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Finals > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

large vessel vasculitides

A

takayasu arteritis

giant cell arteritis

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2
Q

medium vessel vasculitides

A

polyarteritis nodosa

kawasaki disease

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3
Q

small vessel vasculitides

A

ANCA-associated
microscopic polyangiitis
granulomatous with polyangiitis (wegeners)
eosinophilic granulomatosis with polyangiitis (churg strauss)

immune complex
cryoglobulinaemic vasculitis
IgA vasculitis (henoch schonlein)
anti C1q vasculitis

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4
Q

features of giant cell arteritis

A

most common form of systemic vasculitis - granulomatous arteritis of aorta + major branches, affects extracranial branches of carotid esp temporal artery

assx with polymyalgia rheumatics

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5
Q

pathophysiology of giant cell arteritis

A

dendritic cells recruit CD4+ cells and activate macrophages which destory internal elastic lamina causing granuloma and giant cell formation

causes thickening and focal lesions - may or not be picked up by temporal artery biopsy

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6
Q

symptoms of giant cell arteritis

A

involvement of cranial nerves - headache, jaw claudication, scalp tenderness, loss of vision

involvement of great vessels - arm claudication

systemic inflammation - fever night sweats weight loss

PMR - proximal myalgua

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7
Q

examination findings of giant cell arteritis

A

tender/thickened/nodular temporal artery
scalp tenderness
proximal muscle weakness
visual loss - ?examine visual acuity - due to anterior ischaemic optic neuropathy

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8
Q

investigation findings in giant cell arteritis

A

ESR, CRP +++
fundoscopy /slit lamp - refer to ophthal
US temporal artery - halo sign
temporal artery biopsy
FDG PET scan - assesses disease activity

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9
Q

management of giant cell arteritis

A

high dose steroids - medical emergency

with ocular sx - IV methylprednisolone
no ocular sx - PO pred

if relapsing - methotrexate

add tocilizumab if risk of steroid toxicity

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10
Q

features of polymyalgia rheumatics

A

inflammatory condition of elderly women >50y characterised by proximal limb girdle weakness
non specifically unwell
stiffness
fever, weight loss, fatigue
bursitis/synovitis

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11
Q

investigation findings for polymyalgia rheumatica

A

high ESR/CRP
broad panel of bloods due to vague presentation - myeloma screen, RH, CCP, renal, bone

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12
Q

management of polymyalgia rheumatica

A

steroids - prednisolone 15mg/day - should respond <48h - reconsider diagnosis if not

PPI and bone protection

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13
Q

definition of takayasu arteritis

A

granulomatous inflammation of aorta and major branches
subclavian artery - arm claudication
renal artery - hypertension
carotid artery - CNS features

occurs in <40y F, asian
rare

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14
Q

pathophysiology of takayasu

A

granulomatous necrotising vasculitis affecting large vessels – adventitial thickening, infiltration of media, fibrosis of intima and thus stenosis

dilatation – aneurysms

can be local or widespread inflammation

commonly starts at L subclavian artery

genetic+ environmental

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15
Q

features of takayasu arteritis

A

absent peripheral pulses
claudication of extremities
transient visual disturbance - amaurosis fugax
TIAs, CVAs - young people with high inflammatory markers
syncope - subclavian steal
BP difference between arms
bruits
weight loss, myalgia, sweats

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16
Q

investigation findings of takayasu arteritis

A

ESR, CRP ++

MRA, CTA - imaging aorta and arterial tree
USS doppler of carotid and subclavian

PET

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17
Q

management of takayasu arteritis

A

high dose steroid and methotrexate (if steroid sparing agent needed)

steroid sparing immunosuppression long term - cyclophosphamide

biologics

endovascular interventions e.g., stenting

serial MRA

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18
Q

definition of polyarteritis nodosa

A

necrotising inflammation of medium-sized arteries (GI, renal, coronary)

assx hepatitis B
occurs in males 60s

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19
Q

investigation of polyarteritis nodosa

A

urinalysis
uPCR
bloods - raised inf markers
hep serology
skin biopsy
nerve conduction studies
angiography
seronegative

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20
Q

management of polyarteritis nodosa

A

mild - oral steroids
systemic - IV steroids, IV cyclophosphamide

if HBV + - treat with antiviral treatment

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21
Q

what are the ANCA associated vasculitides

A

small vessel vasculitides

granulomatosis with polyangiitis (Wegeners)

eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

microscopic polyangiitis

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22
Q

common features in ANCA associated vasculitides

A

renal impairment - due to immune complex glomerulonephritis causing raised creatinine, haematuria, proteinuria

respiratory symptoms - dyspnoea, haemoptysis

systemic sx - fatigue, weight loss, fever

vasculitis rash

ENT sx - sinusitis

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23
Q

the two types of ANCA

A

cANCA - granulomatosis with polyangiitis
cANCA levels related to disease activity

pANCA - eosinophilic granulomatosis with polyangiitis + microscopic polyangiitis, UC, PSC, anti GBC disease, Crohns
pANCA levels unrelated to disease activity

