Diagnosis and Treatment of Childhood Lupus Nephritis: SHARE Recommendations

The development of lupus nephritis (LN), which is reported in 50% to 60% of individuals with childhood-onset systemic lupus erythematosus (cSLE), is associated with significant morbidity and mortality. To prevent renal damage, timely and accurate recognition of renal involvement, together with appropriate treatment choices, will help improve clinical outcomes among patients.

The Single Hub and Access Point for Pediatric Rheumatology in Europe (SHARE) initiative, launched in 2012, was designed to optimize and generate diagnostic and treatment regimens for children and adolescents with such rheumatic diseases as cSLE, in which uniform and high-quality care is essential. Findings from the SHARE recommendations were published in the Annals of the Rheumatic Diseases.

A systemic literature search was conducted to identify the most relevant articles on the diagnosis, treatment, and management of patients with cSLE. A total of 55 articles were selected for inclusion in the analysis.

In this portion of the SHARE initiative, six recommendations concerning diagnosis, and 20 recommendations regarding treatment choices and goals, were accepted for investigation. Recommendations were developed via use of standard operating procedures of the European League Against Rheumatism. Patients with each of the 5 classes of LN, as described in the International Society of Nephrology/Renal Pathology 2003 classification system, were examined.

The goal of treatment for patients with LN should be complete renal response. In those with class I LN, treatment should be guided largely by the appearance of additional symptoms. Patients with class II LN should receive low-dose prednisone as initial treatment, with a disease-modifying antirheumatic drug added only after 3 months of continued proteinuria or steroid dependency. In persons with class III/IV LN, induction therapy should include the use of mycophenolate mofetil (MMF) or intravenous cyclophosphamide in combination with corticosteroids; maintenance treatment should include MMF or azathioprine for ≥3 years. In patients with pure class V LN, MMF plus low-dose prednisone should be used as induction therapy, and MMF as maintenance therapy.

The SHARE guidelines are the first of their kind to focus specifically on evidence in cSLE for the diagnosis and treatment of all classes of LN via use of a systematic literature search. Because data on cSLE were limited, the need for new, high-quality studies is warranted.

Reference

Groot N, de Graeff N, Marks SD, et al. European evidence-based recommendations for the diagnosis and treatment of childhood-onset lupus nephritis: the SHARE initiative [published online September 6, 2017]. Ann Rheum Dis. doi: 10.1136/annrheumdis-2017- 211898