CLINICAL CASE 60 Answers and Summary

WHICH OF THE FOLLOWING DISEASES IS NOT ASSOCIATED WITH A DYSREGULATION OF THE ALTERNATIVE PATHWAY OF COMPLEMENT?

Dense Deposit Disease 9%

C3 Glomerulonephritis 2%

Atypical HUS 4%

Minimal Change Disease 73%

Chronic FSGS 9%

Dysregulation of the alternative complement pathway can either result in a proliferative glomerular process or a thrombotic microangiopathy.  The 2 proliferative processes are C3 glomerulonephritis and dense deposit disease. The thrombotic microangiopathies can be from acute in terms of atypical HUS or chronic from FSGS. A chronic state of FSGS can lead to a vascular insult and cause chronic alteration of the atypical pathway. A recent case was described where FSGS occurred in association with CFH and C3 polymorphisms and dysregulation of complement pathway. MCD is usually not been associated with this; at least thus far.