Role of complement system in patients with biopsy-proven IgG4 related kidney disease

Hypocomplementaemia has been frequently reported in IgG4 related kidney disease (IgG4-RKD). However, studies on the role of complement system in IgG4-RKD are lacking. A total of 40429 renal biopsies from January 2010 to January 2018 were re-examined in the present study, and 17 patients were confirmed to meet the criteria of IgG4-RKD. According to the serum C3 levels, they were divided into two groups: the low C3 group (C3<0.8g/L, n=7) and the normal C3 group (C3≥0.8g/L, n=10). Compared with the normal C3 level group, the patients in the low C3 level group had lower serum C4 concentrations (P=.025), higher serum IgG4 concentrations (P=.003), higher positive rates in rheumatoid factor (P=.033), more severe storiform fibrosis (P=.007) at diagnosis, and higher blood urea nitrogen levels at the latest test (P=.04).

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