Crystal Arthropathies and Polymyalgia Rheumatica Flashcards

1
Q

What are crystal deposition diseases?

A

Characterised by deposition of mineralised material within joints and peri-articular tissue

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2
Q

What are commonly seen crystal deposition diseases in practice?

A

Gout - monosodium urate
Pseudogout - calcium pyrophosphate dihydrate (DPPD)
Calcific peri-arthritis/ tendonitis - basic calcium phosphate hydroxy-apatite (BCP)

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3
Q

Describe the metabolism of purines

A

Purines are from diet and DNA + RNA
Then make hypoxanthine then xanthine - purine recycled by HGPRT
Then plasma urate which is excreted as urine uric acid

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4
Q

What causes an overproduction of uric acid causing hyperuricaemia?

A

Malignancy - lymphoproliferative and tumour lysis syndrome
Severe exfoliative psoriasis
Drugs - ethanol and cytotoxic drugs
Inborn errors of metabolism
HGPRT deficiency

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5
Q

What causes an under excretion of uric acid leading to hyperuraemia?

A

Renal impairment
Hypertension
Hypothyroidism
Drugs - alcohol, low dose aspirin, diuretics and cyclosporine
Exercise, starvation and dehydration
Lead poisoning

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6
Q

Describe Lesch Nyan syndrome

A

HGPRT deficiency
Is X-linked recessive
Intellectual disability
Aggressive and impulsive behaviour
Self mutilation, gout and renal disease

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7
Q

What is the prevalence of gout in the UK?

A

Highest in 75+
Higher in males
Increses with age

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8
Q

What is the investigation for gout?

A

Aspirate and blood test for uric acid but low in serum as all in joint so after treatment of flare then check bloods.
Also renal function tests in blood
US - in chronic there is double layer of crystals seen

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9
Q

What is tophus?

A

Massive accumulation of uric acid

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10
Q

Can gout be polyarticular?

A

Yes but commonly mono-articular

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11
Q

How does gout look under microscope?

A

Needle shaped crystals of gout and negatively bifringed

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12
Q

What is the management of an acute flare of gout?

A

NSAIDs
Colchicine
Steroids - IA, IM and oral

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13
Q

What is the long term management for gout?

A

1st attack not treated unless - single attack of polyarticular gout, tophaceous gout, urate calculi and renal insufficiency
Treat 2nd attack within 1 year
Do not treat asymptomatic hyperuricaemia
Address CV and lifestyle factors

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14
Q

What is 1st and 2nd line for lowering uric acid?

A

1st line - Xanthine oxidase inhibitor ex. Allopurinol
2nd - Febuxostat

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15
Q

What is 3rd line for lowering uric acid?

A

Uricosuric agents (excrete uric acid) - probenecid and benzbromarone
Canakinumab

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16
Q

What is the rules for lowering uric acid levels?

A

1 - wait until the acute attack has settled before attempting to reduce
2 - use prophylactic NSAIDs or lose doe colchicine/ steroids until urate levels normal
3 - adjust allopurinol dose according to renal function

17
Q

What is pseudogout?

A

The knee is pseudogout and toe is gout
Elderly females
Erratic flares

18
Q

What is the aetiology and triggers for psuedogout?

A

Idiopathic, familial and metabolic
Triggers - trauma and intercurrent illness

19
Q

What can be seen on x-ray of pseudogout?

A

Chondrocalcinosis -Deposits of calcium in cartilage of joint
Loss of joint space
Sub-chondrosclorosis
Evidence of OA in wrist

20
Q

How does pseudogout look under microscope?

A

Pyrophosphate crystals
Very small and not as many
Intracellular rhomboid crystals - positive bifringes

21
Q

What is the management of pseudogout?

A

NSAIDs
Intra-articular steroids
There are no prophylactic therapies

22
Q

What other conditions is polymyalgia rheumatica associated with?

A

Giant cell arteritis - 20% have this and 50% of GCA have PMR
High ESR anaemia

23
Q

What is the presentation of polymyalgia rheumatica?

A

Sudden onset of shoulder and possible pelvic griddle stiffness
ESR usually more than 45 (often 100)
Anaemia, malaise, weight loss, fever and depression
Arthralgia/ synovitis occasionally

24
Q

What are the factors for polymyalgia rheumatica?

A

More females to males (2:1)
Rare in under 50 years and usually over 70 year olds

25
Q

How is polymyalgia rheumatica diagnosed?

A

Compatible history
Age over 50
ESR over 50
Dramatic steroid response
No specific diagnostic test

26
Q

What is the differential diagnosis for polymyalgia rheumatic (PMR)?

A

Myalgia onset inflammatory joint disease
Underlying malignancy - multiple myeloma and lung cancer
Inflammatory muscle disease
Hypo/ hyperthyroidism
Bilateral shoulder capsulitis
Fibromyalgia

27
Q

What is the treatment of PMR?

A

Prednisolone 15mg pre day initially
18-24 month course
Bone prophylaxis - alendronic acid, calcium and Vitamin D