Childhood-onset systemic lupus erythematosus (cSLE) is a severe, potentially life- threatening, chronic, multisystem autoimmune disease that is associated with significant morbidity. With good-quality evidence-based guidelines on the diagnosis and treatment of cSLE limited, the Single Hub and Access point for pediatric Rheumatology in Europe (SHARE) initiative was launched, in part, to facilitate optimization with respect to the management of this rare disorder. Findings from the SHARE recommendations were published in Annals of the Rheumatic Diseases.
An expert panel of 16 pediatric rheumatologists and pediatric nephrology representatives was convened. Evidence-based recommendations on cSLE were generated using standard operating procedures of the European League Against Rheumatism. A total of 25 recommendations on key approaches to the diagnosis and treatment of cSLE were identified, including 11 on diagnosis, 9 on disease monitoring, and 5 on general treatment. The topics evaluated included the following: (1) appropriate use of SLE classification criteria, disease activity, and damage indices; (2) adequate assessment of autoantibody profiles; (3) secondary macrophage activation syndrome; (4) use of hydroxychloroquine and corticosteroid-sparing regimens; and (5) importance of addressing poor adherence.
Overall, 10 recommendations were accepted with respect to general diagnostic strategies and treatment indications of neuropsychiatric (NP) manifestations of cSLE, which occur often.
A literature search was undertaken to identify the most relevant articles on the diagnosis, treatment, and management of patients with cSLE. A total of 133 articles were selected for inclusion in the analysis. Of these, 51 articles covered the diagnosis and management of cSLE in general, 27 pertained to the NP manifestations of cSLE, and 55 were related to lupus nephritis (the latter is discussed in a separate publication).
No validated diagnostic criteria for cSLE exist, and prompt, accurate diagnosis in a specialist center is critical for timely initiation of appropriate therapy, including multidisciplinary care. Similarities between adult-onset SLE and cSLE have enabled the use of established adult SLE American College of Rheumatology classification criteria in patients with cSLE.
The SHARE investigators emphasized the need for more research on diagnostic procedures, as well as treatment recommendations, in patients with cSLE. International collaboration is crucial, with large cohorts being difficult to assemble.
Groot N, Graeff N, Avcin T, et al. European evidence-based recommendations for diagnosis and treatment of childhood-onset systemic lupus erythematosus: the SHARE initiative. [published online June 19, 2017]. Ann Rheum Dis. doi:10.1136/annrheumdis-2016- 210960.