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24
Q

features of ankylosing spondylitis

A

HLA-B27 associated spondyloarthropathy presenting in males 20-30y

lower back pain and stiffness (worse in morning, improves with exercise)
pain at night

7As
Apical fibrosis
Anterior uveitis
Aortic regurgitation
Achilles tendonitis
AV node block
Amyloidosis
and cauda equina syndrome
peripheral arthritis (25%, more common if female)

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25
Q

clinical examination findings in ankylosing spondylitis

A

reduced lateral flexion
reduced forward flexion - schober’s test
reduced chest expansion

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26
Q

investigation findings in ankylosing spondylitis

A

XR - sacroiliitis (subchondral erosions, sclerosis), squaring of lumbar vertebrae, bamboo spine, syndesmophytes

CXR - apical fibrosis

MRI - early inflammation of sacroiliac joints - bone marrow oedema

spirometry - restrictive defect

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27
Q

management of ankylosing spondylitis

A

regular exercise
NSAIDs
physiotherapy
DMARDs - useful if peripheral joint involvement
anti TNF if refractory high disease activity

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28
Q

definition of antiphospholipid syndrome

A

acquired disorder - predisposition to venous and arterial thrombosis, foetal loss, thrombocytopenia

may be primary disorder or secondary (e.g., SLE)

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29
Q

features of antiphospholipid syndrome

A

venous/arterial thrombosis
recurrent miscarriages
livedo reticularis
other features: pre-eclampsia, pulmonary hypertension

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30
Q

investigation findings in antiphospholipid syndrome

A

antibodies
anticardiolipin antibodies
anti-beta2 glycoprotein I (anti-beta2GPI) antibodies
lupus anticoagulant

thrombocytopenia

prolonged APTT

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31
Q

management of antiphospholipid syndrome

A

primary thromboprophylaxis - low dose aspirin

secondary thromboprophylaxis
after initial event - lifelong warfarin INR 2-3
recurrent - lifelong warfarin +/- low dose aspirin, INR 3-4

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32
Q

overview of antisynthetase syndrome

A

aused by autoantibodies against aminoacyl-tRNA synthetase e.g. anti-Jo1.

It is characterised by
myositis
interstitial lung disease
thickened and cracked skin of the hands (mechanic’s hands)
Raynaud’s phenomenon

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33
Q

moa and S/E of azathioprine

A

inhibits purine synthesis
TPMT test before commencing drug

Adverse effects include
bone marrow depression
consider a full blood count if infection/bleeding occurs
nausea/vomiting
pancreatitis
increased risk of non-melanoma skin cancer

C/I with allopurinol - use lower doses

safe in pregnancy

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34
Q

definition of behcet’s syndrome

A

autoimmune inflammation of arteries and veins
more common in men, 20-40y

triad of sx - oral ulcers, genital ulcers and anterior uveitis
assx HLA B51

clinical diagnosis

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35
Q

features of behcet’s syndrome

A

1) oral ulcers 2) genital ulcers 3) anterior uveitis

thrombophlebitis and deep vein thrombosis
arthritis
neurological involvement (e.g. aseptic meningitis)
GI: abdo pain, diarrhoea, colitis
erythema nodosum

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36
Q

use and moa of bisphosphonates

A

inhibits osteoclasts by reducing recruitment and promoting apoptosis

Clinical uses
prevention and treatment of osteoporosis
hypercalcaemia
Paget’s disease
pain from bone metatases

correct hypocalcaemia/VD before giving bisphosphonates

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37
Q

adverse effects of bisphosphonates

A

oesophageal reactions: oesophagitis, oesophageal ulcers

osteonecrosis of the jaw

increased risk of atypical stress fracture

acute phase response - fever, myalgia and arthralgia

hypocalcaemia - due to reduced calcium efflux from bone

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38
Q

lab values of osteoporosis

A

calcium
phosphate
ALP
PTH

all normal

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39
Q

lab values of osteomalacia

A

calcium -
phosphate -
ALP +
PTH +

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40
Q

primary hyperparathyroidism

A

calcium +
phosphate -
ALP +
PTH +

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41
Q

lab values (bone) of chronic kidney disease

A

calcium -
phosphate +
ALP +
PTH +

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42
Q

lab values of paget’s disease

A

calcium
phosphate
ALP +
PTH

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43
Q

lab values of osteopetrosis

A

calcium
phosphate
ALP
PTH
all normal

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44
Q

features of osteochondroma

A

most common benign bone tumour

more in males, usually diagnosed in patients aged < 20 years

cartilage-capped bony projection on the external surface of a bone

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45
Q

features of osteoma

A

benign ‘overgrowth’ of bone, most typically occuring on the skull

associated with Gardner’s syndrome (a variant of familial adenomatous polyposis, FAP)

