rheumatology conditions Flashcards
osteoarthritis
- non-inflammatory condition
- occurs in synovial joints
imbalance between cartilage wearing down + chondrocytes repairing it
osteoarthritis appearance on x-ray
L - loss of joint space
O - osteophytes (bone spurs)
S- subarticular sclerosis (increased density of bone along joint line)
S - subchondral cysts (fluid filled holes in bone)
presentation of osteoarthritis
- joint pain + stiffness that’s worse with activity + @ end of day
- bulky, bony enlargement of joint - Hebredens + bouchards nodes
- restricted range of movements / crepitus
- fluid around joints
what is the difference between heberdens nodes and bouchards nodes?
heberdens nodes = joints of distal finger (DIPs)
bouchards nodes = proximal finger (PIPs)
what is the main clinical difference between rheumatoid arthritis and osteoarthritis?
osteoarthritis - worse at with activity + at end of day, non-inflammatory
rheumatoid - improves with activity + worst after rest, inflammatory
is rheumatoid arthritis more common in men or women?
3 x more commo in women - usually presents middle aged + with family history
genes associated with rheumatoid arthritis
HLA DR1 - occasionally present in rheumatoid arthritis patients
HLA DR4 - often present in rheumatoid FACTOR positive patients
rheumatoid arthritis
autoimmune
inflammatory
chronic inflammation of synovial lining (synovitis) of joints, tendon sheaths + bursa - inflammation increases risk of tendon rupture
usually symmetrical + affects multiple joints = symmetrical polyarthritis
pathophysio of rheumatoid arthritis
rheumatoid factor = autoantibody in 70% of RA patients
–> it targets the Fc portion of the IgG antibody causing activation of immune system against patients own IgG causing systemic inflammation
RF is most commonly IgM but can be any
anti-CCP antibodies are distributed through circulation + form immune complexes with CCP produced in the inflamed synovium
signs of rheumatoid arthritis in hands
- Z shaped thumb
- swan neck = hyperextended PIP + flexed DIP
- boutonnieres = hyperextended DIP + flexed PIP
- ulnar deviation of fingers at the knuckle
extra-articular manifestations of rheumatoid arthritis
- pulmonary fibrosis - caplan’s syndrome
- broncholitis obliterans
- Felty’s syndrome
- lymphadenopathy
- carpel tunnel syndrome
diagnosis of rheumatoid arthritis
clinical if - symmetrical polyarthropathy affecting small joints
check rheumatoid factor + anti-CCP
inflammatory markers - CRP + ESR
management of rheumatoid arthritis
MDT - occupational, physio, podiatry
steroids for flares
NSAIDs/COX-2 inhibitors - prescribe with PPI
1st line = monotherapy of either: methotrexate, leflunomide, sulfasalazine
2nd = 2 of above in combo
3rd = methotrexate + biological therapy (usually TNF inhibitor)
4th = methotrexate + rituximab
hydroxychloroquine = mildest rheumatic drug
does pregnancy improve or worsen symptoms of rheumatoid arthritis?
improves
medication of choice during pregnancy = sulfasalazine / hydroxychloroquine
management of septic arthritis
- empirical IV antibiotics until sensitivities known
- antibiotics for 3-6weeks
1st line = flucloxacillin + rifampicin
penicillin allergy, MRSA or prosthetic joint = vancomycin + rifampicin
alternative = clindamycin
septic arthritis investigations
aspirate joint prior to antibiotics, send for -
- gram staining
- crystal microscopy
- culture
- antibiotic sensitivities
most common causative organism of septic arthritis
staph aureus !
neisseria gonorrhoea (gram neg diplococcus) - sexually active
Group A Strep - strep pyogenes
haemophilus influenza
e. coli
diagnosis of osteoarthritis
no need for investigations if over 45 and has typical symptoms + NO morning stiffness or lasts under 30 mins
what can trigger rheumatoid arthritis?
infections + smoking
scoring used for rheumatoid arthritis
DAS28 score
- disease activity score - useful for monitoring + response to treatment
when are biologics used in the treatment of rheumatoid arthritis?
when 2 DMARDs tried or DAS28 >5.1
side effects of DMARDs
- increased risk of infection
- liver function derrangement
- pneumonitis !
- nausea
methotrexate
- teratogenic - must be stopped at least 3 months prior to conception
- needs regular blood monitoring
anti-TNF side effects
- increased risk of infection
- increased risk of skin cancer
- exacerbation of heart failure
- reactivation of latent TB
what gene is common in seronegative spondylarthritis’s?
