C4 glomerulopathy overview

Complement system activation includes three pathways – the classical pathway, alternate pathway, and lectin pathway. C4d is a split product of C4 glomerulopathy, which itself results by overactivation of the lectin pathway. C4 glomerulopathy is further categorized into dense deposit disease and C4 glomerulonephritis.

There is limited information about the historical perspective of C4 glomerulopathy.

C4 glomerulopathy may be classified based on complement deposition into dense deposit disease and C4 glomerulonephritis.

It is thought that C4 glomerulopathy is the result of the activation of immune complex glomerulonephritis or lectin pathway of the complement.

C4 glomerulopathy must be differentiated from other diseases that cause proteinuria, hematuria, and peripheral edema, such as IgA nephropathy, membranous nephropathy, focal segmental glomerulus /minimal change disease, membranoproliferative glomerulonephritis, and lupus nephritis.

C4 glomerulopathy may be caused by mutation of complement factor,anti-factor H, anti-factor B, or C4 nephritic factor.

There are no established risk factors for C4 glomerulopathy.

There is insufficient information to recommend routine screening for C4 glomerulopathy.

There is not much information available about the incidence and prevalence.

If left untreated, C4 glomerulopathy may progress to develop renal failure.

The most common symptoms of C4 glomerulopathy include hematuria, proteinuria, and hypertension.

Physical examination of patients with C4 glomerulopathy is usually remarkable for high blood pressure, peripheral edema, pale skin, and periorbital edema.

Laboratory findings consistent with the diagnosis of C4 glomerulopathy include abnormal urinalysis, abnormal BUN: Creatinine ratio, and abnormal serum C3 and C4 levels

There are no other imaging findings associated with C4 glomerulopathy.

A kidney biopsy may be helpful in the diagnosis of C4 glomerulopathy. Findings diagnostic of C4 glomerulopathy include glomerular capillary walls thickening, large subendothelial osmiophilic dense deposits, and ribbonlike material on the glomerular basement membrane.

The mainstay of treatment for C4 glomerulopathy is antihypertensive and lipid-lowering drugs.

Surgical intervention is not recommended for the management of C4 glomerulopathy.

There are no established measures for the primary or secondary prevention of C4 glomerulopathy.

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