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46
Q

features of osteosarcoma

A

most common primary malignant bone tumour

affects children and adolescents

occurs most frequently in the metaphyseal region of long bones prior to epiphyseal closure - femur, tibia, humerus

x-ray shows Codman triangle (from periosteal elevation)
‘sunburst’ pattern

assx with Rb gene mutation

other predisposing factors include Paget’s disease of the bone and radiotherapy

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47
Q

features of ewing’s sarcoma

A

small round blue cell tumour in children and adolescents

occurs in pelvis and long bones

causes severe pain

onion skin appearance

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48
Q

features of chondrosarcoma

A

malignant tumour of cartilage
most commonly affects the axial skeleton
more common in middle-age

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49
Q

features of chronic fatigue syndrome

A

sleep problems, such as insomnia, hypersomnia, unrefreshing sleep, a disturbed sleep-wake cycle
muscle and/or joint pains
headaches
painful lymph nodes without enlargement
sore throat
cognitive dysfunction, such as difficulty thinking, inability to concentrate, impairment of short-term memory, and difficulties with word-finding
physical or mental exertion makes symptoms worse
general malaise or ‘flu-like’ symptoms

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50
Q

definition of chronic fatigue syndrome

A

at least 3 months of disabling fatigue affecting mental and physical function more than 50% of the time in the absence of other disease which may explain symptoms
more common in females

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51
Q

managing chronic fatigue syndrome

A

physical activity and exercise - overseen by ME/CFS team

supportive CBT

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52
Q

causes of dactylitis

A

Dactylitis describes the inflammation of a digit (finger or toe).

Causes include:
spondyloarthritis: e.g. Psoriatic and reactive arthritis
sickle-cell disease
other rare causes include tuberculosis, sarcoidosis and syphilis

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53
Q

overview of dermatomyositis

A

inflammatory disorder causing symmetrical proximal muscle weakness and skin lesions

idiopathic / connective tissue disorders / underlying malignancy

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54
Q

features of dermatomyositis

A

skin - photosensitive, macular rash (back and shoulder), periorbital heliotrope rash, gottron’s papules (on extensor surface of fingers), dry and scaly hands

proximal muscle weakness +/- tenderness
Raynaud’s
respiratory muscle weakness
interstitial lung disease: e.g. Fibrosing alveolitis or organising pneumonia
dysphagia, dysphonia

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55
Q

investigation findings in dermatomyositis

A

ANA positive

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56
Q

features of discoid lupus erythematosus

A

benign autoimmune disorder of younger females - rarely progresses to SLE

follicular keratin plugs
erythematous rash, scaly - on face, neck, ears, scalp
photosensitive
lesions heal with atrophy, scarring, pigmentation

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57
Q

management of discoid lupus erythematosus

A

topical steroids
oral anti malarials
avoid sun exposure

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58
Q

features of drug induced lupus

A

arthralgia
myalgia
skin (e.g. malar rash) and pulmonary involvement (e.g. pleurisy) are common

ANA positive
anti-histone antibodies are found in 80-90%
anti-Ro, anti-Smith positive in around 5%

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59
Q

causes of drug induced lupus

A

procainamide
hydralazine

less common
isoniazid
minocycline
phenytoin

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60
Q

features of ehler-danlos

A

autosomal dominant connective tissue disorder of type III collagen

tissue is more elastic – joint hypermobility and increased skin elasticity (fragile)
recurrent dislocation
easy bruising
aortic regurg, mitral valve prolapse, aortic dissection
subarachnoid haemorrhage

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61
Q

features of fibromyalgia

A

widespread pain with tender points at specific anatomical sites

chronic pain: at multiple site, sometimes ‘pain all over’
lethargy
cognitive impairment: ‘fibro fog’
sleep disturbance, headaches, dizziness are common

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61
Q

management of fibromyalgia

A

explanation
aerobic exercise: has the strongest evidence base
cognitive behavioural therapy
medication: pregabalin, duloxetine, amitriptyline

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62
Q

features of gout

A

flares - few days, sx free between episodes

pain, swelling erythema

affects 1st MTP, ankle, wrist, knee

can cause joint damage and chronic problems

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63
Q

investigation findings of gout

A

synovial fluid analysis
needle shaped negatively birefringent monosodium urate crystals under polarised light

uric acid - check >2w when acute episode has settled - may vary during attack

radiological features
joint effusion
punched out erosions with sclerotic margins
joint space preserved
no periarticular osteopenia
soft tissue tophi

