Objectives: Class IV lupus nephritis (LN) is one of the most frequent and severe types of involvement in pediatric systemic lupus erythematosus. Gold standard treatment consists of intravenous (i.v.) Cyclophosphamide (CYC) associated with corticosteroids. Recent studies in adults have shown similar efficacy of oral Mycophenolate Mofetil (MMF) with fewer adverse events. Our aim was to compare the efficacy and tolerance of CYC and MMF as induction therapy in children with class IV LN.
Methods: We conducted a retrospective study of children diagnosed with class IV LN who started oral MMF or i.v. CYC treatment at Necker Enfants Malades Hospital (Paris, France).
Results: The study included 33 patients, 17 treated with oral MMF (51%) and 16 with i.v. CYC (48%). The characteristics at treatment induction did not significantly differ between the two groups except for the neurological involvement, that was only present in the CYC group. Complete remission was obtained in 9/17 (53%) children treated with MMF versus 10/16 (71%) treated with CYC (p = 0.46). Relapse was observed in 59% of patients receiving MMF versus 50% receiving CYC (p = 0.87), after a median of 3.4 years and 4.7 years after the beginning of treatment, respectively (p = 0.41). During the 6.5 years of follow-up, we observed no significant difference regarding the number of treatment-related adverse events between the two groups (p = 0.48).
Conclusion: We report similar efficacy and tolerance of MMF or CYC as induction therapy of class IV LN in children. However, the long-term adverse events such as infertility could not be systematically evaluated in this retrospective pediatric study. Overall, however, considering the long-term safety profile reported in the literature, we suggest that MMF may be used as first-line induction therapy in LN.
Keywords: Cyclophosphamide; Lupus erythematosus; Mycophenolate Mofetil; Nephritis; Pediatric; Systemic; Treatment outcome.
© 2022. The Author(s) under exclusive licence to Italian Society of Nephrology.