Chapter: Medicine and surgery: Genitourinary system

Nephritic syndrome - Glomerular disease

Nephritic syndrome is characterised by hypertension, haematuria and acute renal failure. - Definition, Incidence, Aetiology, Pathophysiology, Clinical features, Complications, Investigations, Management, Prognosis.

Nephritic syndrome

 

Definition

 

Nephritic syndrome is characterised by hypertension, haematuria and acute renal failure.

 

Aetiology

 

Acute diffuse proliferative, e.g. post-streptococcal glomerulonephritis.

Goodpasture’s disease (anti-Glomerular Basement Membrane (GBM) disease).

 

Systemic diseases such as systemic lupus erythematosus (SLE), Henoch–Schonlein¨ purpura (HSP), vasculitis (Wegener’s granulomatosis, polyarteritis), malignant hypertension and haemolytic-uraemic syndrome (HUS).

 

Pathophysiology

 

Proliferation of endothelial cells and mesangial cells, or vasculitis, leads to occlusion of the capillary lumen, reduced blood flow, oliguria and acute renal failure. Damaged glomeruli leak red blood cells causing microscopic (occasionally macroscopic) haematuria. The low GFR also leads to activation of the renin–angiotensin system, exacerbating hypertension. Proteinuria may also be present.

 

·        Focal nephritis: When less than 50% of the glomeruli are affected this usually manifests as haematuria with or without minor proteinuria. The majority of glomeruli are unaffected so renal failure is minimal or absent.

 

·        Diffuse nephritis: If more than 50% of the glomeruli are affected then oliguria and acute renal failure results. If diffuse nephritis is severe (with crescents in most of the glomeruli) then rapidly progressive glomerulonephritis results.

 

Clinical features

 

The full nephritic syndrome includes haematuria, proteinuria, hypertension and oedema (from salt and water retention), oliguria and uraemia, but the features are variably present. Often, the patient is unwell and there may be features of the underlying illness, for example haemoptysis with Goodpasture’s syndrome, rash, joint pains, a preceding infection, e.g. diarrhoea or a sore throat. Headache and loin pains are common nonspecific features. Salt and water retention can lead to hypertensive encephalopathy and pulmonary oedema.

 

Macroscopy/microscopy

 

The kidneys are oedematous, swollen, with scattered petechiae sometimes seen. The microscopic appearances are described in greater detail in section on Glomerular Disease and under each individual condition.

 

Investigations

 

·        Urine dipstick is positive for blood and may be positive for protein. Urine microscopy may demonstrate the dysmorphic red blood cells and red cell casts, which indicate a nephritic urine.

 

·        Patients should be investigated as for acute renal failure.

 

·        Renal biopsy is required in most cases to help identify the underlying cause, deomonstrate the pattern of disease, indicate prognosis and guide management.

 

·        FBC and peripheral blood film particularly to look for thrombocytopenia, and evidence of haemolysis.

 

·        More specific tests which are useful in nephritic syndrome include:

 

1.     ANA and anti-dsDNA (anti-double stranded DNA antibody is specific for SLE)

 

2.     ANCA - Anti-neutrophil cytoplasmic Antibody (found in vasculitides such as Wegener’s)

 

3.     Anti-GBM antibody

4.     Complement C3 and C4 – these are low in certain conditions.

 

5.     ASO and anti-DNAase – these are evidence of a pre-ceding streptococcal infection

 

6.     Serum cryoglobulins

 

 

Management

 

This is as for acute renal failure. Urgent treatment of the underlying cause is often needed to prevent permanent loss of renal function and early referral to a renal physician is necessary.

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Medicine and surgery: Genitourinary system : Nephritic syndrome - Glomerular disease |


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