Rheumatology Flashcards
large vessel vasculitides
takayasu arteritis
giant cell arteritis
medium vessel vasculitides
polyarteritis nodosa
kawasaki disease
small vessel vasculitides
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
features of giant cell arteritis
most common form of systemic vasculitis - granulomatous arteritis of aorta + major branches, affects extracranial branches of carotid esp temporal artery
assx with polymyalgia rheumatics
pathophysiology of giant cell arteritis
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
symptoms of giant cell arteritis
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
examination findings of giant cell arteritis
tender/thickened/nodular temporal artery
scalp tenderness
proximal muscle weakness
visual loss - ?examine visual acuity - due to anterior ischaemic optic neuropathy
investigation findings in giant cell arteritis
ESR, CRP +++
fundoscopy /slit lamp - refer to ophthal
US temporal artery - halo sign
temporal artery biopsy
FDG PET scan - assesses disease activity
management of giant cell arteritis
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
features of polymyalgia rheumatics
inflammatory condition of elderly women >50y characterised by proximal limb girdle weakness
non specifically unwell
stiffness
fever, weight loss, fatigue
bursitis/synovitis
investigation findings for polymyalgia rheumatica
high ESR/CRP
broad panel of bloods due to vague presentation - myeloma screen, RH, CCP, renal, bone
management of polymyalgia rheumatica
steroids - prednisolone 15mg/day - should respond <48h - reconsider diagnosis if not
PPI and bone protection
definition of takayasu arteritis
granulomatous inflammation of aorta and major branches
subclavian artery - arm claudication
renal artery - hypertension
carotid artery - CNS features
occurs in <40y F, asian
rare
pathophysiology of takayasu
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
features of takayasu arteritis
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
investigation findings of takayasu arteritis
ESR, CRP ++
MRA, CTA - imaging aorta and arterial tree
USS doppler of carotid and subclavian
PET
management of takayasu arteritis
high dose steroid and methotrexate (if steroid sparing agent needed)
steroid sparing immunosuppression long term - cyclophosphamide
biologics
endovascular interventions e.g., stenting
serial MRA
definition of polyarteritis nodosa
necrotising inflammation of medium-sized arteries (GI, renal, coronary)
assx hepatitis B
occurs in males 60s
investigation of polyarteritis nodosa
urinalysis
uPCR
bloods - raised inf markers
hep serology
skin biopsy
nerve conduction studies
angiography
seronegative
management of polyarteritis nodosa
mild - oral steroids
systemic - IV steroids, IV cyclophosphamide
if HBV + - treat with antiviral treatment
what are the ANCA associated vasculitides
small vessel vasculitides
granulomatosis with polyangiitis (Wegeners)
eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
microscopic polyangiitis
common features in ANCA associated vasculitides
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
the two types of ANCA
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
features of ankylosing spondylitis
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)
clinical examination findings in ankylosing spondylitis
reduced lateral flexion
reduced forward flexion - schober’s test
reduced chest expansion
investigation findings in ankylosing spondylitis
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
management of ankylosing spondylitis
regular exercise
NSAIDs
physiotherapy
DMARDs - useful if peripheral joint involvement
anti TNF if refractory high disease activity
definition of antiphospholipid syndrome
acquired disorder - predisposition to venous and arterial thrombosis, foetal loss, thrombocytopenia
may be primary disorder or secondary (e.g., SLE)
features of antiphospholipid syndrome
venous/arterial thrombosis
recurrent miscarriages
livedo reticularis
other features: pre-eclampsia, pulmonary hypertension
investigation findings in antiphospholipid syndrome
antibodies
anticardiolipin antibodies
anti-beta2 glycoprotein I (anti-beta2GPI) antibodies
lupus anticoagulant
thrombocytopenia
prolonged APTT
management of antiphospholipid syndrome
primary thromboprophylaxis - low dose aspirin
secondary thromboprophylaxis
after initial event - lifelong warfarin INR 2-3
recurrent - lifelong warfarin +/- low dose aspirin, INR 3-4
overview of antisynthetase syndrome
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
moa and S/E of azathioprine
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
definition of behcet’s syndrome
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
features of behcet’s syndrome
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
use and moa of bisphosphonates
