Background Hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) were historically thought to be distinct entities, often managed in isolation. In fact, these conditions are closely related. A collaborative approach, which incorporates expertise from subspecialties that previously treated HLH/MAS independently, is needed. We leveraged quality improvement (QI) techniques in the form of an Evidence-Based Guideline (EBG) to build consensus across disciplines on the diagnosis and treatment of HLH/MAS. Methods A multidisciplinary work group was convened that met monthly to develop the HLH/MAS EBG. Literature review and expert opinion were used to develop a management strategy for HLH/MAS. The EBG was implemented, and quality metrics were selected to monitor outcomes. Results An HLH/MAS clinical team was formed with representatives from subspecialties involved in the care of patients with HLH/MAS. Broad entry criteria for the HLH/MAS EBG were established and included fever and ferritin ≥500 ng/mL. The rheumatology team was identified as the “gate-keeper,” charged with overseeing the diagnostic evaluation recommended in the EBG. First-line medications were recommended based on the acuity of illness and risk of concurrent infection. Quality metrics to be tracked prospectively based on time to initiation of treatment and clinical response were selected. Conclusion HLH/MAS are increasingly considered to be a spectrum of related conditions, and joint management across subspecialties could improve patient outcomes. Our experience in creating a multidisciplinary approach to HLH/MAS management can serve as a model for care at other institutions. Electronic supplementary material The online version of this article (10.1186/s12969-019-0309-6) contains supplementary material, which is available to authorized users.

BackgroundWithholding live-attenuated vaccines in patients using interleukin (IL)-1 or IL-6 blocking agents is recommended by guidelines for both pediatric and adult rheumatic diseases, since there is a risk of infection in an immune suppressed host. However, this has never been studied. This retrospective, multicenter survey aimed to evaluate the safety of live-attenuated vaccines in patients using IL-1 or IL-6 blockade.MethodsWe contacted physicians involved in the treatment of autoinflammatory diseases to investigate potential cases. Patients were included if a live-attenuated vaccine had been administered while they were on IL-1 or IL-6 blockade.ResultsSeventeen patients were included in this survey (7 systemic juvenile idiopathic arthritis (sJIA), 5 cryopyrin associated periodic syndrome (CAPS), 4 mevalonate kinase deficiency (MKD) and 1 familial Mediterranean fever (FMF). Three patients experienced an adverse event, of which two were serious adverse events (a varicella zoster infection after varicella zoster booster vaccination, and a pneumonia after MMR booster). One additional patient had diarrhea after oral polio vaccine. Further, seven patients experienced a flare of their disease, which were generally mild. Eight patients did not experience an adverse event or a flare.ConclusionWe have described a case series of seventeen patients who received a live-attenuated vaccine while using IL-1 or IL-6 blocking medication. The findings of this survey are not a reason to adapt the existing guidelines. Prospective trials are needed in order to acquire more evidence about the safety and efficacy before considering adaptation of guidelines.

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Background Refugees face circumstances where their health and well-being are compromised. In this qualitative study, the aim was to understand Syrian refugee women’s needs for care and the predisposing and enabling factors to healthcare access and utilisation. Methods Out of 945 Syrian mothers who gave birth in our university hospital between 2014 and 2018, 195 were reached; out of which, 47 women were included. Semi-structured in-depth interviews were conducted and were later analysed using a qualitative content analysis approach. Depression was assessed by the Patient Health Questionnaire-9 at the end of the interview. Results Social isolation and maternal depression, language barrier and challenges while navigating the healthcare system emerged as the main themes of the study. Low educational and occupational status of the women, poor social resources, limited Turkish proficiency and unfamiliarity with the host healthcare system were identified as the predisposing factors for poor healthcare services utilisation. Conclusion Recommendations include bridging language gaps, improving the navigation of the healthcare system by visual support or in-person interpretation, and psychosocial support. Providing hospital-based language courses to mothers and social integration programs for families will improve the mothers’ well-being and indirectly care of the child.

Background Perinatal mental health is a major public health concern. In Turkey, the public hospitals operate pregnancy schools which provides an opportunity to integrate an evidence-based Thinking Healthy Programme (THP) for perinatal depression. The aim of this study is to adapt the THP for universal use in the group setting, and to understand its acceptability and feasibility for integration into the existing antenatal care programme for both face-to-face and online delivery. Methods Following an expert-led adaptation process using the Bernal Framework, field-testing was conducted on a group of women and facilitators (n=21) followed by in-depth interviews and group discussions. Data were analysed using Thematic Framework Analysis. Results Minor but significant adaptations were made to the individually delivered THP for use in the universal group pregnancy schools. Initial findings indicate that the THP-group version was relatable and acceptable to its target population and could be integrated into the antenatal care plan for delivery during face-to-face and on-line group classes. Conclusion THP is transferable to the Turkish cultural and healthcare context. The THP – group version has the potential to add value to Turkey's existing perinatal healthcare programme.

Background/Objectives: Clinically amyopathic juvenile dermatomyositis (CAJDM) is an uncommon but important subset of patients with juvenile dermatomyositis, characterized by pathognomonic cutaneous findings without clinically evident muscle weakness. With limited data available and lack of standardized management guidelines for CAJDM, we sought to describe common features, including early indicators that may be associated with progression of muscle disease, and review the course and treatment of these patients. Methods:A retrospective chart review of patients with CAJDM was conducted at four North American academic centers between the years 2000 and 2015.Results: Twenty-nine patients were included, of whom 21 (72%) were female. After a median follow-up of 4 years (IQR 1.8-5.8 years), 5 of the 29 (17%) patients with CAJDM evolved into classic juvenile dermatomyositis. Median time to develop weakness was 12 months (IQR 8-19 months) after diagnosis. The skin disease of CAJDM patients who did not develop weakness was often found to be recalcitrant with 58% of them requiring multiple systemic therapies to control their cutaneous disease. Conclusion:These results highlight the need for long-term monitoring for the development of myositis in CAJDM and for prospective studies on treatment of recalcitrant skin disease.

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