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64
Q

management of acute gout

A

NSAIDs, colchicine
prescribe max NSAID dose until 1-2days after sx settle
PPI
colchicine - slower onset of action, use with caution if renal impairment - may cause diarrhoea

oral steroids if ^ C/I - 15mg/day prednisolone

intraarticular steroid injection

if already on allopurinol - continue

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65
Q

indications for urate lowering therapy

A

indicated after first attack

allopurinol - 100mg od, titrate dose every few weeks

febuxostat - if allopurinol is not tolerated

if refractory - uricase

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66
Q

conservative management of gout

A

reduce alcohol intake and avoid during an acute attack
lose weight if obese
avoid food high in purines
stop precipitants e.g., thiaxides

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67
Q

predisposing factors for gout

A

microcrystal synovitis caused by the deposition of monosodium urate monohydrate in the synovium due to chronic hyperuricaemia - uric acid >0.45mmol/l

reduced uric acid excretion - diuretics, CKD, lead toxicity

increased uric acid production - myeloproliferative disorder, cytotoxic drugs, severe psoriasis

lesch-nyhan syndrome -

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68
Q

causes of hip pain in adults

A

osteoarthritis
inflammatory arthritis
referred lumbar spine pain
greater trochanteric pain syndrome
meralgia paraesthetica
avascular necrosis
pubic symphysis dysfunction
transient idiopathic osteoporosis c

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69
Q

HLA-B51

A

Behcet’s disease

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70
Q

HLA-B27

A

ankylosing spondylitis
reactive arthritis
acute anterior uveitis
psoriatic arthritis

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71
Q

HLA-DQ2/DQ8

A

coeliac disease

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72
Q

HLA-DR2

A

narcolepsy
Goodpasture’s

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73
Q

HLA-DR3

A

dermatitis herpetiformis
Sjogren’s syndrome
primary biliary cirrhosis

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74
Q

HLA DR4

A

type 1 diabetes mellitus*
rheumatoid arthritis - in particular the DRB1 gene (DRB104:01 and DRB104:04 hence the association with DR4)

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75
Q

action and S/E of hydroxychloroquine

A

mx rheumatoid arthritis and sysemic/discoid lupus erythematous

S/E - bulls eye retinopathy - severe and permanent visual loss

76
Q

mechanism and example of type 1 hypersensitivity

A

anaphylactic - antigen reacts with IgE bound to mast cells

e.g., anaphylaxis, atopy

77
Q

mechanism and example of type 2 hypersensitivity

A

IgM or IgG binds to antigen

e.g., Autoimmune haemolytic anaemia
* ITP
* Goodpasture’s syndrome
* Pernicious anaemia
* Acute haemolytic transfusion reactions
* Rheumatic fever

78
Q

mechanism and example of type 3 hypersensitivity

A

immune complex mediated - free antigen and antibody combine

e.g., serum sickness, SLE, post-streptococcal glomerulonephritis

79
Q

mechanism and example of type 4 hypersensitivity

A

T cell mediated

  • Tuberculosis / tuberculin skin reaction
  • Graft versus host disease
  • Allergic contact dermatitis
  • Scabies
  • Extrinsic allergic alveolitis (especially chronic phase)
  • Multiple sclerosis
  • Guillain-Barre syndrome
80
Q

mechanism and example of type 5 hypersensitivity

A

Antibodies that recognise and bind to the cell surface receptors.

This either stimulating them or blocking ligand binding

graves disease, myasthenia gravis

81
Q

overview of IgG

A

monomer

  • Enhance phagocytosis of bacteria and viruses
  • Fixes complement and passes to the fetal circulation
  • Most abundant isotype in blood serum
82
Q

overview of IgM

A
  • First immunoglobulins to be secreted in response to an infection
  • Fixes complement but does not pass to the fetal circulation
  • Anti-A, B blood antibodies (note how they cannot pass to the fetal circulation, which could of course result in haemolysis)
    Pentamer when secreted
83
Q

overview of interferons

A

cytokines released by the body in response to viral infections and neoplasia

classified according to cellular origin and the type of receptor they bind to

84
Q

features of langerhans cell histiocytosis

A

rare condition associated with the abnormal proliferation of histiocytes
presents in childhood with bony lesions

Features
bone pain, typically in the skull or proximal femur
cutaneous nodules
recurrent otitis media/mastoiditis
tennis racket-shaped Birbeck granules on electromicroscopy

84
Q

features of lateral epicondylitis

A

‘tennis elbow’

pain and tenderness of lateral epicondyle
pain worsens on wrist extension against resistance and supination of forearm with elbow extended

episodes last 6m-2y
acute pain for 6-12w

85
Q

management of lateral epicondylitis

A

advice on avoiding muscle overload
simple analgesia
steroid injection
physiotherapy

