Presentation and outcome of pediatric lupus nephritis from a large single centre contemporary cohort in Eastern India

Abstract

Background We present clinical, biochemical, and histopathological characteristics and treatment outcomes of biopsy proven childhood lupus nephritis (LN) from a low/middle income setting treated in the current era of increased use of Mycophenolate Mofetil (MMF) and biologics. Methods Retrospective observational study of children (1–18 years) with biopsy proven LN treated from 01.01.2010 to 31.01.2020. Results 60 children met our inclusion criteria (80%, n = 48 were females). The median age at diagnosis was 11 (IQR: 9–12) years. The most common extra-renal manifestation was mucocutaneous ( n = 54, 90%) and the most common kidney manifestation was edema ( n = 50, 83.3%). The median 24-h urinary protein excretion was 1117.8 (IQR: 795.4–1941.7) mg/m2/day with 67% ( n = 40) having nephrotic range proteinuria (>1000 mg/m2/day). 75% ( n = 45) children had eGFR <90 mL/min/1.73 m2 (median eGFR = 71; IQR: 56–90 mL/min/1.73 m2). Anti-Nuclear Antibody was positive in all, both complement three and four were low in 82% ( n = 49) and anti-double stranded DNA antibodies were positive in 63% ( n = 38). 85% ( n = 51) had proliferative LN with majority being class IV (57%, n = 34). All children received steroids for induction therapy. MMF was given as the sole induction agent in 48% ( n = 29) and cyclophosphamide in 27% ( n = 16). Rituximab was added in 17% ( n = 10) as a rescue agent. Median follow up duration was 50 (IQR: 28–82) months. Six children (10%) died as a result of serious infections and none of them had shown complete response (CR). Out of the 52 children who had a follow up duration of at least 2 years, CR was achieved in 46 children (88%) and partial response (PR) or no response (NR) in three children (6%) each. Although children who were in CR/PR at last follow up had lower proteinuria, higher eGFR, and lower histopathology activity index at onset; low numbers in the NR group precluded us from subjecting them to any statistical correlation tests. 36% ( n = 22) of children developed 36 episodes of renal flares with overall incidence of 0.14/person-year. Conclusion Our study on a contemporary cohort of childhood LN highlights the importance of achieving CR and its feasibility.