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Flashcards in Renal medicine Deck (41)
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Granulomatosis with polyangitis biopsy findings:

Pauci-immune crescenteric glomerulonephritis (ANCA positive)


Anti-GBM disease biopsy findings:

Crescenteric glomerulonephritis with linear immunoglobulin G (IgG) deposition


Who presents with focal segmental glomerulosclerosis?

Diabetes, those on antiretrovirals, SLE, sickle cell disease


Membranous glomerulonephritis associations

Associated with SLE and polyarteritis nodosa


Minimal change association and treatment 

Seen in the young


High dose pred. If contraindicated can think about tacrolimus + low dose steroids or rituximab for frequently relapsing disease


Difference nephrotic and nephritic


What types of glomerulonephritides are there?


General treatment approach to glomerulonephritis?


Non-proliferative vs proliferative 

Non-proliferative glomerulonephritis is characterised by a lack of glomerular cell proliferation and typically presents with nephrotic syndrome.


Proliferative glomerulonephritis is characterised by increased numbers of cells in the glomerulus

It typically presents with nephritic syndrome


Non proliferative: Minimal change glomerulonephritis

Minimal change glomerulonephritis presents with nephrotic syndrome.

It accounts for 80% of all nephrotic syndrome in children and 20% of nephrotic syndrome in adults.

The underlying cause of minimal change glomerulonephritis is unknown.

Typical investigation findings

Urinalysis – proteinuria

Light microscopy – no visible abnormalities

Immunofluorescence – no immunoglobulins or complement deposits bound to the kidney tissue ¹

Electron microscopy – diffuse loss of visceral epithelial cells’ foot processes (i.e. podocyte effacement), vacuolation, and growth of microvilli on the visceral epithelial cells, allowing for excess protein loss in the urine ²


Supportive care – nutritional support, salt and fluid restriction

Corticosteroids (first-line) – used in both adults and children (more data for efficacy in children) ¹

Other immunosuppressants (second-line – effectiveness unclear) – e.g. calcineurin inhibitor, mycophenolate mofetil, rituximab ¹


Non-proliferative: Focal segmental glomerulosclerosis (FSGS)

Typically presents with nephrotic syndrome(usually haematuria, hypertension and impaired renal function)


Primary FSGS (i.e. genetic mutations)

Secondary FSGS (e.g. HIV, lupus, reflux nephropathy)

Typical investigation findings

Specific segments of certain glomeruli show segmental scarring in addition to foot process fusion


Supportive care – salt restriction and fluid management

High-dose prednisolone (approximately 50% of patients respond – treatment can be up to 4 months in adults)

Additional cyclophosphamide or ciclosporin is used in some cases to reduce proteinuria.


Typically progresses to end-stage kidney disease over the course of several years in up to 50% of patients

Corticosteroids can halt progression in some cases


Non-proliferative: Membranous glomerulonephritis

Membranous glomerulonephritis typically presents with nephrotic syndrome

It is usually a slowly progressive disease primarily affecting individuals between the ages of 30-50

It is most commonly idiopathic but can be associated with hepatitis B, malaria and system lupus erythematosus (SLE)


Immune complex deposition, resulting in complement activation against glomerular basement membrane proteins

Typical investigation findings

Microscopic analysis shows thickened glomerular basement membrane (but not mesangium)

Immunofluorescence shows diffuse uptake of IgG 


- Prednisolone and cyclophophamide

- escalate to rituximab when necessary

Corticosteroids are often used to treat progressive disease


1/3 have chronic membranous glomerulonephritis

1/3 go into remission

1/3 progress to end-stage renal failure


Proliferative: IgA nephropathy

IgA nephropathy is also known as Berger’s disease

It is the most common type of GN in adults worldwide

It typically presents as nephritic syndrome (macroscopic haematuria) 24-48 hours after an upper respiratory tract infection

Typical investigation findings

Microscopically the disease is characterised by:

increased numbers of mesangial cells

increased matrix (the cellular scaffolding that holds everything together)

Immunohistochemistry reveals IgA deposition in the matrix.


Some studies suggest that a course of high-dose prednisolone can reduce proteinuria and delay renal impairment

In patients with deteriorating renal function, immunosuppressive drugs are also often used


Prognosis is variable, with 20-30% of patients progressing to end-stage renal failure


Proliferative: Post-infectious glomerulonephritis

Post-infectious glomerulonephritis (PIGN) is an immunologically mediated glomerular injury triggered by an infection

PIGN is most commonly associated with streptococcal infections (referred to as post-streptococcal glomerulonephritis)

The disease typically presents approximately 2 weeks after infection with nephritic syndrome (i.e. gross haematuria, oliguria, oedema)

Typical investigation findings

Diffuse proliferative and exudative glomerular histology

Dominant C3 staining and subepithelial humps


Diagnosis is typically based on the presence of:

Symptoms and signs of GN

History of recent infection (e.g. streptococcal)

Raised streptococcal titres 


Management is largely supportive, with careful monitoring of fluid balance


PIGN is usually a self-limited disease, especially in children, but long-term follow-up studies indicate persistent low-grade renal abnormalities in a significant proportion of patients


Proliferative: Membranoproliferative glomerulonephritis

Membranoproliferative glomerulonephritis is a group of immune-mediated disorders characterised histologically by glomerular basement membrane (GBM) thickening and proliferative changes on light microscopy.

