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Flashcards in Nephritic Syndromes Deck (14):

Nephritic syndrome

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


Causes of nephritic syndrome

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


Wegener's granulomatosis with polyangitis (GPA)

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


Treatment of Wegener's

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


Lupus nephritis

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


Treatments of lupus nephritis

Immunosuppressants--> steroids +- tacrolimus, mycophenolate or cyclophosphamide


Typical presentation of lupus nephritis

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


IgA (Berger's) nephropathy AKA synpharynigitic GN

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


Alport's syndrome

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


Goodpasture's syndrome

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%)


Treatment of IgA nephropathy



Treatment for Goodpasture's syndrome

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


Post-infectious GN

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


Treatment of post-infectious GN

Mainly supportive and treating any serious complications