86
Q

features of marfan’s syndrome

A

AD connective tissue disorder due to defective fibrillin-1

tall stature with arm span to height ratio > 1.05
high-arched palate
arachnodactyly
pectus excavatum
pes planus
scoliosis of > 20 degrees
dilation of aortic sinus - aortic aneurysm, aortic dissection, aortic regurgitation
lungs - repeated pneumothoraces
eyes - upwards lens dislocation, blue sclera, myopia

87
Q

management of marfan’s syndrome

A

regular echo monitoring
beta-blocker/ACEi therapy

88
Q

features of McArdle’s disease

A

autosomal recessive type V glycogen storage disease - causes decreased muscle glycogenolysis

Features
muscle pain and stiffness following exercise
muscle cramps
myoglobinuria
low lactate levels during exercise

89
Q

action and indications of methotrexate

A

antimetabolite that inhibits dihydrofolate reductase, preventing purine/pyrimidine synthesis
taken weekly

Indications
inflammatory arthritis, especially rheumatoid arthritis
psoriasis
some chemotherapy acute lymphoblastic leukaemia

90
Q

adverse effects of methotrexate

A

mucositis
myelosuppression
pneumonitis
pulmonary fibrosis
liver fibrosis

FBC, U&E and LFTs - baseline, then weekly until stabilised then 2-3m

co-prescribe folate

avoid for 6m before pregnancy (men and women)
avoid concurrent trimethoprim and high dose aspirin

91
Q

features of myopathy

A

symmetrical muscle weakness (proximal > distal)
common problems are rising from chair or getting out of bath
sensation normal, reflexes normal, no fasciculation

91
Q

causes of myopathies

A

inflammatory: polymyositis
inherited: Duchenne/Becker muscular dystrophy, myotonic dystrophy
endocrine: Cushing’s, thyrotoxicosis
alcohol

92
Q

management of osteoarthritis

A

weight loss
muscle strengthening exercise
topical NSAIDs
oral NSAIDs + PPI (avoid if taking aspirin)
walking aids
intra-articular steroid injections

93
Q

features of osteoarthritis

A

M=F, older age

affects large weight bearing joints, carpometacarpal joint, DIP, PIP

pain following use, improves with rest, unilateral sx, no systemic upset

94
Q

X ray features of osteoarthritis

A

aetiology
localised loss of cartilage
remodelling of adjacent bone
associated inflammation

Loss of joint space
Subchondral sclerosis
Subchondral cysts
Osteophytes forming at joint margins

95
Q

features of osteogenesis imperfecta

A

autosomal dominant disorder of type 1 collagen metabolism - leads to bone fragility and fractures

presents in childhood
fractures following minor trauma
blue sclera
deafness secondary to otosclerosis
dental imperfections are common

normal biochemistry

96
Q
A
96
Q

causes of osteomalacia

A

softening of the bones secondary to low vitamin D levels that in turn lead to decreased bone mineral content

vitamin D deficiency (malabsorption, lack of sunlight, diet, chronic kidney disease)
drug induced e.g. anticonvulsants
inherited: hypophosphatemic rickets (previously called vitamin D-resistant rickets)
liver disease: e.g. cirrhosis
coeliac disease

97
Q

features of osteomalacia

A

bone pain
bone/muscle tenderness
fractures: especially femoral neck
proximal myopathy: may lead to a waddling gait

98
Q

investigation findings of osteomalacia

A

low VD
low calcium
low phosphate
high ALP

X ray - translucent bands - looser’s zones, pseudofractures

99
Q

treatment of osteomalacia

A

vitamin D loading dose then maintenance

calcium supplementation if diet inadequate.

100
Q

assessing patients post-fragility fracture

A

if >75y - assume osteoporosis, start oral bisphosphonate without need for DEXA scan

if <75y - DEXA scan then FRAX score

101
Q

managing patients with long term corticosteroid use

A
  1. Patients over the age of 65 years or those who’ve previously had a fragility fracture should be offered bone protection.
  2. Patients under the age of 65 years should be offered a bone density scan, with further management dependent:

T score Management
Greater than 0 Reassure
Between 0 and -1.5 Repeat bone density scan in 1-3 years
Less than -1.5 Offer bone protection

102
Q

managing osteoporosis

A

offer vitamin D and calcium supplementation to all women

bisphosphonates - alendronate, risendronate

if not alendronate
raloxifene - SERM - prevents bone loss
strontium ranelate
denosumab
teriparatide
HRT

103
Q

risk factors for osteoporosis

A

corticosteroid use
smoking
alcohol
low body mass index
family history

increases the risk of fragility

104
Q

assessing for osteoporosis

A

FRAX or QFracture to assess the 10-year risk of a patient developing a fragility fracture

assess patients for osteoporosis following fragility fractures

DEXA of hip and lumbar spine

105
Q

interpreting DEXA scan results

A

T score: based on bone mass of young reference population
T score of -1.0 means bone mass of one standard deviation below that of young reference population
Z score is adjusted for age, gender and ethnic factors

> -1.0 = normal
-1.0 to -2.5 = osteopaenia
< -2.5 = osteoporosis

106
Q

overview of paget’s disease

A

increased but uncontrolled bone turnover
primarily disorder of osteoclasts - excess osteoclastic resorption followed by increased osteoblastic activity

The skull, spine/pelvis, and long bones of the lower extremities are most commonly affected.

