Agenesis of the gallbladder and cystic duct

Agenesis of the gallbladder and cystic duct is a rare anomaly that is usually asymptomatic. The patient may present symptoms characteristic of cholelithiasis. Its surgical confirmation requires careful dissection of the common bile duct and intraoperative cholangiography or ultrasonography to be performed, to exclude the possibility of an ectopic gallbladder. The authors describe two cases of this unusual affection and comment on its clinical, pathophysiological and diagnostic aspects

Durable renal response and safety with add-on belimumab in patients with lupus nephritis in real-life setting (BeRLiSS-LN). Results from a large, nationwide, multicentric cohort

Belimumab was recently approved for treatment of lupus glomerulonephritis (LN)

Effectiveness and safety of belimumab in patients with active systemic lupus erythematosus: results from a large, nationwide, multicentric study.

Background: Belimumab is the unique biologic therapy available for patients with SLE. Objectives: To investigate effectiveness and safety of belimumab in SLE patients in clinical practice. Methods: 458 active SLE patients (ACR criteria) from 24 Italian Centers, mean±SD age 43.5±11.3 years; mean±SD disease duration 12.3±8.7 years, were treated with belimumab (10 mg/kg day 0, 14, 28 and then every 28 days), as add-on therapy. SLEDAI-2K, anti-dsDNA, C3, C4, prednisone daily dose, DAS-28, 24H proteinuria, CLASI, PGA, Fatigue (VAS 0-10) were recorded at baseline and every 6 months. Flares were defined according to SFI. Response was evaluated according to SRI-4. Statistics were performed by pairs Ttest, chi-square test and multiple logistic regression (SPSS, version 22.0). Results: Mean±SD follow-up was 21.2±15.3 months (range 3-60). Most common features treated with belimumab were articular in 67%, mucocutaneous in 55%, and renal in 17% of cases. Improvement of clinical and serological variables, including daily prednisone dosage, was observed (Table). SRI-4 is summarized in the Figure. At the end of follow-up 293 patients (66%) were still on belimumab. Most common cause of discontinuation were inadequate response (36%), AEs (31%), and pregnancy (8%). Mean number of flare during belimumab treatment compared with the corresponding period before belimumab initiation decreased (p<0.001). SLEDAI-2K 10 was an independent predictor of response by logistic regression at month 12 and 24 (p=0.003 and p=0.025). 9,998 infusions were analyzed. 784 AEs were observed in 330 patients, SAEs were 43 in 36 patients. No severe infusion reactions were observed; 16 patients had infective SAEs, and 22 non infective SAEs. Conclusion: We confirmed the effectiveness, the steroid sparing effect and good safety profile of belimumab in our cohort

FRI0199\u2005EFFECTIVENESS AND SAFETY OF BELIMUMAB IN PATIENTSWITH ACTIVE SYSTEMIC LUPUS ERYTHEMATOSUS: RESULTS FROM A LARGE, NATIONWIDE, MULTICENTRIC STUDY

Background: Belimumab is the unique biologic therapy available for patients with SLE. Objectives: To investigate effectiveness and safety of belimumab in SLE patients in clinical practice. Methods: 458 active SLE patients (ACR criteria) from 24 Italian Centers, mean±SD age 43.5±11.3 years; mean±SD disease duration 12.3±8.7 years, were treated with belimumab (10 mg/kg day 0, 14, 28 and then every 28 days), as add-on therapy. SLEDAI-2K, anti-dsDNA, C3, C4, prednisone daily dose, DAS-28, 24H proteinuria, CLASI, PGA, Fatigue (VAS 0-10) were recorded at baseline and every 6 months. Flares were defined according to SFI. Response was evaluated according to SRI-4. Statistics were performed by pairs Ttest, chi-square test and multiple logistic regression (SPSS, version 22.0). Results: Mean±SD follow-up was 21.2±15.3 months (range 3-60). Most common features treated with belimumab were articular in 67%, mucocutaneous in 55%, and renal in 17% of cases. Improvement of clinical and serological variables, including daily prednisone dosage, was observed (Table). SRI-4 is summarized in the Figure. At the end of follow-up 293 patients (66%) were still on belimumab. Most common cause of discontinuation were inadequate response (36%), AEs (31%), and pregnancy (8%). Mean number of flare during belimumab treatment compared with the corresponding period before belimumab initiation decreased (p<0.001). SLEDAI-2K 10 was an independent predictor of response by logistic regression at month 12 and 24 (p=0.003 and p=0.025). 9,998 infusions were analyzed. 784 AEs were observed in 330 patients, SAEs were 43 in 36 patients. No severe infusion reactions were observed; 16 patients had infective SAEs, and 22 non infective SAEs. Conclusion: We confirmed the effectiveness, the steroid sparing effect and good safety profile of belimumab in our cohort

