Through the Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) initiative, Noortje Groot and colleagues have presented recommendations for diagnosis and management of childhood-onset systemic lupus erythematosus (cSLE). The recommendations were generated using the European League Against Rheumatism standard operating procedure.
cSLE is classified as a rare disease. Its low incidence makes clinical research difficult, leading to few evidence-based guidelines and recommendations. Collaboration among countries was needed to gather enough evidence to produce the cSLE guidelines.
The primary objective was to provide guidance regarding the best practices for the diagnosis and management of childhood rheumatologic disease. The full recommendations appear in Annals of the Rheumatic Diseases.
A panel of 16 pediatric rheumatologists from across Europe was established to develop recommendations based on a systematic literature review. A total of 133 articles were included in the analysis. Following are highlights.
Recommendations for diagnosis
• Based on the current evidence (mainly in adults), the Systemic Lupus International Collaborating Clinics (SLICC) criteria can be used as classification criteria in cSLE.
• In the presence of a positive antinuclear antibody (ANA) test result combined with at least 2 clinical SLICC criteria, or in the presence of a positive ANA combined with at least 1 clinical and 1 immunological SLICC criterion, referral to a pediatric rheumatologist is warranted.
• When a diagnosis of cSLE is considered, anti-Sm, anti-RNP-a, anti-Ro/SS-A, and anti-La/SS-B should be included routinely.
• In a clinical context, when a patient is ANA positive, but anti-dsDNA and ENA negative, a diagnosis of cSLE still can be made.
• In patients with cSLE, hereditary complement deficiencies should be considered.
• A chest X-ray should be obtained for all patients with cSLE at diagnosis.
• All patients with cSLE should be screened for cardiac abnormalities using ECG and echocardiography at diagnosis.
• A pulmonary function test should be performed for patients with cSLE who have respiratory symptoms or signs (in the absence of acute infection).
This project was funded by the European Union.
Groot N, de Graeff N, AVcin T, et al. “European evidence-based recommendations for diagnosis and treatment of childhood-onset systemic lupus erythematosus: the SHARE initiative.” Ann Rheum Dis. Published Online First: July 13, 2017. doi:10.1136/ annrheumdis-2016-210960