107
Q

features of paget’s disease

A

only 5% of patients are symptomatic

the stereotypical presentation is an older male with bone pain and an isolated raised ALP
bone pain (e.g. pelvis, lumbar spine, femur)
classical, untreated features: bowing of tibia, bossing of skull

108
Q

investigation findings in paget’s

A

ALP++
normal calcium and phosphate
possibly hypercalcaemia if prolonged immobilisation

XR - osteolysis (early disease) – mixed lytic/sclerotic lesions later
skull XR - thickened vault, osteoporosis circumscripta

bone scintigraphy - focally increased uptake at active bone lesions

109
Q

management of paget’s

A

indications for treatment include:
bone pain
skull or long bone deformity
fracture
periarticular Paget’s

give bisphosphonate

110
Q

overview of polyarteritis nodosa

A

vasculitis affecting medium sized arteries with necrotising inflammation leading to aneurysm formation

occurs in middle aged men
assx with hepatitis b

111
Q

features of polyarteritis nodosa

A

fever, malaise, arthralgia
weight loss
hypertension
mononeuritis multiplex, sensorimotor polyneuropathy
testicular pain
livedo reticularis
haematuria, renal failure

pANCA + in 20%
hep B + in 30%

112
Q

features of polymyalgia rheumatica

A

> 60y
muscle stiffness (morning, proximal limb muscles) and raised inflammatory markers
lethargy, depression, low grade fever
no weakness

assx temporal arteritis
rapid onset <1m

112
Q

investigation findings in polymyalgia rheumatica

A

raised inflammatory markers - ESR.40
CK/EMG normal

113
Q

management of polymyalgia rheumatica

A

prednisolone 15mg OD

should have dramatic response

114
Q

overview of polymyositis

A

inflammatory disorder - symmetrical proximal muscle weakness
T cell mediated cytotoxicity towards muscle fibres

assx malignancy

affects middle aged F 3:1

115
Q

features of polymyositis

A

proximal muscle weakness +/- tenderness
Raynaud’s
respiratory muscle weakness
interstitial lung disease
dysphagia, dysphonia

116
Q

investigation findings of polymyositis

A

CK++
LDH, AST, ALT++
EMG
muscle biopsy
antisynthetase antibodies - anti Jo

117
Q

management of polymyositis

A

high dose corticosteroids
azathioprone - steroid sparing

118
Q

overview of pseudogout

A

microcrystal synovitis due to deposition of calcium pyrophosphate dihydrate crystals in synovium

++ with age

risk factors - haemochromatosis
hyperparathyroidism
low magnesium, low phosphate
acromegaly, Wilson’s disease

119
Q

features of pseudogout

A

knee, wrist and shoulders most commonly affected
joint aspiration: weakly-positively birefringent rhomboid-shaped crystals

x-ray: chondrocalcinosis
in the knee this can be seen as linear calcifications of the meniscus and articular cartilage

120
Q

management of pseudogout

A

aspiration of joint fluid, to exclude septic arthritis
NSAIDs or intra-articular, intra-muscular or oral steroids as for gout

121
Q

overview of psoriatic arthropathy

A

inflammatory arthritis associated with psoriasis - seronegative spondyloarthropathies

poor correlation with cutaneous psoriasis, precedes skin lesions

122
Q

features of psoriatic arthropathy

A

symmetric polyarthritis - v similar to rheumatoid
asymmetrical oligoarthritis - affects hands and feet
sacroilitis
DIP joint disease
arthritis mutilans
psoriatic skin lesions
periarticular disease - tenosynovitis, soft tissue inflammation
nail changes - pitting, onycholysis

123
Q

investigation findings of psoriatic arthropathy

A

X ray = erosive changes + new bone formation, periostitis, pencil in cup

124
Q

management of psoriatic arthropathy

A

manage with rheumatologist

NSAID - if mild
+ methotrexate
mAb - ustekinumab

better prognosis than RA

125
Q

overview of Raynaud’s phenomenon

A

exaggerated vasoconstrictive response of the digital arteries and cutaneous arteriole to the cold or emotional stress

primary - raynaud’s disease
secondary - raynaud’s phenomenon

primary - usually presents in young women
bilateral symptoms

126
Q

secondary causes of raynauds phenomenon

A

connective tissue disorders (scleroderma (most common), rheumatoid arthritis, systemic lupus erythematosus)

leukaemia
type I cryoglobulinaemia, cold agglutinins
use of vibrating tools
drugs: oral contraceptive pill, ergot
cervical rib

