New Childhood-Onset Lupus Guidelines


Best practices for the diagnosis and management of childhood rheumatologic disease is the goal of SHARE.

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.

The study

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).

Exertional intolerance in patients with cSLE should be investigated.

In patients with cSLE, unexplained fever should trigger a search for infection and macrophage activation syndrome (MAS).

When MAS is suspected, a bone marrow aspirate should be considered.

Monitoring and management

Active disease should be monitored regularly.

Clinical evaluation usually should occur every 2 to 4 weeks for the first 2 to 4 months after diagnosis or a flare and then according to the response to treatment.

Children who are receiving systemic corticosteroids should be checked regularly for linear growth.

For all children with cSLE, disease activity should be assessed regularly and disease damage assessed yearly, each with a standardized measure.

All patients with cSLE should have access to an ophthalmologist.

Annual eye screening should be considered for patients who are receiving hydroxychloroquine.

Sun protection may be beneficial in patients with skin manifestations and should be considered.

In lupus, a coordinated transition program including pediatric and adult specialists is crucial.

Treatment recommendations

All children with lupus should receive hydroxychloroquine routinely.

In all decisions about treatment change or modification, compliance should be checked actively.

When tapering the prednisone dose is not possible, a disease modifying antirheumatic drug (DMARD) should be added.

For mild or moderate hematological involvement: When hemolysis is present and the hemoglobin level is lower than normal, a DMARD should be added.

If rituximab is required, the recommended dose is 750 mg/m2/dose (up to a maximum of 1 g) at day 1 and day 15 or 375 mg/m2/dose once a week for 4 doses.


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

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