Objective The aim of this study was to determine the prevalence, predictors and progression of organ damage in a monocentric cohort of systemic lupus erythematosus patients with a long follow-up. Organ damage was assessed by the Systemic Lupus International Collaborating Clinics Damage Index one year after diagnosis and every five years. Disease activity was measured by the systemic lupus erythematosus disease activity index (SLEDAI)-2K at the beginning of the follow-up. Univariate and multivariable analyses were used to detect items associated with damage. A total of 511 systemic lupus erythematosus patients (92% females, 95% Caucasian), prospectively followed from 1972 to 2014, were included. Results After a mean disease duration of 16 years (SD: 9.5) and a mean follow-up of 12.9 years (SD: 8.8), 354 patients (69.3%) had accrued some damage: 49.7% developed mild/moderate damage, while 19.5% showed severe damage. Damage was evident in 40% of 511 patients one year after diagnosis, and its prevalence linearly increased over time. Longer disease duration, higher SLEDAI, severe Raynaud's, chronic alopecia and cerebral ischaemia were significantly associated with organ damage. No associations between damage and autoantibodies, including anti-dsDNA, anti-Sm or antiphospholipid antibodies, were observed. Anyway, antiphospholipid syndrome and anticardiolipin antibodies predicted the development of neuropsychiatric damage. The ocular, musculoskeletal and neuropsychiatric systems were the most frequently damaged organs, with a linear increase during follow-up. Conclusion A high rate of moderate and severe damage has been detected early in a wide cohort of young lupus patients, with a linear trend of increase over time. Disease activity and long duration of disease predict damage, while antiphospholipid antibodies play a role in determining neuropsychiatric damage.

A clinically significant aPL profile is associated with an increased risk of organ damage accrual during a 15-year follow-up in SLE patients.

summaryThe objective is to investigate the role of clinically significant antiphospholipid antibodies (aPL) in a cohort of systemic lupus erythematosus (SLE) patients. All SLE patients followed for at least 5 years and with available aPL profile at the beginning of the follow-up in our center were studied. Clinically significant aPL were defined as: positive lupus anticoagulant test, anti-cardiolipin and/or antiβ2Glycoprotein I IgG/IgM >99 th percentile on two or more occasions at least 12 weeks apart. Patients with and without clinically significant aPL were compared by univariate (Chi square or Fisher's exact test for categorical variables and Student's t or Mann-Whitney test for continuous variables) and multivariate analysis (logistic regression analysis). P values <0.05 were considered significant. Among 317 SLE patients studied, 117 (37%) had a clinically significant aPL profile at baseline. Such patients showed at univariate analysis an increased prevalence of deep venous thrombosis, pulmonary embolism, cardiac valvular disease, cognitive dysfunction and antiphospholipid syndrome (APS), but a reduced prevalence of acute cutaneous lupus and anti-extractable nuclear antigens (ENA) when compared with patients without clinically significant aPL. Multivariate analysis confirmed the association between clinically significant aPL and reduced risk of acute cutaneous lupus [p=0.003, odds ratio (OR) 0.43] and ENA positivity (p<0.001, OR 0.37), with increased risk of cardiac valvular disease (p=0.024, OR 3.1) and APS (p<0.0001, OR 51.12). Triple positivity was the most frequent profile and was significantly associated to APS (p<0.0001, OR 28.43). Our study showed that one third of SLE patients had clinically significant aPL, and that this is associated with an increased risk, especially for triple positive, of APS, and to a different clinical and serological pattern of disease even in the absence of APS.

Background Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disorder that can be severe and life threatening. Death in patients with SLE may be due to many factors, including persistent lupus activity, complications of treatment, or to long-term sequelae. Objectives The aim of our study was to evaluate an advantage on surviving in SLE patients related to treatment. Methods We retrospectively evaluated 120 SLE patients, attending our outpatients Clinic, from 1983 to 2013, revised according to SLICC/ACR criteria [1]. Patients were divided into 2 groups: dead patients (n=38) and control group of patients alive (n=82). Clinical, laboratoristic and treatment data were obtained from clinical charts. SLE activity was calculated with Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) at the onset and while damage was evaluated with System Lupus International Collaborating Clinics/ACR Damage Index for Systemic Lupus Erythematosus (SLICC) at the last visit. Two-tailed Student's t-test for continuous variables and Fisher's exact test or Yates's Chi squared test for categorical variables were used. Results clinical manifestations and autoantibodies profile did not show statistical differences among the groups; also the mean age at the onset (36.5 vs 35.6 years) and duration of the disease (14 vs 16 years) were similar. Disease activity at the onset in the deceased group, obtained with SLEDAI-2K, was significantly lower than controls (11.3 vs 8.7; p=0.015), while SLICC/ACR damage index calculated at the last visit was not statistically significant (1.9 vs 2.5; p=n.s). The most frequent causes of death were cancer (12 patients) and circulatory disease (10 patients), while SLE was the assigned cause of death in 2 cases. Assumption of hydroxychloroquine was less frequent (55% vs 87%, p≤0.0001, OR 0.17; CI95% 0.05-0.47) and duration of assumption was shorter among patients dead than alive controls (31 vs 72 months, p=0.0002). The number of the patients that assumed steroids among the two groups was not different (94.7 vs 93.9%) but deceased patients received a mean daily dosage higher than alive (12 mg/day of prednisone equivalent vs 8.4 mg/day; p=0.01), and higher number of deceased patients received a daily dosage of prednisone equivalent more than 12.5mg (31% vs 13%; p=0.025, OR 2.97; CI95% 1.06-8.38). Comparison between the deceased patients that received more than 12.5mg of steroid versus lower dosage revealed that the subgroup that received a higher dosage was more frequently treated with at least one immunosuppressant drug than the other subgroup (91.6% vs 45.8%, p=0.01, OR 13; CI95%1.30-30); moreover SLEDAI-2K at the onset was higher among deceased patients treated with higher dosage of steroid than the other deceased patients (11.1 vs 7.31, p=0.01); while SLICC was higher but did not reach statistical difference (3.16 vs 2.19. p=0.06). Conclusions Our preliminary result seems to confirm that the use of elevated dosage of steroids seems to be associated with higher disease activity, hi...