Clinical Presentation and Treatment of Childhood‐Onset Lupus Nephritis

Abstract

Introduction: Lupus nephritis (LN) in childhood usually presents after the age of 10 years and is more common in female children. Despite the availability of effective immunosuppressive therapies, treatment response in childhood‐onset LN remains suboptimal, with 50% to 78% patients in full remission at 24 months. Improvement in survivals have also plateaued in past decades, and up to 9% and 14% of children with childhood‐onset LN died or developed end‐stage kidney disease during the course of illness, respectively. In our study, we aim to evaluate clinical presentation of LN with childhood‐onset, the treatment regimen instituted and the transition to adulthood. Methods: Retrospective study of patients with LN followed by pediatric nephrology with subsequent transition to adult nephrology since 1995 to 2020. Results: Fourteen patients (female:78.6%) with a median age of 26.3 ± 5.2 years (range 21‐44) were included. The median age of LN diagnosis was 13.6 years. The most common manifestation was proteinuria (71.4%). Of non‐renal manifestations, polyarthritis (100.0%) and rash (57.1%) were the most common. All patients underwent kidney biopsy with a majority of diffuse LN (class IV) (n=11, 78.6%). Induction therapy consisted of corticosteroids combined with other immunosuppressants, primarily cyclophosphamide (CYC) (n=5, 35.3%) or mycophenolate (MMF) (n=9, 64.7%). Maintenance therapy consisted of corticosteroids combined with primarily CYC (n=6, 42.9%) with posterior transition to MMF, or MMF (n=5, 35.7%) or azathioprine (n=3, 21.4%). A complete remission occurred in all patients. At the end of follow‐up, 11 patients (78.6%) were with MMF and oral corticosteroids and 3 patients were with oral corticosteroids. None of these patients developed end‐stage kidney disease or died. Conclusion: The diagnosis of LN in pediatric age has its particularities and constitutes a challenge for the nephrology community. Biological therapy currently shows promise in improving the prognosis of these patients.