Miscellaneous

Question 1

Which of the following is the best definition of chronic inflammation?

1. Inflammation which persists for effectively more than 30 days in any 6-month period
2. Inflammation persisting for more than 7 days with no discernible cause after appropriate work up
3. A tissue response characterised by tissue injury, inflammation, and repair persisting in variable amounts for weeks to months
4. Tissue infiltration by polyclonal neutrophils for over 2 weeks
5. A tissue response characterised by granuloma formation

Answer 1

3. *A tissue response characterised by tissue injury, inflammation, and repair persisting in variable amounts for weeks to months

Question 2

Concerning Reperfusion Injury, which of the following is most correct?

1. It describes the volume tissue initially at-risk MINUS that saved after successful reperfusion is established
2. It describes the volume of tissue at risk but still viable before reperfusion is established
3. It describes the volume of tissue still viable considered saved after reperfusion is established
4. It is a term for the borderzone ischaemic territory / penumbra
5. It describes a condition of FURTHER cell damage occurring with reperfusion after hypoperfusion, SEPARATE to the damage of the original insult.

Answer 2

5. *It describes a condition of FURTHER cell damage occurring with reperfusion after hypoperfusion, SEPARATE to the damage of the original insult.

Question 3

Which of the following is LEAST correct about shock?

1. Shock can occur with spinal cord injury and anaesthetic spinal blocks
2. Cardiogenic shock is most commonly due to infarct but it may be seen with pericardial effusion
3. Hypovolaemic shock is associated with severe burns
4. Shock with PE may be due to poor left atrial filling
5. Ventricular dysrhythmia is not an accepted cause of shock

Answer 3

5. *Ventricular dysrhythmia is not an accepted cause of shock

Question 4

What TNM staging classification is not possible? (March 2015)

a. PTxN1M1
b. T1N0M1

Answer 4

Unsure – all theoretically possible. T1N0M0 seems least likely.

ANSWER: Possible T1N0M1

Question 5

Which of the following is included in the diagnostic criteria for SLE? (March 2014)

a. Pericarditis

Answer 5

Classification of SLE is by clinical & immunologic criteria. Must have > or equal to 4 criteria for Dx, w at least one clinical & one immunologic criteria

Clinical criteria:

- Acute cutaneous lupus including malar rash
- Chronic cutaneous lupus including classic discoid rash
- Oral or nasal ulcers
- Non-scarring alopecia
- Synovitis - At least 2 joints w at least 30 min of morning stiffness. Swelling & effusion
- Serositis: Pleuritis/effusion, Pericarditis
- Renal
- Neurologic inc seizures & altered mental state
- Haemolytic anaemia
- Leukopaenia
- Thrombocytopaenia

Immunologic:
	ANA
	Anti-DNA
	Anti-Sm
	Antiphospholipid antibody
	Low complement
	Direct Coombes test positive in the absence of haemolytic anaemia

ANSWER: Pericarditis is part of the classification under the heading of ‘serositis’, which also includes pleuritis / pleural effusion

Question 6

Which association is false? (March 2014)

a. Anti-endomycial antibodies with diarrhoea
b. Anti-cardiolipin antibodies and symmetric arthritis
c. Anti-basement membrane membranes and haemoptysis
d. Anti-neutrophil cytoplasmic antibodies and epistaxis
e. Anti-mitochondrial antibodies and jaundice

Answer 6

• Anti-endomysial antibody part of the work-up for coeliac disease
- AKA anti-transglutaminase antibody
- Has been also implicated in IBD
• Anti-cardiolipin antibodies can be seen in SLE which gives a symmetric polyarthritis, seen in ~70% of patients
• Anti-basement membrane antibodies are assoc w Goodpastures syndrome (gives pulmonary haemorrhage & nephritis)
• Anti-neutrophil cystoplasmic antibodies (ANCA) & epistaxis
- cANCA is assoc w GPA/Wegeners which can present w epistaxis
• Anti-mitochondrial antibodies are assoc w primary biliary cirrhosis
- This presents w a cholestatic picture

ANSWER: All associations are correct

Question 7

Which is true regarding amyloid? (August 2014)

a. B2 causes cardiomyopathy
b. AA is seen in medullary thyroid
c. AL is associated with chronic lymphoid leukaemia
d. … is associated with bronchiectasis
e. … is associated with haemolysis

Answer 7

Amyloid:
o Amyloid is a heterogenous group of fibrillar proteins that share the ability to aggregate into an insoluble, cross-beta pleated sheet tertiary conformation
- Fibrils accumulate in extra-cellular tissues
• Excess synthesis & resistance to catabolism
- Accumulation of amyloid leads to progressive pressure atrophy on the adjacent parenchyma
- Clinical manifestations range from life-threatening to occult

Lab
Congo red staining: Salmon pink, apple green birefringence

Types of amyloid:
AL: Amyloid light chain
• Excess production of light chains from abnormal plasma cells
o Seen in multiple myeloma
• Most commonly lambda light chain (less commonly kappa)

AA:
• Assoc w chronic inflammatory states
• Derived from SAA, which is an acute phase reactant synthesized in hepatocytes

AB:
• Forms the core of cerebral plaques, deposits in the walls of vessels in Alzheimer disease
• Derived from transmembrane amyloid precursor protein

B2 microglobulin:
• Complicates long term haemodialysis

TTR: Transthyretin
• Normal serum protein which transport thyroxine & retinol
• Older patients: can deposit in the heart
• Assoc w familial amyloid polyneuropathy

Systemic amyloidosis conditions:
Primary amyloidosis:
• AL type amyloid – 10-15% of patients w MM

Reactive secondary amyloidosis:
• AA type amyloid
• Chronic inflammatory states (e.g. RA, scleroderma, dermatomyositis, bronchiectasis, chronic OM) & non-immunocyte tumours (e.g. Hodgkin lymphoma, RCC)

Heredofamilial amyloidosis:
• E.g. Familial Mediterranean Fever: overproduction of IL-1, assoc w AA amyloid suggesting underlying chronic inflammation

Haemodialysis associated:
• Deposition of B2 amyloid in joints, synovium, tendon sheaths
• B2 amyloid is not filtered by traditional dialysis membranes (improved)

Patterns of organ involvement in systemic amyloid:
Kidneys:
• Mesangial & subendothelial initially
• Progresses to complete hyalization of the glomerulus
• Deposits in the BM, extend to interstitium over time
• Hyaline thickening of vessel walls causing chronic ischaemia - Tubular necrosis & fibrosis

Spleen: Splenomegaly
• Patterns:
- Sago spleen: deposition limited to splenic follicles
- Lardaceous spleen: deposition in the red pulp, giving large geographic areas of amyloid deposition

Liver:
• Hepatomegaly
• Initial deposition in the space of Disse, w progressive involvement of the parenchyma & sinusoids
• Pressure atrophy w massive hepatic replacement

Heart:
• Subendocardial droplets or amyloid accumulation
• Vascular & subepicardial deposits may also occur
• Interstitial & perimyocyte deposition - Progressive pressure atrophy

Other:
• Nodular deposits in the tongue
• Carpal ligament of the wrist (especially w haemodialysis assoc)
• AL can bind & inactivate clotting factors => bleeding diathesis
• Medullary thyroid cancer assoc amyloid is composed of calcitonin chains

ANSWER: No responses are correct. Cardiomyopathy is most assoc w AL & AA amyloid. Thyroid medullary carcinoma is assoc w calcitonin amyloid. AL is assoc w MM rather than CLL. Bronchiectasis is assoc w AA amyloid. Haemolysis is assoc w AL amyloid.