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Flashcards in Nephritic Syndromes Deck (14):
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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

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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)

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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

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Treatment of Wegener's

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

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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

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Treatments of lupus nephritis

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

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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

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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

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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

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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%)

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Treatment of IgA nephropathy

Steroids
+-ACEi
+-cyclophosphamide

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Treatment for Goodpasture's syndrome

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

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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

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Treatment of post-infectious GN

Mainly supportive and treating any serious complications