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
adverse effects of bisphosphonates
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
lab values of osteoporosis
calcium
phosphate
ALP
PTH
all normal
lab values of osteomalacia
calcium -
phosphate -
ALP +
PTH +
primary hyperparathyroidism
calcium +
phosphate -
ALP +
PTH +
lab values (bone) of chronic kidney disease
calcium -
phosphate +
ALP +
PTH +
lab values of paget’s disease
calcium
phosphate
ALP +
PTH
lab values of osteopetrosis
calcium
phosphate
ALP
PTH
all normal
features of osteochondroma
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
features of osteoma
benign ‘overgrowth’ of bone, most typically occuring on the skull
associated with Gardner’s syndrome (a variant of familial adenomatous polyposis, FAP)
features of osteosarcoma
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
features of ewing’s sarcoma
small round blue cell tumour in children and adolescents
occurs in pelvis and long bones
causes severe pain
onion skin appearance
features of chondrosarcoma
malignant tumour of cartilage
most commonly affects the axial skeleton
more common in middle-age
features of chronic fatigue syndrome
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
definition of chronic fatigue syndrome
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
managing chronic fatigue syndrome
physical activity and exercise - overseen by ME/CFS team
supportive CBT
causes of dactylitis
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
overview of dermatomyositis
inflammatory disorder causing symmetrical proximal muscle weakness and skin lesions
idiopathic / connective tissue disorders / underlying malignancy
features of dermatomyositis
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
investigation findings in dermatomyositis
ANA positive
features of discoid lupus erythematosus
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
management of discoid lupus erythematosus
topical steroids
oral anti malarials
avoid sun exposure
features of drug induced lupus
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%
causes of drug induced lupus
procainamide
hydralazine
less common
isoniazid
minocycline
phenytoin
features of ehler-danlos
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
features of fibromyalgia
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
management of fibromyalgia
explanation
aerobic exercise: has the strongest evidence base
cognitive behavioural therapy
medication: pregabalin, duloxetine, amitriptyline
features of gout
flares - few days, sx free between episodes
pain, swelling erythema
affects 1st MTP, ankle, wrist, knee
can cause joint damage and chronic problems
investigation findings of gout
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
management of acute gout
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
indications for urate lowering therapy
indicated after first attack
allopurinol - 100mg od, titrate dose every few weeks
febuxostat - if allopurinol is not tolerated
if refractory - uricase
conservative management of gout
reduce alcohol intake and avoid during an acute attack
lose weight if obese
avoid food high in purines
stop precipitants e.g., thiaxides
predisposing factors for gout
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 -
causes of hip pain in adults
osteoarthritis
inflammatory arthritis
referred lumbar spine pain
greater trochanteric pain syndrome
meralgia paraesthetica
avascular necrosis
pubic symphysis dysfunction
transient idiopathic osteoporosis c
HLA-B51
Behcet’s disease
HLA-B27
ankylosing spondylitis
reactive arthritis
acute anterior uveitis
psoriatic arthritis
HLA-DQ2/DQ8
coeliac disease
HLA-DR2
narcolepsy
Goodpasture’s
HLA-DR3
dermatitis herpetiformis
Sjogren’s syndrome
primary biliary cirrhosis
HLA DR4
type 1 diabetes mellitus*
rheumatoid arthritis - in particular the DRB1 gene (DRB104:01 and DRB104:04 hence the association with DR4)
action and S/E of hydroxychloroquine
mx rheumatoid arthritis and sysemic/discoid lupus erythematous
S/E - bulls eye retinopathy - severe and permanent visual loss
mechanism and example of type 1 hypersensitivity
anaphylactic - antigen reacts with IgE bound to mast cells
e.g., anaphylaxis, atopy
mechanism and example of type 2 hypersensitivity
IgM or IgG binds to antigen
e.g., Autoimmune haemolytic anaemia
* ITP
* Goodpasture’s syndrome
* Pernicious anaemia
* Acute haemolytic transfusion reactions
* Rheumatic fever
mechanism and example of type 3 hypersensitivity
immune complex mediated - free antigen and antibody combine
e.g., serum sickness, SLE, post-streptococcal glomerulonephritis
mechanism and example of type 4 hypersensitivity
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
mechanism and example of type 5 hypersensitivity
Antibodies that recognise and bind to the cell surface receptors.
