• Clinical science

IgA nephropathy (Berger's disease)

Abstract

IgA nephropathy (Berger disease) is the most common primary glomerulonephritis worldwide. It most frequently affects males in the second to third decades of life. Clinical manifestations are usually triggered by upper respiratory tract or gastrointestinal infections and include gross hematuria and flank pain. In some cases, it may present as rapidly progressive glomerulonephritis (RPGN). Urinalysis of asymptomatic patients often shows persistent microhematuria and minor proteinuria, while more severe cases may present with recurrent episodes of nephritic syndrome. A kidney biopsy is necessary to make a definitive diagnosis. Treatment consists of measures to slow the progression of the disease (e.g., ACE-inhibitors), as well as immunosuppressive therapy in more severe cases. Even with the appropriate treatment, up to 50% of the patients progress to end-stage renal disease within 20–25 years.

Epidemiology

  • IgA nephropathy is the most common primary glomerulonephritis
  • Peak incidence: second to third decade of life
  • Sex: > (2:1)
  • Ethnicity: more common in Asian and white populations

Epidemiological data refers to the US, unless otherwise specified.

Pathophysiology

Clinical features

The course of disease is highly variable. It can present in the following forms:

  • Asymptomatic
  • Recurring episodes of gross hematuria, flank pain, low-grade fever, and/or nephritic syndrome (including hypertension), usually during or immediately following a respiratory or gastrointestinal infection
  • Can progress to RPGN and/or nephrotic syndrome (< 10% of patients)
  • Up to 50% of patients progress to end-stage renal disease within 20–25 years.
    • Risk factors associated with a worse outcome: persistent gross hematuria, hypertension, proteinuria (> 1g/24hours), males < 30 years of age, elevated serum creatinine, histopathologic signs of interstitial fibrosis

Diagnostics

Diagnosis is based on clinical presentation and laboratory results. In some cases, renal biopsy may be indicated to confirm the diagnosis.

Differential diagnoses

  • Poststreptococcal glomerulonephritis
    • Associated with low complement levels
    • Occurs 10–20 days following an infection
  • Henoch-Schönlein purpura (HSP)
    • Also assoicated with abdominal symptoms, palpable purpura, arthritis
    • Patients are usually < 10 years of age

The differential diagnoses listed here are not exhaustive.

Treatment

last updated 09/03/2018
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