Treatment strategies for Sjögren's syndrome with childhood onset: a systematic review of the literature.

OBJECTIVES: SS with childhood onset is a rare autoimmune disease characterized by heterogeneous presentation. The lack of validated classification criteria makes it challenging to diagnose. Evidence-based guidelines for treatment of juvenile SS are not available due to the rarity of disease and the paucity of research in this patient population. This systematic review aims to summarize and appraise the current literature focused on pharmacological strategies for management of SS with childhood onset. METHODS: PubMed and MEDLINE/Scopus databases up to December 2020 were screened for suitable reports highlighting pharmacological treatment of SS with childhood onset using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses 2009 reporting checklist. Animal studies were excluded. RESULTS: A total of 43 studies (34 case reports, 8 mini case series and 1 pilot study) were eligible for analysis. The studies retrieved included girls in 88% (120/137) of cases and had very low confidence levels. HCQ was prescribed for parotid swelling, as well as in association with MTX and NSAIDs in patients with arthritis and arthralgia. Corticosteroids such as long courses of oral prednisone and i.v. methylprednisolone were commonly prescribed for children with severe disease presentations. Rituximab was mainly indicated for mucosa-associated lymphoid tissue lymphoma and renal and nervous system complications. Other conventional DMARDs were prescribed in selected cases with extraglandular manifestations. CONCLUSION: Various therapies are used for the management of juvenile SS and are prescribed based on expert clinician's opinion. There are currently no good-quality studies that allow clinical recommendations for treatment of SS with childhood onset.

Biomarkers Associated with Organ-Specific Involvement in Juvenile Systemic Lupus Erythematosus.

Juvenile systemic lupus erythematosus (JSLE) is characterised by onset before 18 years of age and more severe disease phenotype, increased morbidity and mortality compared to adult-onset SLE. Management strategies in JSLE rely heavily on evidence derived from adult-onset SLE studies; therefore, identifying biomarkers associated with the disease pathogenesis and reflecting particularities of JSLE clinical phenotype holds promise for better patient management and improved outcomes. This narrative review summarises the evidence related to various traditional and novel biomarkers that have shown a promising role in identifying and predicting specific organ involvement in JSLE and appraises the evidence regarding their clinical utility, focusing in particular on renal biomarkers, while also emphasising the research into cardiovascular, haematological, neurological, skin and joint disease-related JSLE biomarkers, as well as genetic biomarkers with potential clinical applications.

Screening for enteroviral meningitis in infants and children-Is it useful in clinical practice?

Enteroviral meningitis in infants and children commonly leads to hospital admission. Diagnosing viral meningitis can be difficult clinically. We examined the usefulness of enteroviral polymerase chain reaction (PCR) testing using cerebrospinal fluid (CSF) samples on clinical practice by comparing positive enteroviral CSF PCR cases (n = 39/136) to negative controls using both clinical outcomes and laboratory parameters. A positive result correlated with a reduced admission to high dependency unit, reduced the duration of antibiotics and a shorter length of stay (P < .05). Adjusted CSF white cell count > 5/µL correlated with positive PCR (P < .05) but would have missed 32% of cases of enteroviral meningitis. Following these findings, an algorithm for the management of suspected viral meningitis has been introduced.

Self-perceived disease activity was the strongest predictor of COVID-19 pandemic-related concerns in young people with autoimmune rheumatic diseases, irrespective of their gender, with females reporting higher concerns.

Objectives: We report the results of a pilot young patient survey that targeted patients with JSLE and JDM, exploring well-being, resilience and general concern about the coronavirus disease 2019 (COVID-19) pandemic as well as self-assessment of disease activity. Methods: The survey was completed anonymously by patients who had been approached via the automatically generated hospital database between June and December 2020. In addition to disease characteristics, geographic location, education and employment level, we explored young patients' resilience, mood and feelings, mental well-being, self-assessed disease activity and general COVID-19 concerns using validated tools and visual analogue scales. Results: This pilot study found that self-perceived disease activity was the strongest predictor of COVID-19 concern, irrespective of gender, employment and education status or well-being and resilience. Generalized concerns regarding the COVID-19 pandemic were significantly higher in females, although their self-reported DASs were comparable to male respondents. Conclusion: Our findings highlight a gender bias in the generalized concern related to the COVID-19 pandemic, irrespective of the examined potential confounders. This suggests the need for further research around young patient self-reported outcomes outside hospital visits, especially in the context of gender differences and potential challenges of future pandemics.

Marital Status and Gender Differences as Key Determinants of COVID-19 Impact on Wellbeing, Job Satisfaction and Resilience in Health Care Workers and Staff Working in Academia in the UK During the First Wave of the Pandemic.

Background: The COVID-19 pandemic is an unprecedented global public health crisis that continues to exert immense pressure on healthcare and related professional staff and services. The impact on staff wellbeing is likely to be influenced by a combination of modifiable and non-modifiable factors. Objectives: The aim of this study is to evaluate the effect of the COVID-19 pandemic on the self-reported wellbeing, resilience, and job satisfaction of National Health Service (NHS) and university staff working in the field of healthcare and medical research. Methods: We conducted a cross sectional survey of NHS and UK university staff throughout the COVID-19 pandemic between May-November 2020. The anonymous and voluntary survey was disseminated through social media platforms, and via e-mail to members of professional and medical bodies. The data was analyzed using descriptive and regression (R) statistics. Results: The enjoyment of work and satisfaction outside of work was significantly negatively impacted by the COVID-19 pandemic for all of staff groups independent of other variables. Furthermore, married women reporting significantly lower wellbeing than married men (P = 0.028). Additionally, the wellbeing of single females was significantly lower than both married women and men (P = 0.017 and P < 0.0001, respectively). Gender differences were also found in satisfaction outside of work, with women reporting higher satisfaction than men before the COVID-19 pandemic (P = 0.0002). Conclusion: Our study confirms that the enjoyment of work and general satisfaction of staff members has been significantly affected by the first wave of the COVID-19 pandemic. Interestingly, being married appears to be a protective factor for wellbeing and resilience but the effect may be reversed for life satisfaction outside work. Our survey highlights the critical need for further research to examine gender differences using a wider range of methods.