This either stimulating them or blocking ligand binding
graves disease, myasthenia gravis
overview of IgG
monomer
- Enhance phagocytosis of bacteria and viruses
- Fixes complement and passes to the fetal circulation
- Most abundant isotype in blood serum
overview of IgM
- 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
overview of interferons
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
features of langerhans cell histiocytosis
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
features of lateral epicondylitis
‘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
management of lateral epicondylitis
advice on avoiding muscle overload
simple analgesia
steroid injection
physiotherapy
features of marfan’s syndrome
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
management of marfan’s syndrome
regular echo monitoring
beta-blocker/ACEi therapy
features of McArdle’s disease
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
action and indications of methotrexate
antimetabolite that inhibits dihydrofolate reductase, preventing purine/pyrimidine synthesis
taken weekly
Indications
inflammatory arthritis, especially rheumatoid arthritis
psoriasis
some chemotherapy acute lymphoblastic leukaemia
adverse effects of methotrexate
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
features of myopathy
symmetrical muscle weakness (proximal > distal)
common problems are rising from chair or getting out of bath
sensation normal, reflexes normal, no fasciculation
causes of myopathies
inflammatory: polymyositis
inherited: Duchenne/Becker muscular dystrophy, myotonic dystrophy
endocrine: Cushing’s, thyrotoxicosis
alcohol
management of osteoarthritis
weight loss
muscle strengthening exercise
topical NSAIDs
oral NSAIDs + PPI (avoid if taking aspirin)
walking aids
intra-articular steroid injections
features of osteoarthritis
M=F, older age
affects large weight bearing joints, carpometacarpal joint, DIP, PIP
pain following use, improves with rest, unilateral sx, no systemic upset
X ray features of osteoarthritis
aetiology
localised loss of cartilage
remodelling of adjacent bone
associated inflammation
Loss of joint space
Subchondral sclerosis
Subchondral cysts
Osteophytes forming at joint margins
features of osteogenesis imperfecta
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
causes of osteomalacia
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
features of osteomalacia
bone pain
bone/muscle tenderness
fractures: especially femoral neck
proximal myopathy: may lead to a waddling gait
investigation findings of osteomalacia
low VD
low calcium
low phosphate
high ALP
X ray - translucent bands - looser’s zones, pseudofractures
treatment of osteomalacia
vitamin D loading dose then maintenance
calcium supplementation if diet inadequate.
assessing patients post-fragility fracture
if >75y - assume osteoporosis, start oral bisphosphonate without need for DEXA scan
if <75y - DEXA scan then FRAX score
managing patients with long term corticosteroid use
- Patients over the age of 65 years or those who’ve previously had a fragility fracture should be offered bone protection.
- 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
managing osteoporosis
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
risk factors for osteoporosis
corticosteroid use
smoking
alcohol
low body mass index
family history
increases the risk of fragility
assessing for osteoporosis
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
interpreting DEXA scan results
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
overview of paget’s disease
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.
features of paget’s disease
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
investigation findings in paget’s
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
management of paget’s
indications for treatment include:
bone pain
skull or long bone deformity
fracture
periarticular Paget’s
give bisphosphonate
overview of polyarteritis nodosa
vasculitis affecting medium sized arteries with necrotising inflammation leading to aneurysm formation
occurs in middle aged men
assx with hepatitis b
features of polyarteritis nodosa
fever, malaise, arthralgia
weight loss
hypertension
mononeuritis multiplex, sensorimotor polyneuropathy
testicular pain
livedo reticularis
haematuria, renal failure
pANCA + in 20%
hep B + in 30%
features of polymyalgia rheumatica
> 60y
muscle stiffness (morning, proximal limb muscles) and raised inflammatory markers
lethargy, depression, low grade fever
no weakness
assx temporal arteritis
rapid onset <1m
investigation findings in polymyalgia rheumatica
raised inflammatory markers - ESR.40
CK/EMG normal
management of polymyalgia rheumatica
prednisolone 15mg OD
should have dramatic response
overview of polymyositis
inflammatory disorder - symmetrical proximal muscle weakness
T cell mediated cytotoxicity towards muscle fibres
assx malignancy
affects middle aged F 3:1
features of polymyositis
proximal muscle weakness +/- tenderness
Raynaud’s
respiratory muscle weakness
interstitial lung disease
dysphagia, dysphonia
investigation findings of polymyositis
CK++
LDH, AST, ALT++
EMG
muscle biopsy
antisynthetase antibodies - anti Jo
management of polymyositis
high dose corticosteroids
azathioprone - steroid sparing
overview of pseudogout
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
features of pseudogout
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
management of pseudogout
aspiration of joint fluid, to exclude septic arthritis
NSAIDs or intra-articular, intra-muscular or oral steroids as for gout
overview of psoriatic arthropathy
inflammatory arthritis associated with psoriasis - seronegative spondyloarthropathies
poor correlation with cutaneous psoriasis, precedes skin lesions
