Lupus nephritis (LN) occurs in 50%–60% of patients with childhood-onset systemic lupus erythematosus (cSLE), leading to significant morbidity. Timely recognition of renal involvement and appropriate treatment are essential to prevent renal damage. The Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) initiative aimed to generate diagnostic and management regimens for children and adolescents with rheumatic diseases including cSLE. Here, we provide evidence-based recommendations for diagnosis and treatment of childhood LN. Recommendations were developed using the European League Against Rheumatism standard operating procedures. A European-wide expert committee including paediatric nephrology representation formulated recommendations using a nominal group technique. Six recommendations regarding diagnosis and 20 recommendations covering treatment choices and goals were accepted, including each class of LN, described in the International Society of Nephrology/Renal Pathology Society 2003 classification system. Treatment goal should be complete renal response. Treatment of class I LN should mainly be guided by other symptoms. Class II LN should be treated initially with low-dose prednisone, only adding a disease-modifying antirheumatic drug after 3 months of persistent proteinuria or prednisone dependency. Induction treatment of class III/IV LN should be mycophenolate mofetil (MMF) or intravenous cyclophosphamide combined with corticosteroids; maintenance treatment should be MMF or azathioprine for at least 3 years. In pure class V LN, MMF with low-dose prednisone can be used as induction and MMF as maintenance treatment. The SHARE recommendations for diagnosis and treatment of LN have been generated to support uniform and high-quality care for all children with SLE.
- disease activity
- lupus nephritis
- systemic lupus erythematosus
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Handling editor Tore K Kvien
Contributors SK and MB are senior authors. NW and SV designed the SHARE initiative. NG and NdG performed the systematic literature review, supervised by MB and SK. Validity assessment of selected papers was done by MWB, SK, TA, AR, IKP, BBM, CP. Recommendations were formulated by NG, MB and SK. The expert committee consisted of TA, BBM, PB, PD, IKP, PL, LM, SO, CP, AR, AvR, YU, NW, SK, MWB, SM, GK; they completed the online surveys and/or participated in the subsequent consensus meetings. NG, NdG, SK and MWB prepared the consensus meetings, and NG and NdG chaired the meetings and took minutes. AR and BF facilitated the consensus procedure using nominal group technique. NG, SK and MWB wrote the manuscript, with contribution and approval of all coauthors.
Funding EAHC; grant number 2011 1202
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.