcome in a monocentric cohort of patients with Systemic Lupus Erythematosus (SLE). METHODS: SLE patients with a disease duration of at least 15 years from diagnosis were enrolled. The number of hospitalizations, the disease flares occurred over the disease course and the organ damage accumulation were evaluated at 1, 2, 3, 4, 5, 10 years from diagnosis and at last observation in 2019 as well. Disease state, ongoing therapies and quality of life measures were also assessed at last visit. RESULTS: 126 Caucasian SLE patients were included in the analysis (95% female, median age 47.5 IQR 41-53, median disease duration 21 IQR19-26). At last visit, the majority of the patients (78.6%) was on LLDAS (remission included), 53.4% were on GC treatment and 35.7% on immunosuppressant. Furthermore, 53.2% had at least one organ damage. The majority of patients (66.7%) presented a relapsing-remitting course, for a total of 158 flares during the disease course (incidence rate: 0.79/patient-year); moreover, 84.9% of the cohort experienced at least one hospital admission, amounting to a total of 328 hospitalizations (incidence rate: 0.85/patient-year). The main reason for admission was disease activity, while the percentage of hospitalizations due to other causes has been growing over the 10 years of follow-up. CONCLUSION: after a very long period of disease, most of the patients with SLE are in remission and are not taking GC therapy; however, the risk of incurring in disease flare remains a real problem.

One year in review 2017: systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that predominately affects women. It is characterised by a broad spectrum of clinical manifestations, however, its course and organ involvement are unpredictable. Although over the last few decades an improvement in survival for SLE patients has been observed, pathogenic mechanisms underlying this disease are still unclear. Comorbidities, due to both disease and treatment, as well as multiple aspects of SLE, are under intensive investigation. Following the previous annual reviews of this series, we hereby provide a critical digest of the recent papers on SLE focusing on pathogenesis, clinical and laboratory features, as well as current and new therapeutic strategies published over the last year.

One year in review 2016: Sjögren's syndrome.

Sjögren's syndrome (SS) is a complex heterogeneous disease charactered by a broad spectrum of clinical and serological manifestations, including non-Hodgkin's lymphoma (NHL). Last year, 2015, was an exciting year for research into SS with novel insights into disease pathogenesis, clinical aspects and long-term outcomes. In addition, the use of biologic therapy in SS is rapidly expanding, with new evidence emerging regarding potential therapeutic targets. In this article, we will provide an overview of the recent literature on the pathogenesis, clinical features and novel treatments of SS.

One year in review 2015: systemic lupus erythematosus.

Systemic lupus erythematosus is a multiorgan autoimmune disease with a highly variable clinical course, typically involving women in childbearing age. At present, many aspects of its pathogenesis still remain unclear. Moreover, although a significant increase of patient survival has been observed in the last decades, morbidity and mortality remain high. Finally, SLE impacts negatively on the health-related quality-of-life of patients. Therefore, multiple aspects of SLE still remain challenging and it continues to be the object of both clinical and translational clinical research. Herewith, we provide a critical digest of the recent literature on this topic.

Illness perception in systemic lupus erythematosus patients: The roles of alexithymia and depression.

OBJECTIVE: Alexithymia and depressive mood have been described as important dimensions of several medical diseases. Systemic lupus erythematosus is a chronic condition characterized by unpredictable clinical manifestations. The relationships between alexithymia, depression, and illness perception were examined in systemic lupus erythematosus patients. The interrelationships between psychological factors, such as alexithymia and depressive mood, were explored in systemic lupus erythematosus patients, and associations between these factors and illness perception in SLE were examined. We hypothesized that alexithymia and negative perceptions of illness would be associated in SLE patients, and depression would mediate this relationship. METHODS: Subjects were 100 consecutive systemic lupus erythematosus patients attending the outpatient clinic at the University of Pisa rheumatology unit. They completed the Toronto Alexithymia Scale, Beck Depression Inventory, and Revised Illness Perceptions Questionnaire. Clinical variables were measured, disease activity was evaluated using the European Consensus Lupus Activity Measure, and damage was assessed using the Systemic Lupus International Collaborative Clinics/American College of Rheumatology Damage Index. RESULTS: There were no associations between clinical variables, alexithymia, and depression. The results highlight the existence of significant links between alexithymia and illness perception for systemic lupus erythematosus patients. Moreover, our data suggest that some of these links are mediated by depression, which is the direct predictor of different aspects of perceived health. CONCLUSION: Our findings suggest that studying the role of psychological factors, such as alexithymia and depression, may contribute to a more comprehensive perspective of systemic lupus erythematosus, including their impact on patients' beliefs about treatment effectiveness and emotional adaptation to chronic disease.

Systemic vasculitis: an annual critical digest of the most recent literature.

Herewith we provide our annual digest of the recent literature on systemic vasculitis in which we reviewed all the articles published during the last 12 months on large-, medium- and small-vessel vasculitis, and selected the most relevant studies regarding the epidemiology, pathogenesis and management of systemic vasculitis. In particular, we focused the attention on giant cell arteritis, ANCA-associated vasculitis and cryoglobulinaemia.

