IgA nephropathy

Not to be confused with Buerger's disease.

Berger's disease (IgA nephropathy)
Synonyms	IgA nephritis
Pronounce
Field	Nephrology Rheumatology Oncology
Symptoms	Hematuria, proteinuria, hypertension, edema
Complications	Chronic kidney disease, End-stage kidney disease, Nephrotic syndrome
Onset	Usually young adulthood
Duration	Lifelong
Types	Aggressive Berger's disease, Typical IgAN
Causes	Abnormal IgA deposits in glomeruli
Risks	Family history, recurrent respiratory or gastrointestinal infections
Diagnosis	Kidney biopsy, Urinalysis
Differential diagnosis	Henoch–Schönlein purpura, Membranoproliferative glomerulonephritis, Lupus nephritis
Prevention	Early management of infections, regular monitoring
Treatment	ACE inhibitors, Corticosteroids, immunosuppressive therapy
Medication	Lisinopril, Prednisone
Prognosis	Variable, potential progression to chronic kidney disease
Frequency	Common, most frequent cause of glomerulonephritis globally
Deaths	Rare, usually from complications

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Doctor Jean Berger

IgA nephropathy (IgAN), also known as Berger's disease ( ), or synpharyngitic glomerulonephritis, is a chronic kidney disease involving the immune system. It is a form of glomerulonephritis, characterized by inflammation of the glomeruli due to abnormal deposition of the antibody IgA.

Aggressive Berger's disease, a rarer and more severe variant, can affect additional organs beyond the kidneys, including the liver, skin, and heart.

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Illustration of IgA deposition in the glomeruli of kidneys

IgA nephropathy is the most common form of glomerulonephritis worldwide, yet the aggressive form of Berger’s disease is classified as a rare disease by the NORD.

Signs and Symptoms

Common clinical presentations include:

• Hematuria (blood in the urine)
• Proteinuria (protein in the urine)
• Hypertension (high blood pressure)
• Edema (swelling due to fluid retention)

Causes

IgA nephropathy is primarily caused by the accumulation of abnormal IgA immune complexes in the glomeruli, leading to inflammation and impaired kidney function.

Risk Factors

Risk factors include:

• Family history of kidney disease
• Recurrent respiratory infections
• Gastrointestinal infections

Diagnosis

Diagnosis typically involves:

• Kidney biopsy showing IgA deposition
• Urinalysis indicating blood and protein

Differential diagnoses to consider include:

• Henoch–Schönlein purpura
• Membranoproliferative glomerulonephritis
• Lupus nephritis

Treatment

Management strategies focus on slowing disease progression and controlling symptoms, including:

• Blood pressure control using ACE inhibitors (e.g., Lisinopril)
• Anti-inflammatory therapy with corticosteroids (e.g., Prednisone)
• Immunosuppressive therapies in severe cases

Prognosis

The prognosis for individuals with IgA nephropathy is variable. While many patients experience a slowly progressive course, some may advance to chronic kidney disease or even end-stage kidney disease. Early diagnosis and appropriate treatment can significantly improve outcomes.

Epidemiology

IgA nephropathy remains the most prevalent glomerular disease globally. However, severe aggressive Berger's disease remains rare and is considered a significant clinical challenge.

Prevention

Preventive strategies involve early detection, management of respiratory and gastrointestinal infections, and regular monitoring of kidney function to delay or prevent progression of the disease.