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OBJECTIVE: To identify immunological pathways and markers of severity of illness associated with clinical outcomes that may represent potential therapeutic targets in the management of secondary hemophagocytic lymphohistiocytosis. INTRODUCTION: A broad range of immunomodulatory therapies is used to manage hemophagocytic lymphohistiocytosis, however the supporting evidence for these therapies is scarce. Identifying patients likely to experience more severe disease, or die, is currently extremely difficult, if not impossible. The identification of implicated cytokines in secondary disease can provide further support for the identification of high-risk patients and the development of targeted therapies. INCLUSION CRITERIA: Studies reporting immune biomarker and cytokine measurement in adult patients (age >18 years) with secondary hemophagocytic lymphohistiocytosis. METHODS: The proposed review will be conducted in line with the JBI methodology for scoping reviews. The MEDLINE (Ovid) and Embase (Ovid) databases will be searched, without date limitations. Data will be extracted using a data extraction tool developed by the reviewers. Relevant sources will be retrieved, and their citation details imported into the JBI System for the Unified Management, Assessment and Review of Information.
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AIMS: The Outcome Measures in Rheumatology Trials (OMERACT) Remission in Rheumatoid Arthritis (RA) patient perspective working group has previously found that patients prioritised independence, pain, and fatigue as key domains of remission in RA. However, there is currently no clear definition of independence. Consequently, this scoping review aimed to explore how independence is represented in the RA literature. METHODS: A comprehensive search of the EMBASE, Medline, and PsycInfo databases was performed for publications that used independence or autonomy as a disease activity measure, description of disease in remission or treatment outcome. Papers were included if they involved adult participants and were written in English, with no restrictions on study design or publication year. Two reviewers (TK and AC, AT or BJ) independently screened the abstracts. A thematic approach was applied to derive common definitions and descriptions of independence. RESULTS: 660 articles were identified, of which 58 (25 qualitative, 28 quantitative, one mixed, and four reviews) met the inclusion criteria. 86% of total participants were female. Ten publications referenced remission. Independence took many forms; in addition to physical and functional capability, it was described in relation to work, social activities, autonomy in healthcare, and household activities. Four common themes describing independence were identified: 1. A return to a state before arthritis. 2. Being physically and functionally able. 3. A sense of freedom without needing to rely on others. 4. Having control over the organisation of one's life. CONCLUSION: Although independence is frequently mentioned in the RA literature, it has various meanings, lacks a consistent definition, and is a concept rarely applied to remission. It is multi-factorial, exceeding functional ability alone, and contextualised within sociodemographic and disease factors. This scoping review provides common descriptions of independence to inform future qualitative work towards the development of an outcome measure of independence for the assessment of RA in remission.