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
Significance: A systematic approach to develop experts-based recommendations could have a favorable impact on clinical problems characterized by scarce and low-quality evidence as heel pressure ulcers. Recent Advances: A systematic approach was used to conduce a formal consensus initiative. A multidisciplinary panel of experts identified relevant clinical questions, performed a systematic search of the literature, and created a list of statements. GRADE Working Group guidelines were followed. An independent international jury reviewed and voted recommendations for clinical practice. Consent was developed according to Delphi rules and GRADE method was used to attribute grade of strength. Critical Issues: The extensive search of the literature retrieved 42 pertinent articles (26 clinical studies, 7 systematic reviews or meta-analysis, 5 other reviews, 2 consensus-based articles, and 2 in vitro studies). Thirty-five recommendations and statements were created. Only 1 of 35, concerning ankle-brachial pressure index reliability in diabetic patients, was rejected by the panel. No sufficient agreement was achieved on toe brachial index test to rule out the orphan heel syndrome, removing dry eschar in adult patients without vascular impairment, and using an antimicrobial dressing in children with infected heel pressure injuries. Eleven recommendations were approved with a weak grade of strength. Experts strongly endorsed 20 recommendations. Offloading, stages I and II pressure injuries, and referral criteria were areas characterized by higher level of agreement. Future Directions: We believe that the results of our effort could improve practice, especially in areas where clear and shared opinions emerged. Barriers and limits that could hinder implementation are also discussed in the article.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.