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MSK > Crystal Arthropathy and Polymyalgia Rheumatica > Flashcards

Crystal Arthropathy and Polymyalgia Rheumatica Flashcards

1
Q

What are the common crystal deposition diseases characterised by?

A

Deposition of mineralised material within the joints and periarticular tissue

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2
Q

What substance is present in gout?

A

Monosodium urate

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3
Q

What substance is present in pseudogout?

A

Calcium Pyrophosphated DiHydrate (CPPD)

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4
Q

What substance is present in calcific periarthrtiis/tendonitis?

A

Basic calcium phosphate hydroxy-apatite (BCP)

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5
Q

What is tophus?

A

Massive accumulation of uric acid

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6
Q

Pathology of Lesch Nyan Syndromes

A

HGPRT deficiencies
- hypoxanthine guanine phosphonbosy transferase
- normally plays a role in recycling purine bases, hypoxanthine and guanine, into purine nucleotide pools
- in the absence of this, the purine bases are degraded and excreted as uric acid
- the synthetic rate for purines also accelerates
The failure of recycling together with increased synthesis of purines is the basis for the over production of uric acid
X linked recessive

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7
Q

Features of Lesch Nyan syndromes

A 
Intellectual disability
Aggressive and impulsive behaviour
self mutilation 
gout
renal disease
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8
Q

Who gets pseudogout?

A

The knee is to pseudogout as the toe is to gout

Elderly females

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9
Q

Causes of pseudogout

A

Idiopathic
Familial
metabolic

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10
Q

Triggers of pseudogout

A

Trauma

Intercurrent illness

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11
Q

Features of pseudogout

A

Erratic flares
Chondrocalcinosis
Pyrophosphate crystals

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12
Q

Treatment of pseudogout

A

NSAIDs

I/A steriods

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13
Q

Is there prophylactic therapy for pseudogout?

A

No

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14
Q

Pathology of gout

A

Purine metabolism

  • endogenous production of uric acid from degradation of purines contributes to 2/3rds of body urate pool, remainder dietary in origin
  • 70% of uric acid produced is excreted by the kidneys and the rest by the biliary tract and bacteria
  • in the vast majority of people with gout, HYPERURICAEMIA RESULTS FROM REDUCED EFFICIENCY OF RENAL URATE CLEARANCE
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15
Q

Causes of hyperuricaemia

A 
Overproduction 
- malignancy e.g. lymphoproliferative 
- severe exfoliative psoriasis
- drugs, e.g. ethanol, cytotoxic drugs 
- inborn errors of metabolism 
- HGPRT deficiency 
Underexcretion 
- renal impairment 
- hypertension 
- hypothyroidism 
- drugs e.g. alcohol, low dose aspirin, cyclosporin
- exercise
- starvation 
- dehydration 
- lead poisoning
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16
Q

Why does alcohol increase the risk of gout?

A

Some alcoholic drinks are rich in purines, noteably beer
Metabolism of ethanol to acetyl CoA leads to the formation of a precursor of uric acid
Alcohol also raises the lactic acid level in the blood which inhibits the uric acid secretion

17
Q

Who gets gout?

A 
Older men (men have higher urate levels than women)
Very rare in young women
18
Q

Gout and women

A

Very rare in young women as oestrogen has a uricosuric effect
after menopause, urate levels rise and gout becomes increasingly prevalent

19
Q

Ageing is a risk factor for both men and women for gout due to …..

A

Increase in sUA levels (mainly due to reduced renal function)
Increased use of diuretics and other drugs that are raised in sUA
Age related changes in connective tissues which may encourage crystal formation
Increased prevalence of OA

20
Q

Treatment of gout/hyperuricaemia

A 
Acute flare of gout 
- NSAIDs
- Colchicine 
- Steroids (I/A, I/M, oral) 
Hyperuricaemia 
- prophylactically prior to treating malignancies 
- lowering uric acid
         - xanthine oxidase inbihitor (allopurinol)
         - febuxostat 
         - uricosuric agents (sulphinpyrazone)
         - canakinumab 
Lifestyle
- BP
- weight loss
- diet
- alcohol and smoking
21
Q

When is hyperuricaemia treated?

A 
DO NOT TREAT IF ASYMPTOMATIC
1st attack not treated unless 
- single attack of polyarticular gout 
- tophaceous gout
- urate calculi 
- renal insufficiency 
Treat if second attack in one year
22
Q

Rules for lowering uric acid levels

A

Wait until the acute attack has settled before attempting to reduce the urate level

Use prophylactic NSAIDs or low dose colchicine/steroids until the urate level Is normal

Adjust allopurinol dose according to renal function

23
Q

What is polymyalgia rheumatica?

A

An inflammatory condition of the elderly causing pain, stiffness and inflammation of muscles around the shoulders, neck and hips

24
Q

What is polymyalgia rheumatica closely related to?

A

GCA

25
Q

Symptoms of gout

A

Sudden severe pain

Swelling, redness and tenderness over joints

26
Q

Where is gout pain often seen?

A

The base of the big toe

27
Q

Who gets polymyalgia rheumatica?

A

rare <50, usually > 70 y/o

F > M 2:1

28
Q

Presentation of polymyalgia rheumatica

A 
Rapid onset < 1 month 
SUDDEN onset of shoulder +/- pelvic girdle stiffness
Aching, morning stiffness in proximal limbs (NOT weakness)
ESR usually > 45 often 100 
Anaemia
Malaise
Weight loss 
Fever
Night sweats 
Depression
29
Q

Diagnosis of polymyalgia rheumatica

A 
Compatible history 
Age > 50 
ESR > 50 
Dramatic steroid response 
*no specific diagnostic test*
30
Q

Differential diagnosis for polymyalgia rheumatica

A 
Myalgic onset inflammatory disease 
underlying malignancy
- multiple myeloma
- lung cancer
Inflammatory muscle disease 
Hypo/hyperthyroidism 
Bilateral shoulder capsulitis 
Fibromyalgia
31
Q

Treatment of polymyalgia rheumatica

A

Prednisolone 15mg per day daily 18-24 month course

Bone prophylaxis

32
Q

Where is most commonly affected in pseudogout?

A

Knee
Wrist
Shoulder

33
Q

How does colchicine work?

A

Inhibits microtubule polymerisation by binding to tubulin, interfering with mitosis. Also inhibits neutrophil motility and activity

34
Q

Classic side effect of colchicine

A

Diarrhoea

35
Q

What does polymyalgia rheumatica NOT have?

A

Weakness

36
Q

What are chemo patients at increased risk of and why?

A

Gout

Increased urate production

37
Q

What should happen to allopurinol treatment if they are already on the treatment, but have an acute flare of gout?

A

Continue allopurinol on current dose

38
Q

RFs for pseudogout

A 
Hyperparathyroidism 
Hemochromatosis
Acromegaly
Low Magnesium 
Low phosphate
Wilsons disease
39
Q

What is seen on joint aspiration if have pseudogout?

A

Weakly-positively birefringent rhomboid-shaped crystals

MSK (25 decks)

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