C4 glomerulopathy overview

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Complement Mediated Glomerular Disorders Main Page
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Historical Perspective
Classification
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Differentiating C4 glomerulopathy from other Diseases
Epidemiology and Demographics
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Diagnosis
History and Symptoms
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Ali Poyan Mehr, M.D. [2]; Associate Editor(s)-in-Chief: Olufunmilola Olubukola M.D.[3]

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.

Historical Perspective

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

Classification

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

Pathophysiology

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

Differential Diagnosis

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.

Causes

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

Risk Factors

There are no established risk factors for C4 glomerulopathy.

Screening

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

Epidemiology and Demographics

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

Natural History, Complications, and Prognosis

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

Diagnosis

History and Symptoms

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

Physical Examination

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

Laboratory Findings

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

Imaging Studies

There are no other imaging findings associated with C4 glomerulopathy.

Other Diagnostic Studies

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.

Treatment

Medical Therapy

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

Surgery

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

Prevention

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