A case of C3 glomerulopathy with nephritis-associated plasmin receptor positivity without a history of streptococcal infection

A 17-year-old lady was diagnosed with proteinuria and microscopic hematuria within the prior 2 years to this presentation. Further tests revealed nephrotic syndrome with hematuria and hypocomplementemia. Renal histopathology showed membranoproliferative glomerulonephritis. Immunostaining and electron microscopy, suggested C3 glomerulonephritis (C3GN). Nephritis-associated plasmin receptor (NAPlr) and plasmin activity (PA), markers of infection-related glomerulonephritis, were identified in the aforementioned pathology specimens. There are several reports suggesting a causal relationship between group A streptococcal infection and C3 glomerulopathy (C3G). There are many intractable cases, whereas some have responded to immunosuppressive therapy, as did our case. However, there is currently no established gold standard treatment for this disease. We herein report a case of C3G with glomerular positive NAPlr and PA despite the absence of a streptococcal infection. Accumulation of cases such as this may help advance treatment by clarifying the etiology and pathogenic mechanism of C3G and future prognosis.

Automated summary

This case report describes a 17-year-old female diagnosed with C3 glomerulonephritis (C3GN) despite the absence of streptococcal infection. Immunotherapy showed some improvement in the patient's condition, but there is currently no established gold standard treatment for this disease. Accumulation of similar cases may help advance treatment strategies by clarifying the etiology and pathogenic mechanism of C3GN for future prognosis.