Autoimmune - PMR and Giant cell arteritis

Question 1

PMR AND GCA
i) over what age do they usually occur? what ethnicity do they affect mostly?
ii) are they more common in F or M?
iii) what will ESR and CRP be?
iv) which two areas manifest with MSK symptoms?
v) what drug is there a dramatic early response to

Answer 1

i) over 50 yrs
ii) F>M 2-3:1
iii) high ESR and CRP
iv) shoulder and hip pain - proximal muscle pain and stiffness
v) corticosteroid

Question 2

PMR
I) what is it? how is it characterised?
ii) what causes it? what is it accompained by?
iii) what does incidence increase with? does it affect men or women more?
iv) which ethnicity does it mostly affect
v) which give things are core inclusion dx criteria?

Answer 2

i) chronic system inflammaory process charac by pain and morning stiffness in neck, shoulder, pelvic girdle muscles
ii) unknown cause accompanied by acute phase response
iii) incidence increases with age and affects women more
iv) mostly affects northern european ancestry
v) age >50yrs, duration > 2weeks, bilat shoulder or pelvic girdle ache. morning stuffness >45mins, acute phase response (ESR and CRP)
can be dz with normal inflam markers if classical pixture is seen and dramatic response to steroid

Question 3

SYMPTOMS OF PMR
i) which two areas is pain seen?
ii) how long after waking or rest is muscle stiffness lasting?
iii) name three systemic symptoms that may be seen?
iv) where is arthritis seen? (2) is it strong or mild?
v) is muscle strength usually impaired?

Answer 3

i) bilateral shoulder and pelvic girdle pain
ii) 45 mins
iii) low grade fever, fatigue, anorexia, weight loss, depression
iv) arth in knees and wrists - mild
v) muscle strength not usually impaired

Question 4

DIAGNOSING PMR
i) over what age should core features be identified in?
ii) what CRP and ESR levels? what drug will there be a positive response to?
iii) what other differential needs to be excluded? how is this done?
iv) name two other general DDs that need to be excluded

Answer 4

i) over 50
ii) high CRP and ESR and positive response to oral prednisolone
iii) exclude GCA as there will be very high CRP and ESR
iv) active infection or cancer

Question 5

TREATMENT OF PMR
i) what steroid should be given? when should this be reviewed?
ii) is CRP or ESR more reliable marker?
iii) what should be given as protection?
iv) what can be given as a steroid sparing agent
v) what is average tx duration

Answer 5

i) oral prednisolone 15mg per day (low dose) review if response is not dramatc
ii) CRP is more reliable
iii) bone protection with bisphosphonate / ca and vit D
iv) MTX
v) 1-2 years

Question 6

GIANT CELL ARTERITIS
i) what size vessel does it affect? what does it commonly co occur in?
ii) why is it essential to recognise and treat early? (2) what happens in the bv?
iii) what is mean age of onset? what is F:M? what descent is it most common in?

Answer 6

i) inflammation in medium and large vessels and commonly co occurs with PMR
ii) to prevent blindness and prevent complicats caused by other arterial occlusion
- increased inflammation, giant cells get stuck down and get occlusion
iii) mean age if 70yrs and 3:1 F:M
most common in northern european descent

Question 7

CLINICAL FEATURES OF GC
I) name three consititutional symptoms? what type of headache can be seen?
ii) name two other symptoms
iii) what is seen in the eyes? (2)
iv) which artery can be red, thick, pulseless and tender
v) what will ESR and CRP levels be

Answer 7

i) fever, fatigue, anorexia, weight loss, polymyalgia
ii) jaw/tongue claudication, scalp tenderness
iii) transient visual loss, partial field defect, temporary curtain over vision
iv) superficial temporal artery
v) very high ESR and CRP

Question 8

INVESTIGATIONS IN GCA
i) what may be seen on FBC? WBC,RBC,plats
ii) is serum free light chain paraprotein seen? will Igs be normal?
iii) will RF or anti CCP Abs be positive or negative? is ANA positive?
iv) name three differentials
v) name three diagnostic tests that may be use when GCA is suspected

Answer 8

i) normochornic normocytic anaemia (anaemia of chronic disease), high plats and high neuts
ii) no paraprotein or raised Igs
iii) negative RF and anti CCP and ANA
iv) herpes zoster, migraine, intracranial pathology, cluster headache, cervical spondylosis, TMJ pain
v) temporal artery biopsy at least 1cm in length (inflam can be patchy), US of temporal and axillary arteries or FDGPET, CTA or axillary atery US

Question 9

GCA TREATMENT
i) what initial treatment is given?
ii) what is given if patient has acute or intermittent visual loss?
iii) what should be co prescribed? what MAB can be given if previous tx fails

Answer 9

i) oral prednisolone - high dose
ii) give 1g IV methylpred daily for up to 3 days
iii) co prescribe bisphos, ca, vit D
give tocilizumab if previous tx fails (MAB to IL-6R)

Question 10

LARGE VESSEL GCA
i) what is it
ii) name two symptoms seen? when do pts present?
iii) give an eg of a large vessel arteritis

Answer 10

i) affects aorta and first order branches (subclavian) - doesnt have head symptoms
ii) claudication in upper limbs and assym BP, vascular bruits, constitutional symptoms
px younger - by 10 yrs
iii) takayasu aortitis