Long-term renal outcomes of childhood-onset global and segmental diffuse proliferative lupus nephritis.

BACKGROUND

Data on global (IV-G) and segmental (IV-S) diffuse proliferative lupus nephritis (DPLN) in children are lacking.

METHODS

To determine the clinicopathology and prognosis of DPLN subclasses IV-G and IV-S, we analyzed the clinical, laboratory, and demographic data of 56 children aged <18 years diagnosed with DPLN [36 (64.3%) with IV-G; 20 (35.7%) with IV-S] between 2004 and 2013. Clinical endpoints were: (1) complete remission (CR), (2) chronic kidney disease [CKD; defined as estimated glomerular filtration rate (eGFR) of <60 mL/min/1.73 m(2) or end-stage renal disease (ESRD)], and (3) death.

RESULTS

Proteinuria and the activity index were higher in patients with IV-G (p < 0.05). Global endocapillary proliferation and leukocyte exudation were predominant in IV-G patients, whereas segmental endocapillary proliferation was predominant in patients with IV-S (p < 0.005). CR rates in IV-G and IV-S patients were 50 and 60%, respectively (p = 0.47). Renal survival rates, defined as an eGFR of ≥60 mL/min/1.73 m(2), were 93, 78, and 64% at 1, 5, and 10 years, respectively. Patient survival rates at 1, 5, and 10 years were 98, 96, and 91%, respectively. Patient and renal survival rates were similar in both subclasses.

CONCLUSIONS

Although patients with IV-G and IV-S displayed some clinical and histopathological disparities, renal outcomes were similar. The majority of children with DPLN reached adulthood but accrued significant renal damage. Treatment regimens which can slow the progression of CKD are needed.