Chapter: Medicine and surgery: Genitourinary system

IgA nephropathy - Glomerular disease

IgA nephropathy (also called mesangial IgA disease or Berger’s disease) is a form of focal segmental glomerulonephritis usually presenting as recurrent haematuria associated with upper respiratory tract infections. - Definition, Incidence, Aetiology, Pathophysiology, Clinical features, Complications, Investigations, Management, Prognosis.

IgA nephropathy

 

Definition

 

IgA nephropathy (also called mesangial IgA disease or Berger’s disease) is a form of focal segmental glomerulonephritis usually presenting as recurrent haematuria associated with upper respiratory tract infections.

 

Incidence

 

The commonest glomerulonephritis in the developed world.

 

Aetiology/pathophysiology

 

Most cases are idiopathic. Associated with Henoch– Schonlein¨ Purpura, cirrhosis, coeliac disease and dermatitis herpetiformis. There is a weak association with HLA-DR4. It is thought that the disease is related to abnormal IgA homeostasis.

 

Clinical features

 

One third of patients present with recurrent macroscopic haematuria during or after upper respiratory tract infections, one third have persistent microscopic haematuria and/or persistent mild proteinuria. The others may present with hypertension, or nephrotic or nephritic syndrome.

 

Macroscopy/microscopy

 

Typically there is mild, focal mesangial proliferation but histology is variable. Immunofluorescence shows extensive IgA and C3 deposits in the mesangium.

 

Complications

 

Acute renal failure can occur with an episode of gross haematuria. It has a good prognosis with renal func tion returning to baseline. Chronic renal failure may also occur.

 

Investigations

 

Serum IgA levels are high in 50%. Renal biopsy is not always required. For those with recurrent macroscopic or microscopic haematuria but no evidence of significant proteinuria the course is usually benign and the diagnosis is made clinically. Those with deterioration in renal function or with persistent significant proteinuria are biopsied, as they may benefit from more aggressive treatment, or may have another diagnosis.

 

Management

 

Best treatment is poorly defined.

 

·        Fish oil may slow progression.

 

·        Hypertension should be treated. ACE-inhibitors are used even in those without hypertension to lower intraglomerular pressure to reduce proteinuria and slow progression. Angiotensin-II receptor antagonists may also be used to maximise the benefit.

 

·        Corticosteroids are only used in selected patients, such as those with nephrotic syndrome, with little or no haematuria, few changes on renal biopsy, as these resemble minimal change disease in their course and response to treatment.

 

·        More aggressive immunosuppression may benefit some patients, such as those with crescentic disease.

 

Prognosis

 

Patients with only haematuria have a good prognosis. Proteinuria, renal impairment and histological evidence of scarring, tubular atrophy and capillary loop deposits signify a worse prognosis. Approximately a third develop renal impairment, and a third reach endstage renal failure (ESRF) and require renal replacement therapy. The disease recurs in approximately 50% of transplant kidneys.

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Medicine and surgery: Genitourinary system : IgA nephropathy - Glomerular disease |


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