P094 Comprehensive comparison of juvenile-onset systemic lupus erythematosus guidelines for diagnosis and management to those used in adult-onset disease
Authors: Kelly Tara E , Beresford Michael W 
Published: April 2024

Background/Aims

Juvenile-onset systemic lupus erythematosus (JSLE) is a rare autoimmune disorder that causes multisystem damage. Paucity of data limits the evidence upon which to base recommendations for diagnosis, monitoring, and management of JSLE. JSLE patients often present more acutely, with more severe complications and greater need for corticosteroids and disease-modifying therapies earlier in life when compared to those with adult-onset SLE (aSLE). The SHARE initiative generated a set of guidelines to standardise JSLE care across Europe. This aim was to undertake a comprehensive comparison aimed at comparing the SHARE guidelines to those developed by the BSR (for aSLE) to identify similarities, differences, and areas in which more research was needed.

Methods

The SHARE evidence-based, consensus-derived recommendations were established by a European wide panel of paediatric rheumatologists according to EULAR standard operating procedures using a systematic literature search with strict inclusion criteria. Adult-derived evidence was included when lack of paediatric literature existed but reviewed to adapt adult guidelines to be more suitable for paediatric use. The BSR guidelines built upon existing EULAR guidelines, selecting specific recommendations by prioritisation. Both SHARE and BSR used the same method of validating guidelines using Level of Evidence, grade of recommendation and strength of agreement by the panel (SOA) to support their recommendations. A systematic comparison was performed by identifying specific subgroups of recommendations related to: diagnosis, background, neuropsychiatric and monitoring and management guidelines. These subgroups were then compared by assessing the number of guidelines related to each group and the average SOA for each. A detailed comparison into the specific guidelines was then performed to provide in depth analysis of the similarities and differences between the groups and identify areas missing and where more work was needed.

Results

There are many similarities between the two sets of recommendations. Although some of these are a result of the manifestations being the same in adults and children, a significant proportion of these similarities were due to the lack of background data to base recommendations on for JSLE. Therefore, adult-derived guidelines, particularly the EULAR guidelines have been used which were also used as background for BSR recommendations. A key difference was the lack of recommendations regarding neuropsychiatric manifestations (NP-aSLE) in adult-onset disease. A detailed comparison between individual guidelines has been generated and potential reasons for the discrepancies between the guidelines identified.

Conclusion

The results demonstrate the need for significantly more research to inform generation of more specific JSLE guidelines, particularly in management of the disease manifestations specific to children. Many similarities between these adult and paediatric guidelines underline the importance of generating common guidelines where possible for JSLE and aSLE. More work is needed in NP-aSLE to produce more balanced generalised results.

Disclosure

T.E. Kelly: None. M.W. Beresford: None.