features of psoriatic arthropathy
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
investigation findings of psoriatic arthropathy
X ray = erosive changes + new bone formation, periostitis, pencil in cup
management of psoriatic arthropathy
manage with rheumatologist
NSAID - if mild
+ methotrexate
mAb - ustekinumab
better prognosis than RA
overview of Raynaud’s phenomenon
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
secondary causes of raynauds phenomenon
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
management of raynaud’s phenomenon
refer to secondary care
first line - CCB nifedipine
IV prostacyclin infusion
main features of reactive arthritis
HLA B27 assx seronegative spondyloarthropathy
urethritis
conjunctivitis
arthritis
following STI (in men)
following dysentry (both sexes)
organisms associated with reactive arthritis
Shigella flexneri
Salmonella typhimurium
Salmonella enteritidis
Yersinia enterocolitica
Campylobacter
management of reactive arthritis
sx treatment - analgesia, SAIDs, intraarticular steroids
sulfasalazine, methotrexate
sx usually <12m
features of reactive arthritis
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
extraarticular features of rheumatoid arthritis
respiratory - pulmonary firosis, pleural effusion, pulm nodules, bronchiolitis obliterans
ocular - keratoconjunctivitis sicca, episcleritis, corneal ulceration, kerastitis
osteoporosis
ischaemic heart disease
depression
elements contributing to rheumatoid diagnosis
joint involvememt
serology
acute phase reactants (crp/esr)
duration of sx>6w
side effects of methotrexate
Myelosuppression
Liver cirrhosis
Pneumonitis
side effects of hydroxychloroquine
Retinopathy
Corneal deposits
side effects of sulfasalazine
Rashes
Oligospermia
Heinz body anaemia
Interstitial lung disease
side effects of prednisolone
Cushingoid features
Osteoporosis
Impaired glucose tolerance
Hypertension
Cataracts
management of rheumtoid arthritis
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
further management of rheumatoid arthritis
~TNF inhibitor if inadequate response to 2 DMARDs
etanercept, infliximab, adalimumab, rituximab, abatacept
investigation findings in rheumatoid arhtritis
IgM RF - positive in 70-80%
anti CCP - higher specificity
XR - hands and feet
typical presentation of rheumatoid arthritis
swollen painful joints in hands and feet
symmetrical
stiffness worse in morning
large joiints become involved
insidious onset
positive MCP squeeze
XR findings in rheumatoid arthritis
loss of joint space
juxta-articular osteoporosis
soft-tissue swelling
periarticular erosions
subluxation
poor prognostic factors of rheumatoid arthritis
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
pathophysiology of septic arthritis
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
function of rotator cuff muscles
supraspinatus - abducts arm before deltoid
infraspinatus - rotates arm laterally
teres minor - adducts and rotates arm laterally
subscapularis -adduct and rotates arm medially c
features of septic arthritis
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
examples of seronegative spondyloarthropathies
ankylosing spondylitis
psoriatic arthritis
reactive arthritis
enteropathic arthritis (associated with IBD)
management of septic arthritis
IV abx covering gram positive cocci - flucloxacillin/clindamycin
switch to oral after 2w
needle aspiration to decompress the joint
arthroscopic lavage required
common features of seronegative spondyloarthropathies
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
features of sjogren’s syndrome
dry eyes: keratoconjunctivitis sicca
dry mouth
vaginal dryness
arthralgia
Raynaud’s, myalgia
sensory polyneuropathy
recurrent episodes of parotitis
renal tubular acidosis (usually subclinical)
antibodies in sjogren;s and other investigation findings
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
pathophysiology of sjogren’s syndrome
AI disorder affecting exocrine glands causing dry mucosal surfaces
primary or secondary to RhA
more common in feemales
increased risk of lymphoid malignancy
management of sjogren’s syndrome
artificial saliva and tears
pilocarpine may stimulate saliva production
features of still’s disease
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
pathophysiology of systemic lupus erythematosus
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)
features of systemic lupus erythematosus
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
managing still’s disease
NSAIDs
steroids
if persistent - consider methotrexate
investigation findings in systemic lupus erythematosus
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)
management in systemic lupus erythematosus
NSAIDs, sun block
hydroxychloroquine
features of limited cutaneous systemic sclerosis
raynaud’s
scleroderma of face and distal limbs
assx - anticentromere antibodies, ANA+ 90%
e.g., CREST - Calcinosis, Raynaud’s phenomenon, oEsophageal dysmotility, Sclerodactyly, Telangiectasia
features of diffuse cutaenous systemic sclerosis
scleroderma - trunk and proximal limbs
assx anti scl-70, ANA+ 90%
most common cause of death - respiratory involvement - interstitiial lung disease, pulmonary arterial hypertension
features of scleroderma
no internal organ involvement
tightening and fibrosis of skin
may be manifest as plaques (morphoea) or linear
ANA+ 90%
features of temporal arteritis
presents >60y
rapid onset <1m
headache
jaw claudication
visual loss
tender, palpable temrpoal artery
assx with PMR
lethargy, depression, fever
investigation findings in temporal arteritis
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
ocular features of temporal arteritis
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
management of temporal arteritis
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
