Acute nephritic syndrome with dense deposit disease

This code describes a specific form of acute nephritic syndrome characterized by the presence of dense deposit disease (DDD), also known as C3 glomerulopathy. It signifies an abrupt onset of kidney inflammation, often presenting with hematuria, proteinuria, hypertension, and impaired renal function, directly linked to abnormal complement pathway activation and dense deposits in the glomerular basement membrane.

Use this code when documentation explicitly states a diagnosis of acute nephritic syndrome and confirms the underlying etiology as dense deposit disease. This typically follows renal biopsy findings demonstrating characteristic electron-dense deposits within the glomerular basement membrane, along with clinical signs of acute nephritis.

• Ensure documentation clearly distinguishes dense deposit disease from other forms of C3 glomerulopathy or membranoproliferative glomerulonephritis.
• Do not use this code for chronic nephritic syndrome or if the dense deposit disease is not explicitly stated as the cause of the acute nephritic presentation.
• Consider sequencing this code as a primary diagnosis when it is the main reason for the encounter, especially during an acute exacerbation.