Introduction:
Systemic lupus erythematosus (SLE) is a chronic inflammatory condition that affects numerous organs, including the skin, joints, lungs, heart, kidneys, and nervous system. Its etiology is multifactorial, involving genetic and environmental factors. Although the disease is more frequently diagnosed in adulthood, up to 20% of cases occur in patients younger than 18 years, with a worldwide incidence between 0.3 and 0.9 cases per 100,000 children. Sixty percent of pediatric cases occur in patients over 10 years of age, with less than 5% occurring in children under 5 years of age. Kidney involvement by lupus nephritis (LN) is one of the most severe clinical manifestations observed in individuals with SLE. LN is more frequent and severe in pediatric patients and has been associated with higher morbidity and mortality rates. There is evidence that the prognosis of lupus nephritis in developing countries is worse than in developed countries. This review aims to describe the general and specific features of LN in children and adolescents, shedding light on the disease’s etiopathogenesis, clinical manifestations, histopathology, and treatment to refine clinical suspicion and aid in the treatment of future patients.
Methods:
This is a retrospective observational study of the clinicopathological presentation, management, and outcomes in 6 children with lupus nephritis in the Department of Nephrology at JSS Hospital, Mysuru, between January 1, 2022, and July 31, 2024. We included cases of biopsy-proven pediatric lupus nephritis with onset age <18 years to ascertain the long-term patient and kidney survival rates and the incidence of advanced chronic kidney disease (CKD) (estimated glomerular filtration rate [eGFR] <60 ml/min per 1.73 m²). Data on disease onset, biopsy, and therapy received were analyzed for short-term outcomes. Children were classified into those with complete remission (CR), partial remission (PR), active disease, no response, and progression to end-stage renal disease (ESRD).
Results:
All cases of lupus nephritis were seen in girls. The mean age of presentation was 11.5 years, with the youngest being a 5-year-old child suspected of having a genetic etiology of lupus. Hypertension was noted in 3 out of 6 children (50%), and proteinuria was present in 100% of patients at presentation. Class IV lupus was the most common (66.6%), followed by one case each of Class II+V and Class II LN. The mean activity index was 5/24, and the chronicity index was 1/12. The most common extra-renal manifestation was CNS involvement, with 50% of the children experiencing seizures. Two out of the six children were in complete remission after initial treatment, and four children showed a partial response after completing initial treatment.
Conclusions:
: Childhood lupus nephritis has a more aggressive course than adult-onset disease. Extra-renal manifestations and CNS involvement are common, with Class IV being the most common type of LN in this case series. In children under 7 years of age, a genetic etiology of lupus needs to be considered.
I have no potential conflict of interest to disclose.
I did not use generative AI and AI-assisted technologies in the writing process.