302 Multisystem diseases

Question 1

What are pulmonary renal syndromes?

Answer 1

A life threatening condition causing renal failure with associated respiratory impairment and manifests in the form of rapidly progressive glomerulonephritis with diffuse alveolar hemorrhage (DAH)

Question 2

What are some causes of Pulmonary renal syndromes?

Answer 2

ANCA associated vasculitis (60%)
Eg. Wegeners, Microscopic polyangitis, Churg Strauss syndrome

Goodpastures Syndrome (20%)

Others: SLE, uremic lung, pneumonia with infectious GN

Question 3

What is ANCA associated vasculitis?

Answer 3

ANCA: Anti-Neutrophil Cytoplasmic Antibody

ANCA associated vasculitis (AAV) is an umbrella term for a group of multi-system autoimmune small vessel vasculitides

Question 4

What is Wegeners disease?

Answer 4

An disorder that causes inflammation of the blood vessels in your nose, sinuses, throat, lungs and kidneys

A type of ANCA associated vasculitis

Question 5

What is Microscopic polyangitis?

Answer 5

An idiopathic autoimmune disease characterized by a systemic vasculitis that predominantly affects the smaller blood vessels

A type of ANCA associated vasculitis

Question 6

What is Churg Strauss syndrome?

Answer 6

A disorder marked by blood vessel inflammation

A type of ANCA associated vasculitis

Question 7

What is Goodpasture’s syndrome?

Answer 7

AKA anti-glomerular basement membrane (anti-GBM) disease

It’s an autoimmune disease that affects both the kidneys and lungs by the formation of autoantibodies that attack their basement membranes

Question 8

What are the manifestations of cutaneous vasculitis?

Answer 8

Palpable purpura, petechiae, urticaria, ulcers, livedo reticularis, and nodules

Question 9

What are Petechiae?

Answer 9

Pinpoint non-blanching spots that measure less than 2 mm in size and affect the skin and mucous membranes

Question 10

What is livedo reticularis?

Answer 10

A cutaneous physical sign characterized by transient or persistent, blotchy, reddish-blue to purple, net-like cyanotic pattern

It’s a manifestation of cutaneous blood flow disturbance

Question 11

What is the treatment for small vessel vasculitis?

Answer 11

Aggressive immunosuppressive therapy using steroids, cyclophosophamide and possibly plasmapharesis

Question 12

Which features diagnose SLE in kidney disorder?

Answer 12

Proteinuria, haematuria, and/or elevated creatinine

Question 13

Name some pathogenic auto-antibodies in SLE?

Answer 13

Anti-dsDNA
Nucleosomes
RO
La
Sm
NMDA
Phospholipids
Alpha-actinin
C1q

Question 14

Describe the classes of lupus nephritis

Answer 14

Class 1: minimal mesangial lupus nephritis

Class 2: mesangial lupus nephritis (mesangial hypercellarity with mesangial immune deposits on immunofluorscence)

Class 3: focal proliferative lupus nephritis affecting <50% of glomeruli

Class 4: diffuse proliferative lupus nephritis affecting >50% of glomeruli

Class 5: membranous lupus nephritis (thickening of capillary walls and global immune deposits)

Class 6: advanced sclerosis lupus nephritis (>90% glomeruli globally sclerosed)

Question 15

What is cardiorenal syndrome?

Answer 15

Any acute or chronic problem in the heart or kidneys that could result in an acute or chronic problem of the other

Question 16

What is the pathogenesis of kidney disease in chronic heart failure?

Answer 16

There is increase in the nuerohormonal vasoconstrictors in CHF leading to afferent arteriolar vasoconstriction causing Na and K retention and water retention

There is increase in the hormonal vasodilators as ANP and renal prostaglandins
The nuerohormonal changes lead to decrease in renal blood flow and GFR and may give a picture of pre- renal azotaemia or lead to worsening of a pre - existing renal impairment to the point that dialysis may be required

Question 17

What is a hepatorenal syndrome?

Answer 17

Progressive oliguric renal failure either insidious or rapid

Usually occur in hospitalized patients

May be precipitated by bleeding aggressive diuresis or abdominal paracentesis
Functional renal failure with very low Na
Should differentiated from ATN and pre renal states

Question 18

What is paracentesis?

Answer 18

The perforation of something like a cyst

Question 19

What is type 1 hepatorenal syndrome?

Answer 19

Rapid progression (<2 weeks)
Trigger: alcohol or sepsis

If untreated, mortality within 2 weeks

Question 20

What is type 2 hepatorenal syndrome?

Answer 20

Slow progression

Presents with diuretic resistant ascites

Better prognosis than type 1

Question 21

What is the criteria for diagnosis of a hepatorenal syndrome?

Answer 21

Cirrhosis with ascites
Serum creatinine >133
Absence of hypovolaemia

Question 22

How can the liver cirrhosis cause kidney disease?

Answer 22

Liver disease will cause retention of Na to the point where the urine in Na free

Very low Na in urine is a marker of disease severity and indicates poor response to diuretics

Question 23

What is the treatment for hepatorenal syndrome?

Answer 23

Search for correctable causes
Na+ and water restriction

Dialysis is not effective unless to support and candidate for transplant

Question 24

Name some disease associated with glomerular diseases?

Answer 24

Bacteria of all types
HIV
Hepatitis C and B
Erythrovirus (parvovirus)

Question 25

What is FSGS?

Answer 25

Focal Segmental glomerulosclerosis

A type of glomerular disease and describes scarring (sclerosis) in your kidney

Question 26

Which tumours are associated with glomerular disease?

Answer 26

Lymphoma
Leukaemia
Myeloma
Carcinoma