Hereditary nephropathy, NEC with C3 glomerulonephritis

This code signifies a genetic kidney disorder, specifically a hereditary nephropathy, that is not classified elsewhere and is characterized by the presence of C3 glomerulonephritis. This condition involves inflammation of the kidney's filtering units (glomeruli) due to the deposition of complement component 3 (C3).

Use this code when documentation clearly indicates a hereditary nephropathy with C3 glomerulonephritis, and the specific type of hereditary nephropathy is not otherwise classified. This code is appropriate when genetic testing or family history confirms a hereditary component, and renal biopsy findings demonstrate C3 deposition in the glomeruli.

• Ensure documentation explicitly states "hereditary nephropathy" and "C3 glomerulonephritis."
• Do not use this code if the hereditary nephropathy is specifically identified as dense deposit disease; refer to the Excludes1 note.
• Review the patient's family history and genetic testing results to support the "hereditary" component of the diagnosis.