Early and Late Response and Glucocorticoid-Sparing Effect of Belimumab in Patients with Systemic Lupus Erythematosus with Joint and Skin Manifestations: Results from the Belimumab in Real Life Setting Study-Joint and Skin (BeRLiSS-JS)

Aim: To assess the efficacy of belimumab in joint and skin manifestations in a nationwide cohort of patients with SLE. Methods: All patients with skin and joint involvement enrolled in the BeRLiSS cohort were considered. Belimumab (intravenous, 10 mg/kg) effectiveness in joint and skin manifestations was assessed by DAS28 and CLASI, respectively. Attainment and predictors of DAS28 remission (<2.6) and LDA (≥2.6, ≤3.2), CLASI = 0, 1, and improvement in DAS28 and CLASI indices ≥20%, ≥50%, and ≥70% were evaluated at 6, 12, 24, and 36 months. Results: DAS28 < 2.6 was achieved by 46%, 57%, and 71% of patients at 6, 12, and 24 months, respectively. CLASI = 0 was achieved by 36%, 48%, and 62% of patients at 6, 12, and 24 months, respectively. Belimumab showed a glucocorticoid-sparing effect, being glucocorticoid-free at 8.5%, 15.4%, 25.6%, and 31.6% of patients at 6, 12, 24, and 36 months, respectively. Patients achieving DAS-LDA and CLASI-50 at 6 months had a higher probability of remission at 12 months compared with those who did not (p = 0.034 and p = 0.028, respectively). Conclusions: Belimumab led to clinical improvement in a significant proportion of patients with joint or skin involvement in a real-life setting and was associated with a glucocorticoid-sparing effect. A significant proportion of patients with a partial response at 6 months achieved remission later on during follow-up

Early disease and low baseline damage predict response to belimumab in patients with systemic lupus erythematosus

Objective. To investigate predictors of response, remission, low disease activity (LDA), damage 3 and drug discontinuation in patients with systemic lupus erythematosus (SLE) treated with 4 belimumab. 5 Methods. We retrospectively analysed data of a multicentre cohort of SLE patients receiving 6 intravenous belimumab. Proportion of patients achieving remission, LDA and SLE Responder 7 Index-4 (SRI-4) were evaluated. SLICC damage index (SDI) was calculated yearly. Predictors of 8 outcomes were investigated by multivariate logistic regression. 9 Results. We included 466 active SLE patients from 24 Italian centres: median (range) follow-up 10 18 (1-60) months. SRI-4 was achieved by 49.2%, 61.3%, 69.7%, 69.6% and 66.7% patients at 6, 11 12, 24, 36 and 48 months. Baseline predictors of response at 6 months were SLEDAI-2K≥10 (OR 12 3.14, 95%CI 2.033-4.860) and disease duration≤2 years (OR 1.94, 95%CI 1.078-3.473); at 12 13 months SLEDAI-2K≥10 (OR 3.48, 95%CI 2.004-6.025), SDI=0 (OR 1.74, 95%CI 1.036-2.923); 14 at 24 months SLEDAI-2K≥ 10 (OR 4.25, 95%CI 2.018-8.940), disease duration ≤2 years (OR 15 3.79, 95%CI 1.039-13.52); at 36 months SLEDAI-2K≥10 (OR 14.59, 95%CI 3.54-59.79) and 16 baseline smoking (OR 0.19, 95%CI 0.039-0.69). Patients spending≥25% follow-up in remission 17 (42.9%) or ≥ 50% in LDA (66.0%) accrued significantly less damage (p=0.046 and p=0.007). 18 Baseline SDI=0 independently predicted LDA ≥50% and remission ≥25%; the lower the baseline 19 damage, the higher the probability of remission ≥ 25%. Number of previous flares negatively 20 predicted belimumab discontinuation due to inefficacy (p= 0.009) 21 Conclusions. The early use of belimumab in patients with active SLE and low baseline damage 22 predicts favourable outcomes in a real-life setting