Crystal Arthropathy Flashcards

1
Q

What are common crystal deposition diseases characterised by?

A

Deposition of mineralised material within joints and peri-articular tissue

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2
Q

What is the crystal responsible for gout?

A

Monosodium urate

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3
Q

What is the crystal responsible for pseudo gout?

A

Calcium pyrophosphate dihydrate (CPPD)

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4
Q

What is the crystal responsible for calcific periarthritis/tendonitis?

A

Basic calcium phosphate hydroxy-apatite (BCP)

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5
Q

What are the 2 most common crystal arthropathies?

A

Gout and pseudogout

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6
Q

What fractions of uric acid in the body comes from dietary purines?

A
  • 1/3
  • (2/3 - DNA, RNA, HGPRT)
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7
Q

What is the breakdown process for purines?

A

purines -> hypoxanthine -> xanthine -> plasma urate

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8
Q

How is uric acid excreted from the body?

A
  • 70% via kidneys
  • 30% into the biliary tract (subsequently converted by colonic bacterial uricase to allantoin)
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9
Q

How does hyperuricaemia occur in the vast majority of people with gout?

A

Reduced efficiency of renal urate clearance

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10
Q

What are the 2 causes of hyperuricaemia?

A

Overproduction and under-excretion

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11
Q

What are the underlying causes of overproduction?

A
  • Malignancy e.g lymphoproliferative, tumour lysis syndrome
  • Severe exfoliative psoriasis
  • Drugs e.g. ethanol, cytotoxic drugs
  • Inborn errors of metabolism
  • HGPRT deficiency
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12
Q

What are the underlying causes of under-excretion?

A
  • Renal impairment
  • Hypertension
  • Hypothyroidism
  • Drugs e.g. alcohol, low dose aspirin, diuretics, cyclosporin
  • Exercise, starvation, dehydration
  • Lead poisoning
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13
Q

Why is alcohol thought to increase the risk of gout?

A

As the metabolism of ethanol to acetyl CoA leads to adenine nucleotide degradation resulting in increased formation of adenosine monophosphate, a precursor of uric acid

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14
Q

What is Lesch Nyan syndrome?

A

Genetic HGPRT deficiency

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15
Q

What are the features of Lesch Nyan syndrome?

A

•X-linked recessive •Intellectual disability •Aggressive and impulsive behaviour •Self mutilation •Gout •Renal disease

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16
Q

What is the importance of HGPRT deficiency?

A
  • HGPRT - hypoxanthine-guanine phosphoribosyl transferase
  • Plays a key role in the recycling of the purine bases (hypoxanthine and guanine) in the purine nucleotide pools
  • In absence of HGPRT, the purine bases cannot be salvaged and they are degraded and excreted as uric acid
  • There is also an increased synthesis of purines (presumably to compensate for purines lost) which also contributes to the over-production of uric acid
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17
Q

Who gets gout?

A
  • Predominantly older men
  • Men always > women
  • Men have higher rate levels
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18
Q

How does oestrogen affect uric acid?

A

It has a uricosuric effect making gout very rare in younger women

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19
Q

How does ageing affect prevalence of gout?

A
  • There is an increased in sUA levels mainly due to reduced renal function
  • Increased use of diuretic and other drugs that raise sUA
  • Age-related changes in connective tissues which may encourage crystal formation
  • Increased prevalence of OA
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20
Q

How is gout diagnosed?

A

•History •Examination •Differential Diagnosis •Investigations

21
Q

Where do gout episodes classically occur?

A

The 1st metatarsophalangeal joint

22
Q

What is the history of a gout episode?

A

•Classically overnight onset •May have been on holiday - alcohol and dehydration •Erythema •Unbearably painful •Lasts 5-7 days •If severe - inflammation of the overlying skin •Potentially discharges f chalky material

23
Q

What are tophi?

A

A tophus is a massive accumulation of uric acid

24
Q

Where do tophi occur?

A

•Bony prominences •Ear

25
What is the gold standard in gout investigation?
* Aspirate the joint and look for crystals * Aspiration is also important for differential diagnosis of sepsis * Don't usually aspirate the 1st MTP for gout as in so common in that location
26
How is an acute flare of gout treated?
* NSAIDs - some patients cannot take this * Colchicine - has anti-inflammatory properties, perhaps slower onset, causes diarrhoea SPECIFICALLY FAVOURED IN GOUT * Steroids - useful in renal impairments
27
When does a patient receive prophylactic intervention after a 1st attack of gout?
•Single attack of polyarticular gout •Tophaceous gout •Urate calculi •Renal insufficiency
28
What are other indications for prophylactic/long-term treatment of gout?
* Treat if 2nd attack within 1 yr * Prophylactically prior to treating certain malignancies
29
How is long-term hyperuricaemia treated?
* Do not treat asymptomatic hyperuricaemia * Xanthine oxidase inhibitor e.g. _Allopurinol_ MOST COMMON * _Febuxostat_ - option for patients who are unable to tolerate allopurinol, can increase risk of cardiovascular death * Uricosuric agents e.g. sulphinpyrazone, probenecid, benzbromarone * Canakinumab - IL 1 antagonist, extremely expensive
30
How to allopurinol and febuxostat work?
They inhibit xanthine oxidase. Hypoxanthine -x\> Xanthine -x\> plasma urate
31
At what point should you attempt to reduce the urate level?
* Wait until the acute attack has settled * Use prophylactic NSAIDs or low dose colchicine/steroids until urate level normal (poss. with allopurinol treatment) * HOWEVER, if already on allopurinol during acute attack, continue treatment
32
What factor is important when using allopurinol?
•Adjust allopurinol dose according to renal function •Increase monthly until uric acid \<300 and begin to wean off NSAIDs/colchicine
33
Where is pseudogout most commonly seen?
The knee
34
Who gets pseudogout?
Elderly females
35
What are the features of pseudogout?
* Erratic flares * Triggers - trauma, intercurrent illness
36
What is the aetiology of pseudogout?
•Idiopathic •Familial •Metabolic
37
What is an x-ray feature of pseudogout?
Chondrocalcinosis
38
What are the crystals seen under the microscope in pseudogout?
Pyrophosphate crystals
39
What is the management of pseudogout?
* NSAIDs * I/A steroids
40
What are the prophylactic therapies for pseudogout?
THERE ARE NONE
41
What is polymyalgia rheumatica?
* Inflammatory condition of the elderly * Close relationship with giant cell arteritis (GCA)
42
What percentage of patients with polymyalgia rheumatic (PMR) have evidence of GCA?
20%
43
What percentage of patients with GCA may have PMR?
50%
44
How does PMR present?
* Sudden onset of shoulder +/- pelvic girdle stiffness * Rare \< 50y * Usually \> 70y * F:M 2:1 * ESR usually \> 45 often 100 * Anaemia * Malaise, weight loss, fever, depression * Arthralgia/synovitis occasionally
45
How is PMR diagnosed?
* Compatible history * Age \> 50 * ESR \> 50 * Dramatic steroid response * No specific diagnostic test
46
What are the differential diagnoses for PMR?
* Myalgiconset Inflammatory joint disease - rheumatoid arthritis * Underlying malignancy e.g Multiple myeloma, lung cancer * Inflammatory muscle disease * Hypo/hyperthyroidism * Bilateral shoulder capsulitis * Fibromyalgia
47
48
What is the treatment for PMR?
* Prednisolone 15mg per day initially * 18-24mthcourse * Bone prophylaxis - \>65 bone protection, \<65 dexascan needed first