Lower BAFF Levels in Myasthenic Patients Treated with Glucocorticoids

B-cell activating factor (BAFF), a member of tumor necrosis factor family, activates B cells, promotes their survival and proliferation. BAFF is considered to have an influence on development of autoimmune diseases including myasthenia gravis (MG). We aimed to evaluate BAFF serum levels in MG patients, their potential connection with therapy and course of MG. Cross-sectional study. Two hundred eighteen adult patients with MG (67% women, age: 18–89 years, 82.6% AChR antibody seropositive (AChRAb(+)). Serum BAFF levels, their relationship with severity of clinical symptoms, therapy conducted, clinical and demographic features and other factors were analyzed. Patients with AChRAb(+) MG demonstrated significantly higher BAFF levels than MuSK-MG patients (831.2 ± 285.4 pg/ml vs. 745.6 ± 633.4 pg/ml, respectively; p = 0.030). Serum BAFF levels in women were significantly higher than in men (855.9 ± 302.5 vs. 756.6 ± 289.4, respectively; p = 0.017). Mean serum BAFF level was significantly decreased in patients who were ever treated with corticosteroids (CS) (770.4 ± 327.8 pg/ml vs. 891.3 ± 246.1 pg/ml, respectively; p = 0.001). Thymoma-MG patients demonstrated significantly lower BAFF levels (671.2 ± 244.9 vs. 833.5 ± 302.4, respectively; p = 0.044). Thymectomized patients did not differ in BAFF levels from the MG patients who had not undergone thymectomy. In multiple linear regression model, recent CS therapy and male sex were found to be independent predictors of lower BAFF levels. Serum BAFF level is decreased in patients treated with CS, which may suggest inhibiting influence of CS on BAFF—a potential mechanism contributing to the effectiveness of such therapy.

Efficacy of BAFF in Monitoring Treatment Response in Early Vietnamese Systemic Sclerosis Patients

BACKGROUND: B-cell activating factor (BAFF) is considered to have a role in the pathogenesis of systemic sclerosis (SSc). AIM: We conducted a longitudinal study on early SSc patients to determine the change in BAFF serum level after treatment and its association with organ involvements. METHODS: A total of 46 patients (32 diffuse, 14 limited) were recruited, among which 35 patients (24 diffuse, 11 limited) completed 12-month follow-up. RESULTS: Higher pretreatment BAFF levels were observed in patients with positive anti-topoisomerase antibody (ATA) (2252.1 ± 899.7 pg/ml versus 1475.5 ± 697.6 pg/ml in ATA-negative patients; p = 0.01) and muscular involvement (2741.9 ± 1039.9 pg/ml versus 1897.2 ± 762.9 pg/ml in patients without muscular involvement; p = 0.005). Lower levels were observed in patients with interstitial lung disease (ILD) (1926.7 ± 757.9 pg/ml versus 2721.6 ± 1131.4 pg/ml in non-ILD patients; p = 0.01). After treatment, BAFF level reduced significantly in diffuse SSc patients (1652.2 ± 892.7 pg/ml versus 2147.6 ± 945.5 pg/ml before treatment; p = 0.03). CONCLUSION: Patients with worsening outcome had the highest pretreatment BAFF level and was associated with increased BAFF level after treatment. BAFF can be used to predict and monitor patients’ response to therapy.

Circulating B-cell activating factor level predicts clinical response of chronic graft-versus-host disease to extracorporeal photopheresis

Extracorporeal photopheresis (ECP) is an important therapeutic option in steroid-refractory chronic graft-versus-host disease (cGVHD). Few biomarkers predicting response exist. We measured serum B-cell activating factor (BAFF) in 46 cGVHD patients receiving ECP before and during treatment course. BAFF level at 1 month of ECP predicted 3- and 6-month skin disease response, with BAFF less than 4 ng/mL associated with significant skin improvement and complete resolution in 11 of 20 patients. High BAFF at 1-month ECP associated with a worsening median 6-month skin score and resolution in 1 of 10 patients. BAFF level at 3 months also predicted the likelihood of maintaining skin disease improvement at 6 months. BAFF level was not correlated directly with extracutaneous cGVHD response, although full cutaneous responders exhibited improved extracutaneous organ response rates compared with skin nonresponders (65% vs 35%). This study suggests that early BAFF measurement during ECP for cGVHD represents a potentially useful biomarker in prediction of treatment outcome.

High Levels of B-Cell Activating Factor During the Peri-Transplant Period Are Associated with the Reduced Incidence of Acute Graft-Versus-Host Disease Following the Myeloablative Allogeneic Stem Cell Transplantation.

Abstract 2229 Poster Board II-206 To determine whether serum B-cell activating factor (BAFF) levels that were previously found to be elevated in patients with chronic graft-versus-host disease (GVHD), and C-reactive protein (CRP) levels known as a predictor for aGVHD as well as a marker for systemic inflammation during the peri-allogeneic stem cell transplantation (SCT) period, could be used to predict the occurrence of acute GVHD (aGVHD), 45 consecutive patients who had undergone myeloablative allogeneic SCT for hematologic malignancies were assessed. Serum BAFF and CRP levels were measured using ELISA (R&D Systems, Minneapolis, MN, USA) before conditioning and on day 0, day +7, and day +14 after transplant. Thirty-three of 45 patients (cumulative incidence, 73=) developed aGVHD between 16 days and 98 days after transplant. Analyses using repeated measures of ANOVA revealed that the serum BAFF levels were significantly lower in patients with aGVHD than in those without aGVHD (P=0.001), whereas no association was detected between CRP levels and aGVHD (P=0.508). Receiver operating characteristic curve (ROC) analysis showed that serum BAFF levels at every time point were available for the prediction of development of aGVHD (pre-conditioning; P=0.005, day 0; P=0.002, day +7; P=0.004, and day +14; P=0.005). Using ROC curve analysis, the identical cutoff value of 43 pg/ml at every time point that divides patients into two groups, high (> 43 pg/ml) or low (≤ 43 pg/ml) BAFF group, was determined, which could assure 75= sensitivity and 73-82= specificity for the prediction of aGVHD at every time point. The analyses of the cumulative incidence of aGVHD at each time point by BAFF groups (high vs. low) showed that serum BAFF level at every time point plays a significant predictive role for the occurrence of aGVHD (pre-conditioning; P=0.040, day 0; P=0.023, day +7; P=0.003, and day +14; P=0.026). This study is, to the best of our knowledge, the first to show that high serum BAFF levels during the peri-transplant period may play a protective role against aGVHD in humans. The results of this study also show that the BAFF levels during peri-transplant period may be considered to be a predictor for aGVHD. Further trials with a larger cohort will be necessary to determine a definite cutoff value of high BAFF level as well as to construct an index with previously known factors for the prediction of aGVHD. Disclosures: No relevant conflicts of interest to declare.