Nephritic Syndromes

Question 0

Causes of nephritic syndrome

Answer 0

Wegener’s granulomatosis with polyangitis
Lupus nephritis
IgA nephropathy
Alport’s syndrome OR Goodpasture’s syndrome
Post infectious glomerulonephritis (strep or anything)

Question 1

Nephritic syndrome

Answer 1

Characterised by haematuria, oliguria or AKI and mild-moderate hypertension. They may also have some oedema
Caused by glomerular pores large enough to let RBCs through
Urine microscopy is urgent, with a different spin protocol than for bacteria–> look for dymorphic red cells or red cell casts

Question 2

Wegener’s granulomatosis with polyangitis (GPA)

Answer 2

Multi-system, ANCA +ve granulomatous vasculitis –> nephritic syndrome in kidney, on biopsy a rapidly progressive necrotising GN+small number of immune complexes||ENT –> sinusitis, rhinorhoea, bloody discharge, nasal cartilage destruction, otitis media etc||Pulmonary –> cough, dyspnoea, haemoptysis and pleurisy. Infiltrates and nodules on CXR

Question 3

Treatment of Wegener’s

Answer 3

General immunosuppression –> steroids and cyclophosphamide (risk of haemorrhagic cystitis)
Rituximab –> monoclonal anti-Bcell antibody

Question 4

Lupus nephritis

Answer 4

A rapidly progressing GN which occurs in patients with lupus
Can present in 6 different forms, may be asymptomatic or as AKI
Necrotising GN with similar biopsy findings to GPA+lots of immune complexes–> must be ANA+, anti-Smith+ or anti-dsDNA for SLE diagnosis. May also have low C3/4 complement levels

Question 5

Treatments of lupus nephritis

Answer 5

Immunosuppressants–> steroids +- tacrolimus, mycophenolate or cyclophosphamide

Question 6

Typical presentation of lupus nephritis

Answer 6

Systemic symptoms of lupus –> ulcers, fevers, arthralgia with a rapidly progressing GN and nephritic syndrome
Most commonly affects middle aged women, more African than white

Question 7

IgA (Berger’s) nephropathy AKA synpharynigitic GN

Answer 7

Usually presents with loin pain, episodic haematuria 1-2days after a URTI with a normal eGFR in the young –> commonest GN
Biopsy shows mesangial proliferative GN with IgA deposits detectable on immnofluoresence staining
25% proceed to ESRD

Question 8

Alport’s syndrome

Answer 8

Hereditary X-linked (in 80% of cases) nephritis
Asymptomatic and occasionally frank haematuria in young people
Associated with sensorineural deafness, lens and retinal abnormalities and a damaged basement membrane on EM
ESRD by their 30’s

Question 9

Goodpasture’s syndrome

Answer 9

Anti-glomerular basement membrane disease causing nephritic syndrome +- pulmonary haemorrhage in 50% of cases
RPGN on biopsy & anti-GBM antibodies serologically
Can occur in Alport’s syndrome after transplant as the host reacts to the normal basement membrane (3%)

Question 10

Treatment of IgA nephropathy

Answer 10

Steroids
+-ACEi
+-cyclophosphamide

Question 11

Treatment for Goodpasture’s syndrome

Answer 11

Plasmapheresis (the removal of blood from the body, treating it or removing some component and returning it)
+ steroids
+ cyclophosphamide

Question 12

Post-infectious GN

Answer 12

Usually occurs 2 weeks (Berger’s disease occurs 2days after infection) after a pharyngeal streptococcal infection in the young
Simple proteinuria»nephritic syndrome»rarely renal failure
Diagnosis is by +ve anti-DNAse B, throat culture and anti-streptolysin O titre. Associated with low C3

Question 13

Treatment of post-infectious GN

Answer 13

Mainly supportive and treating any serious complications