Remission and low disease activity in systemic lupus erythematosus: an achievable goal even with fewer steroids? Real-life data from a monocentric cohort.

OBJECTIVES: To evaluate what proportion of patients fulfil the DORIS definition of remission, the definition of lupus low disease activity state (LLDAS) and LLDAS with a glucocorticoid (GC) dosage ≤5 (LLDAS5) in a longitudinal monocentric cohort of patients with SLE; to identify predictors of sustained remission and LLDAS attainment; to evaluate the effect of sustained remission and LLDAS on damage accrual over a period of 5 years and compare the two conditions in terms of clinical outcomes. METHODS: Retrospective analysis of data prospectively collected from patients with SLE followed from 2012 to 2016. RESULTS: 115 patients were included in this analysis. At baseline, 72% of patients were on LLDAS and almost all patients also fulfilled the LLDAS5 definition; 45% of patients were in remission on treatment, 12% were in remission off treatment, 26% were in complete remission on treatment, 2% were in complete remission off treatment. Disease activity at baseline was the strongest predictor of subsequent LLDAS and remission; the presence of joint and cutaneous manifestations was associated with a minor likelihood to achieve LLDAS or remission during follow-up.Patients in remission and LLDAS for the whole follow-up period accrued significantly less organ damage; on the contrary, patients who maintained all kinds of remissions or LLDAS for less than 50% of the time did not show any differences in damage accrual with respect to the rest of the cohort. CONCLUSION: Remission and LLDAS, even with reduced GC use, are an achievable goal in clinical practice; sustained LLDAS and remission are both associated with reduced damage accrual.

Tacrolimus in non-Asian patients with SLE: a real-life experience from three European centres.

OBJECTIVES: To analyse the real-life practice on the use of Tacrolimus (TAC) in patients with systemic lupus erythematosus (SLE) from three European SLE referral centres. METHODS: Adult patients with SLE regularly followed at three European referral centres were included. Demographics, cumulative organ involvement, treatment history, Systemic Lupus Disease Activity Index (SLEDAI), laboratory features and physician's judgement were collected at baseline and at 3-6-12 months after starting TAC. RESULTS: 29 patients were included (89% female, mean age 38±9 years). Ethnicity was predominantly Caucasian (82%), Black African (11%), Hispanic (3.5%) and Caribbean (3.5%). The main indications for TAC prescription were renal involvement (82.7%), arthritis (10.3%), cutaneous manifestations (6.8%), haematological manifestations (6.8%), serositis (3.4%). At 3 months, there was a clinical improvement in 21 patients (72.4%) and 9 of these experienced a complete resolution of symptoms (31%). This corresponds to: (1) a significant decrease in the mean SLEDAI; (2) a significant decrease in the mean 24 hours proteinuria; a significant increase in C3 and stable creatinine values. At 6 months (n=25), the physician declared an improvement in 19 patients (76%) and a complete resolution of symptoms in 9 (36%). The same trend was observed at 12 months of follow-up. TAC was discontinued in nine pts (31%); reasons for discontinuation were inefficacy (13.8%), drug intolerance (10%) and disease remission (6.9%). CONCLUSIONS: Despite the limitation due to the small number of patients and the uncontrolled nature of the study, these data show that TAC can be considered a valid therapeutic option in patients with SLE, especially for renal involvement.

Pregnancy and menopause in patients with systemic lupus erythematosus and/or antiphospholipid syndrome. Practical messages from the EULAR guidelines.

Over the last few decades, reproductive medicine has observed an improvement in the management and outcome of pregnancy in connective tissue diseases, such as systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS). However, pregnancy and related issues remain a challenge in these patients. In routine clinical practice, health professionals dealing with SLE and APS need to consider the numerous aspects of the reproductive life of their patients, such as pregnancy, family planning, fertility, contraception, cancer surveillance, and menopause. The new European League Against Rheumatism recommendations for women's health and family planning reflect the need for a novel approach to communication in the patient-physician relationship. Preconception counseling is essential to ensure optimal pregnancy outcomes through a careful risk stratification involving disease activity, organ involvement, autoantibody profile, use of drugs, and previous pregnancy outcomes, as well as to ensure better preventive and therapeutic strategies to limit complications. In patients with stable/inactive disease and low risk of thrombosis, adequate hormonal contraception and menopausal replacement therapy should be recommended. Assisted reproductive techniques can be safely used in these patients, but anticoagulation or low-dose aspirin (or both) should be added in those with positive antiphospholipid antibody titers. All menstruating women should be counseled on the possibility to preserve fertility with gonadotropin- ­releasing hormone analogues if receiving alkylating agents. Strict clinical, serological, laboratory, and multidisciplinary monitoring during pregnancy is mandatory to early recognize and effectively treat disease flares or obstetric complications. Doppler ultrasonography and fetal biometry should be regularly performed, especially in the second and third trimesters. Physicians should recommend screening for cervical dysplasia related to human papillomavirus (HPV) infection, especially during immunosuppressive therapy, and HPV immunization can be used in women with stable/inactive disease.