127
Q

management of raynaud’s phenomenon

A

refer to secondary care

first line - CCB nifedipine

IV prostacyclin infusion

128
Q

main features of reactive arthritis

A

HLA B27 assx seronegative spondyloarthropathy

urethritis
conjunctivitis
arthritis

following STI (in men)
following dysentry (both sexes)

129
Q

organisms associated with reactive arthritis

A

Shigella flexneri
Salmonella typhimurium
Salmonella enteritidis
Yersinia enterocolitica
Campylobacter

130
Q

management of reactive arthritis

A

sx treatment - analgesia, SAIDs, intraarticular steroids
sulfasalazine, methotrexate

sx usually <12m

131
Q

features of reactive arthritis

A

develops <4w of initial infection with sx lasting 4-6m
25% pt - recurrent

arthritis - asymmetrical oligoarthritis lower limbs

dactylitis

urethritis

conjunctivititis, anterior urethritis

circinate balanitis
keratoderma blenorrhagica

132
Q

extraarticular features of rheumatoid arthritis

A

respiratory - pulmonary firosis, pleural effusion, pulm nodules, bronchiolitis obliterans

ocular - keratoconjunctivitis sicca, episcleritis, corneal ulceration, kerastitis

osteoporosis
ischaemic heart disease

depression

133
Q

elements contributing to rheumatoid diagnosis

A

joint involvememt

serology

acute phase reactants (crp/esr)

duration of sx>6w

134
Q

side effects of methotrexate

A

Myelosuppression
Liver cirrhosis
Pneumonitis

135
Q

side effects of hydroxychloroquine

A

Retinopathy
Corneal deposits

135
Q

side effects of sulfasalazine

A

Rashes
Oligospermia
Heinz body anaemia
Interstitial lung disease

136
Q

side effects of prednisolone

A

Cushingoid features
Osteoporosis
Impaired glucose tolerance
Hypertension
Cataracts

137
Q

management of rheumtoid arthritis

A

if evidence of joint inflammation - DMARD monotherapy +/- short course prednisolone

usually methotrexate with FBC + LFT monitoring

monitor response with CRP and disease activity, DAS28

manage flares with corticosteroid

138
Q

further management of rheumatoid arthritis

A

~TNF inhibitor if inadequate response to 2 DMARDs

etanercept, infliximab, adalimumab, rituximab, abatacept

138
Q

investigation findings in rheumatoid arhtritis

A

IgM RF - positive in 70-80%

anti CCP - higher specificity

XR - hands and feet

138
Q

typical presentation of rheumatoid arthritis

A

swollen painful joints in hands and feet
symmetrical
stiffness worse in morning
large joiints become involved
insidious onset
positive MCP squeeze

139
Q

XR findings in rheumatoid arthritis

A

loss of joint space
juxta-articular osteoporosis
soft-tissue swelling
periarticular erosions
subluxation

139
Q

poor prognostic factors of rheumatoid arthritis

A

rheumatoid factor positive
anti-CCP antibodies
poor functional status at presentation
X-ray: early erosions (e.g. after < 2 years)
extra articular features e.g. nodules
HLA DR4
insidious onset

139
Q

pathophysiology of septic arthritis

A

staph aureus
young, sexually active adults - neisseria gonorrhoea (disseminated gonococcal infection)

most common cause - haematogenous spread - e.g., due to distant bacterial infections

most commonly affects the knee

139
Q

function of rotator cuff muscles

A

supraspinatus - abducts arm before deltoid

infraspinatus - rotates arm laterally

teres minor - adducts and rotates arm laterally

subscapularis -adduct and rotates arm medially c

139
Q

features of septic arthritis

A

acute swollen joint
restricted movememt
warm to touch, fluctuant
fever

synovial fluid sampling prior to abx
blood culture
joint imaging - septic joint can destroy joint in <24h

139
Q

examples of seronegative spondyloarthropathies

A

ankylosing spondylitis
psoriatic arthritis
reactive arthritis
enteropathic arthritis (associated with IBD)

139
Q

management of septic arthritis

A

IV abx covering gram positive cocci - flucloxacillin/clindamycin
switch to oral after 2w

needle aspiration to decompress the joint
arthroscopic lavage required

140
Q

common features of seronegative spondyloarthropathies

A

associated with HLA-B27
rheumatoid factor negative - hence ‘seronegative’
peripheral arthritis, usually asymmetrical
sacroiliitis
enthesopathy: e.g. Achilles tendonitis, plantar fasciitis

extra-articular manifestations: uveitis, pulmonary fibrosis (upper zone), amyloidosis, aortic regurgitation

