Expert collaborative evidence-based consensus for childhood lupus nephritis diagnosis and treatment: unifying clinical approach in Saudi Arabia

Lupus nephritis (LN) affects approximately 60–70% of children with systemic lupus erythematosus (cSLE). Clinical practice for childhood lupus nephritis (cLN) management in Saudi Arabia varies significantly between pediatric rheumatologists and nephrologists. Unified, evidence-based national guidelines are needed to standardize care and improve outcomes. We aimed to develop the first evidence-based Saudi consensus recommendations for the diagnosis, monitoring, and treatment of childhood LN through collaboration between pediatric rheumatologists and nephrologists. A multidisciplinary working group developed evidence-based recommendations for cLN under the Saudi Rheumatology Society and Saudi Society of Nephrology and Transplantation. PICO questions guided a comprehensive literature search in Embase, PubMed, and Cochrane, extending the EULAR SHARE review (up to July 2013) to include studies through December 2022. Two independent reviewers screened, selected, and assessed studies, consulting adult LN guidelines when pediatric evidence was lacking. Recommendations were drafted and refined through a two-round e-Delphi process involving 20 national experts, with ≥70% agreement required for inclusion. Study quality was appraised using a modified Downs and Black checklist, and each statement was assigned a Level of Evidence and Grade of Recommendation per Oxford criteria. The consensus panel established comprehensive recommendations for early diagnosis, therapeutic goals, treatment by LN class, and supportive care in cLN. Early evaluation for renal involvement in all cSLE patients and timely biopsy were emphasized, using the 2018 ISN/RPS classification with added histopathologic criteria. Prognostic targets include ≥25% proteinuria reduction at 3 months, ≥50% at 6 months, and <0.7 g/day at 12 months. Class-specific therapy was outlined: corticosteroids (low–moderate dose) for Class I–II, with DMARDs if needed; high-dose corticosteroids + MMF as first-line for proliferative LN, cyclophosphamide as an alternative, and multi-target or biologic regimens for refractory disease. For pure membranous LN, corticosteroids + MMF are preferred, with other agents as second-line. Adjuvant recommendations include hydroxychloroquine, early ACEi/ARB, infection prevention, and ovarian protection during cyclophosphamide. These evidence-based, locally adapted guidelines aim to standardize and optimize the management of cLN in Saudi Arabia, improving early diagnosis, guiding treatment selection by LN class, and promoting supportive strategies to enhance renal outcomes, survival, and quality of life.