Nephritic Syndrome

This refers to a group of signs and symptoms secondary to glomerular disease.

It reflects inflammatory damage to the glomerulus which increases the permeability to proteins and blood causing proteinuria and haematuria.

Left untreated, it can progress to end-stage renal failure.

Symptoms

Haematuria

Less proteinuria (< 3.5 g/24 h)

Hypertension and blurred vision

Oliguria – low urine output

Azotemia – high urea/creatinine

Can progress onto renal failure

Key tests

Kidney biopsy 

There are many specific conditions which give rise to nephritic syndrome, which have specific treatments:

IgA nephropathy

This is most common cause of nephritic syndrome, called Berger’s disease

It occurs due to IgA immune complexes being deposited in the mesangium of the glomerulus.

It is usually seen in young males and is associated with alcohol and coeliac disease.

Symptoms

The classic presentation is macroscopic haematuria, usually 1–2 days after URTI.

The condition carries a good prognosis and is unlikely to lead to CKD.

Biopsy findings

Shows IgA deposition in glomerulus

Management

Corticosteroids

Henoch-Schonlein purpura (HSP)

This is similar to IgA nephropathy, but in addition, there is also IgA deposition in other parts of the body such as the skin and joints (IgA vasculitis)

It leads to symptoms of IgA nephropathy, as well as arthritis and a typical purpuric rash on the extensor surfaces.

It is usually a self-limiting illness, but steroids can also be given.

Symptoms

IgA Nephropathy

Arthritis

Rash on extensor surfaces

Management

Steroids

Analgesia for joint pain (it is usually self-limiting)

Post-Streptococcal Glomerulonephritis

This is a complication which arises 1–2 weeks after infection with group A beta-haemolytic streptococcal bacterial infection.

Antibodies produced against the bacteria lead to immune complex formation which then end up getting deposited in the glomerulus, causing inflammation.

It usually self-resolves in children, but some adults can get a rapidly progressing glomerulonephritis, causing haematuria as well as other signs of nephritic syndrome

Symptoms

Haematuria

Other signs of full nephritic syndrome (hypertension + proteinuria/oedema)

Key tests

Blood tests – check for anti-streptolysin antibodies or DNase antibodies

Biopsy – endothelial neutrophil proliferation giving granular “starry sky” appearance

Management

Antibiotics to clear bacteria

Supportive treatment for renal dysfunction

Anti-GBM disease

This is a disease which used to be called Goodpasture’s disease, which occurs due to autoantibodies against type IV collagen.

This causes damage to the exchange surfaces which rely on the basement membranes, notably the lungs and kidney.

It leads to a rapidly progressive nephritic syndrome, as well as lung symptoms, including shortness of breath and pulmonary haemorrhage leading to haemoptysis.

Key tests

Blood tests – shows the present of anti-GBM antibodies

Kidney biopsy – shows linear IgG deposits in membrane on renal biopsy. These lead to crescent formation, which is the histopathologic hallmark of anti-GBM disease

Management

Plasmapheresis can be used remove antibodies form blood

Immunosuppression is the mainstay of treatment

Alport syndrome

This is a genetic X-linked dominant condition the protein chain of type IV collagen

This causes the glomerular basement membrane to be excessively thin, meaning it can easily tear.

Symptoms

Presents in childhood with microscopic haematuria, progressing to renal failure

Bilateral sensorineural hearing loss

Retinitis pigmentosa and blindness

Key tests

Renal biopsy show splitting of lamina densa

Management

Definitive management is a kidney transplant.

But some transplant patients can develop anti-GBM antibodies which can cause graft dysfunction.