It should not be confused with membranous glomerulonephritis, a condition in which the basement membrane is thickened, but the mesangium is not.

The disease is associated with hepatitis C and several autoimmune conditions including systemic lupus erythematosus (SLE).

Typical investigation results


Thickened basement membrane

Thickened mesangium

Tram tracking” appearance


Subendothelial deposition of IgG


Children (with nephrotic-range proteinuria) – corticosteroids

Adults (dipyridamole and aspirin)

Kidney transplantation for patients with end-stage renal disease


Proliferative: Rapidly progressive glomerulonephritis (crescentic glomerulonephritis)

Rapidly progressive glomerulonephritis (RPGN) is acute nephritic syndrome accompanied by microscopic glomerular crescent formation with progression to renal failure within weeks to months.

RPGN is relatively uncommon, affecting 10 to 15% of patients with glomerulonephritis

It primarily occurs in patients aged 20 to 50 years


Proliferative: Anti-glomerular basement membrane antibody disease (Anti-GBM)

Anti-GBM disease is an immune-mediated pathology involving antibodies directed against glomerular basement membrane antigens (anti-GBM antibodies).

These antigens are located in the glomeruli and in the alveoli of the lungs.

A cell-mediated inflammatory response occurs as a result of the anti-GBM antibodies which can affect both the kidneys (nephritic syndrome) as well as the lungs (alveolar haemorrhage presenting with haemoptysis).

If the lungs and kidneys are involved the condition is known as Goodpasture’s syndrome.

Anti-GBM disease accounts for approximately 10% of RPGN.

Progression to renal failure is often rapid without medical intervention.

Typical investigation results

Immunohistochemistry –IgG deposits along the basement membrane of the glomerulus

Antibodies – anti-GBM antibodies


High dose immunosuppression is required:

IV prednisolone


(azathioprine for long-term maintenance)



Vasculitic: Granulomatosis with polyangiitis (Wegener's)



Granulomatosis with polyangiitis (GPA), previously known as Wegener’s granulomatosis, is an extremely rare long-term systemic disorder that involves the formation of granulomas and inflammation of blood vessels (vasculitis)

It is a form of vasculitis that affects both small and medium-sized vessels in many organs (commonly the upper respiratory tract, lungs and kidneys

Symptoms are highly variable, depending on which vessels are affected

GPA affecting the lungs, kidneys or heart can be life-threatening

The vasculitis is caused by anti-neutrophil cytoplasmic antibodies (c-ANCA)


A history of unexplained symptoms

Positive c-ANCA test

Biopsy (e.g. of kidney or cutaneous tissue) – leukocytoclastic vasculitis with necrotic changes and granulomatous inflammation (clumps of typically arranged white blood cells) on microscopy


Induction of remission – rituximab or cyclophosphamide in combination with high dose corticosteroids

Maintenance – methotrexate and corticosteroids


Vasculitic: Microscopic Polyangiitis

A systemic small-vessel vasculitis without clinical or pathological evidence of necrotising granulomatous inflammation

Clinical features can include weight loss, fevers, fatigue and renal failure

Ongoing inflammation is driven by anti-neutrophil cytoplasmic antibodies (p-ANCA)


Raised ESR and CRP


p-ANCA is positive in almost all cases


Long-term prednisolone and cyclophosphamide therapy (often cycled)

Plasmapheresis can be helpful acutely to remove p-ANCA antibodies


Peritoneal dialysis infection 

Staph epidermidis, treated with vancomycin


What is thin basement renal membrane nephropathy?

Autosomal dominant disorder that runs in families. Similar to Alport syndrome but not progressive and no hearing loss


Treatment option for recurrent renal calcium stones?

Bendroflumethiazide as it reduces calciu secretion


Site of action of diuretics

Ascending limb of Henle is impermeable to water


Bronchiectasis effect on kidney

Chronic inflammation leads to systemic amyloidosis leading to deposition with progressively worsening protein leak


Eosinophilic pgranulomatosis with polyangiitis presentation (CHurg-Strauss)

Poorly controlled asthma, sinusitis, mononeuritis and peripheral blood eosinophilia.

30% are pANCA positive and biopsies show necrotising granuloma


Which drug do you use to slow renal progression in SLE?

Use mycophenolate mofetil before. using cyclophosphamide because of better side effect profile.


What are the renal cystic disorders?

ADPKD, ARPKD, vHL and tuberous sclerosis


vHL presentation


Tuberous sclerosis presentation

HAMARTOMAS: Hamartoma, Adenoma sebaceum, Mental retardation (now properly referred to as intellectual disability), Ash leaf spots, Rhabdomyoma, Tubers, Optic hamartomas (phakomas), Mitral regurgitation, Astrocytomas, Seizures