140
Q

features of sjogren’s syndrome

A

dry eyes: keratoconjunctivitis sicca
dry mouth
vaginal dryness
arthralgia
Raynaud’s, myalgia
sensory polyneuropathy
recurrent episodes of parotitis
renal tubular acidosis (usually subclinical)

140
Q

antibodies in sjogren;s and other investigation findings

A

rheumatoid factor (RF) positive in nearly 50% of patients
ANA positive in 70%
anti-Ro (SSA) antibodies in 70% of patients with PSS
anti-La (SSB) antibodies in 30% of patients with PSS

Schirmer’s test: filter paper near conjunctival sac to measure tear formation
histology: focal lymphocytic infiltration
also: hypergammaglobulinaemia, low C4

140
Q

pathophysiology of sjogren’s syndrome

A

AI disorder affecting exocrine glands causing dry mucosal surfaces

primary or secondary to RhA

more common in feemales
increased risk of lymphoid malignancy

140
Q

management of sjogren’s syndrome

A

artificial saliva and tears
pilocarpine may stimulate saliva production

140
Q

features of still’s disease

A

arthralgia
elevated serum ferritin
rash: salmon-pink, maculopapular
pyrexia
typically rises in the late afternoon/early evening in a daily pattern and accompanies a worsening of joint symptoms and rash
lymphadenopathy

rheumatoid factor (RF) and anti-nuclear antibody (ANA) negative

140
Q

pathophysiology of systemic lupus erythematosus

A

F >M, afrocaribbeans/asians
AI disease - type 3 hypersensitivity reaction

assx - HLAB8, DR2, DR3

immune system dysregulation leading to immune complex formation - can affect any organ (skin, joints, kidneys, brain)

140
Q

features of systemic lupus erythematosus

A

fatigue
fever
mouth ulcers
lymphadenopathy

skin - malar rash sparing nasolabial folds
discoid rash - scaly, erythematous, well demarcated rash in sun-exposed areas
photosensitivity
raynaud’s phenomenon
livedo reticularis
non scarring alopecia

arthralgia, arthritis

pericarditis*, myocarditis

pleurisy, fibrosing alveolitis

proteinuria, glomerulonephritis

neuropsychiatric

140
Q

managing still’s disease

A

NSAIDs
steroids

if persistent - consider methotrexate

140
Q

investigation findings in systemic lupus erythematosus

A

ANA positive - high sensitivity, low specificity
RhF + 20%
anti dsDNA - highly specific
anti-smith -highly specific

ESR
CRP+ indicates underlying infection (may otherwise be normal)

complement C3C4 low in active disease (formation of complexes = consumption of complement)

141
Q

management in systemic lupus erythematosus

A

NSAIDs, sun block

hydroxychloroquine

141
Q

features of limited cutaneous systemic sclerosis

A

raynaud’s
scleroderma of face and distal limbs

assx - anticentromere antibodies, ANA+ 90%

e.g., CREST - Calcinosis, Raynaud’s phenomenon, oEsophageal dysmotility, Sclerodactyly, Telangiectasia

142
Q

features of diffuse cutaenous systemic sclerosis

A

scleroderma - trunk and proximal limbs

assx anti scl-70, ANA+ 90%

most common cause of death - respiratory involvement - interstitiial lung disease, pulmonary arterial hypertension

143
Q

features of scleroderma

A

no internal organ involvement
tightening and fibrosis of skin
may be manifest as plaques (morphoea) or linear

ANA+ 90%

144
Q
A
144
Q
A
144
Q
A
145
Q
A
145
Q
A
145
Q
A
145
Q
A
145
Q
A
145
Q
A
146
Q
A
147
Q
A
148
Q

features of temporal arteritis

A

presents >60y
rapid onset <1m
headache
jaw claudication
visual loss
tender, palpable temrpoal artery
assx with PMR
lethargy, depression, fever

148
Q

investigation findings in temporal arteritis

A

raised inflammatory markers
ESR > 50 mm/hr (note ESR < 30 in 10% of patients)
CRP may also be elevated

temporal artery biopsy
skip lesions may be present

note creatine kinase and EMG normal

148
Q

ocular features of temporal arteritis

A

anterior ischaemic optic neuropathy - causes majoriy of ocular complications
due to occlusion of posterior cilliary artery - causes ischaemia of optic nerve head

fundoscopy - swollen pale disc and blurrred margins

temporary visual loss - amaurosis fugax
permanent visual loss = most feared complication

148
Q

management of temporal arteritis

A

urgent high dose glucocorticoids - before tmeporal arteyr biopsy
no visual loss - high dose pred
visual loss - IV methyl pred

urgent ophthal review if visual sx

bone protection with bisphosphonates

148
Q
A
148
Q
A
148
Q
A
148
Q
A
148
Q
A
148
Q
A
148
Q
A

Finals (27 decks)

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