Pasireotide is a multireceptor-targeted somatostatin analogue acting at pituitary level recently approved in both the EU and US for the treatment of adult patients with CD in whom surgery has failed or is declined. The Phase III study found that pasireotide treatment is effective in reducing biochemical markers as well as in improving signs and symptoms of hypercortisolism in about 30-50 % of CD patients with a safety profile similar to other somatostatin analogues with the exception of the increased risk of hyperglycemia [8, 9]. The 2-year extended Phase II and Phase III studies confirmed these results and the longterm benefit achieved during pasireotide treatment [10, 11]. In this study, we report our 10-year experience with pasireotide in CD by reviewing and analyzing data about all the patients treated with pasireotide at our referral center and regularly

Rationale:IgG4-related disease (IgG4-RD) is an emerging immune-mediated disease characterized by multi-organ involvement and variable clinical behavior.Patient concerns:We describe the case of a 50-year-old woman affected by a rare variant of IgG4-RD, characterized by eyelid xanthelasmas, adult-onset asthma and salivary and lacrimal glands enlargement. Multiple lymphadenopathies and a pulmonary mass were present at initial evaluation.Inteventions:After a single course of rituximab (2g in 2 refracted doses), an almost complete clinical remission was achieved without chronic steroid administration.Outcomes:Magnetic resonance imaging (MRI), high-resolution computed tomography (HRCT) of the thorax, and positron emission tomography (18FDG-PET-CT) confirmed good response to treatment. Circulating plasmablasts dropped to undetectable levels as well. Xanthelasmas only remained unchanged. Remission persisted at 1-year follow-up.Lessons:Steroid therapy is still considered standard first-line therapy in IgG4-RD. However, high doses are generally required and relapses are common during the tapering phase. Rituximab is a well described steroid-sparing strategy, so far reserved to refractory cases only. In our experience, rituximab has been used as first-line monotherapy, showing great and sustained efficacy and optimal tolerability. The peculiar variant of IgG4-RD affecting our patient, the relatively low baseline plasmablast concentration, and the early placement of rituximab therapy may have facilitated the good response.

Objective to analyze the available evidence about the use of rituximab (RTX) and other biologic agents in Eosinophilic Granulomatosis with Polyangiitis (EGPA) patients and to provide useful findings to inform the design of future, reliable clinical trials. Methods A systematic review was performed. A systematic search was conducted in PubMed/MEDLINE, Scopus, Web of Science and the Cochrane library databases and an extensive literature search on other biologic agents. Results 45 papers pertinent to our questions were found: 16 retrospective cohort studies, 8 case series, 3 prospective cohort studies and 18 single case reports, for a total of 368 EGPA patients. More than 80% of evaluable patients achieved complete or partial remission with a tendency towards a higher rate of complete response in pANCA positive subgroup. Conclusion Although the majority of the evaluable EGPA patients treated with RTX appears to achieve complete remission, we strongly believe that a number of sources of heterogeneity impairs a clear interpretation of results and limits their transferability in clinical practice. Differences in design, enrollment criteria, outcome definition and measurement make a comparison among data obtained from studies on RTX and other biologic agents unreliable.

P1a In adult or pediatric patients affected by malignant hematological diseases who are candidate for autologous BMT Outcomes: Influence RR/AR Interventions Detection of circulant HBV DNA or HCV RNA (vs HV genoma not detectable) Detection of latent HBV infection (HBsAg+; HBVDNA-) (vs no latent HBV) Detection of occult HBV infection (HBsAg-; HBVDNA-; Ac anti HBcAg+) (vs no occult HBV) Detection of coagulative defect at baseline (vs no coag defects) Increased liver stiffness (Fibroscan) at baseline (vs fibroscan F0) P1c In adult or pediatric patients affected by malignant hematological diseases who are candidate for autologous BMT