HLA B27
common presentations amongst spondyloarthropathies
- enthesitis = inflammation at insertion of tendons into bones
- dactylitis = sausage digits
- anterior uveitis, conjunctivitis
- mucocutaneous lesions
- aortic incompetence or heart block
key joints affected in ankylosing spondylitis
sacroiliac joints + vertebral column
inflammation causes joint pain + stiffness - can progress to fusion of joints (“bamboo spine”)
classic presentation of ankylosing spondylitis
- young male in late teens / 20s
- symptoms develop gradually over 3 months + can flare
- lower back pain + stiffness + sacroiliac pain in buttock region
- – worse with rest + improves with movement
- – worse at night + in morning - may wake from sleep
associations = weight loss, chest pain, enthesitis, dactylitis, pulmonary fibrosis
what is Schobrt’s test and what is it used to diagnose?
used to assess mobility spine
find L5 vertebra - mark point 10cm above + 5cm below
ask patient to bend forward as far as they can + measure distance between 2 points
ankylosing spondylitis - distance <20cm
investigations in ankylosing spondylitis
- Schober’s test
- CRP + ESR - raised
- x-ray of spine + sacrum
- MRI of spine can show bone marrow oedema in early disease pre x-ray changes
ankylosing spondylitis x-ray appearance
bamboo spine
squaring of vertebral bodies
sunchondral sclerosis + erosion
syndesmophytes = areas of bone growth where lligaments insert into bone
ossification of ligaments, discs + joints (they turn into bone)
fusion of facet, sacroiliac + costovertebral joints
treatment of ankylosing spondylitis
- NSAIDs for pain
- steroids during flares
- anti-TNF - etsnercept, infliximab
- secukinumab if above inadequate
- physio, avoid smoking
- bisphosphonates to treat osteoporosis
- surgery sometimes required for deformities to spine / other joints
psoriatic arthritis appearance on x-ray
- periostitis = inflammation of periosteum causing thickened + irregular outline of bone
- ankylosis = bones joining together causing stiffening
- osteolysis
pencil-in-cup appearance - central erosions of bone beside joint
severe form of psoriatic arthritis
arthritis mutilans
- occurs in phalanxes
- osteolysis around joints + digits
-> leads to progressive shortening of digit, skin then folds as the digit shortens giving “telescopic finger”
reactive arthritis
where synovitis occurs in the joints as a reaction to a recent trigger
-> usually 1-4 weeks post infection (nothing can be cultured from synovial fluid)
triggers of reactive arthritis
gastroenteritis (enterogenic) - salmonella, shigella, yersinia
sexually transmitted infection - chlamydia
reactive arthritis presentation
“can’t see, pee or climb a tree”
- bilateral conjunctivitis / anterior uveitis
- circinate balantis = dermatitis of head of penis
- warm, swollen, painful joint - acute monoarthritis affecting single joint of lower limb (knee = commonest)
reactive arthritis management
- NSAIDs
- steroid injections into affected joints (systemic steroids if multiple joints)
- may resolve within 6 months + no recur
- -> recurrent = DMARDs / anti-TNF
septic arthritis presentation
rapid onset of -
- hot, red, swollen, painful
- stiffness + reduced range of movement
- systemic symptoms - fever, lethargy + sepsis
common causative organisms in septic arthritis
most common = staph aureus
- neisseria gonorrhoea (gonococcus, gram neg diplococcus)
- -> in sexually active, exclude in young patients
- Group A Strep - strep pyogenes
- haemophilus influenza
- e. coli
enteropathic arthritis
associated with IBD - symptoms worse during flares
–> watery stool with mucus + blood
risk factors for gout
- high purine diet (meat, seafood), diuretics
- obesity, alcohol, psoriasis
- renalfailure, hypothyroidism
- fam history
rare under 20s, decreases after age 80
more common in men, very rare in premenopausal women
causes of gout
hyperuricaemia
–> hyperuricaemia results in crystallisation encouraged by lower temp (synovial fluid = 32 degrees)
- increased urate production
- reduced urate excretion
typical joints affected in gout
base of big toe (metarsophalangeal)
wrists
base of thumb (carpometacarpal)
gout presentation
- affecting big toe, wrists, base of thumb
- rapid onset severe pain
- tophi - subcutaneous deposits of uric acid
- erythema + warmth
gout investigations
- serum uric acid raised
- polarised microscopy of synovial fluid
- – strongly negative birefringent needle-shaped crystals
- – monosodium urate crystals
- x-ray = punched out erosions with sclerotic borders
treatment of acute attack of gout
ACUTE
- NSAIDs - not in CKD
- colchicine
- steroids
PREVENTATIVE treatment
-> start 2 weeks after acute attack until urate is <360
preventative treatment of gout
start 2 weeks after acute attack until urate <360
- allopurinol (xanthine oxidase inhibitors)
- ->DON’t give in acute, rash in elderly + renal impaired, interacts with azathioprine
- uricosuric drugs - probenecid, sulphinpyrazone
- interleukin-1 inhibitors
lose weight, drink less, eat less meat/seafood
investigations for pseudogout
aspirate synovial fluid
- no bacterial growth
- calcium pyrophosphate crystals
- rhomboid shaped crystals
- positive birefringent of polarised light
treatment of pseudogout
- NSAIDs
- colchicine
- steroids
- rehydration
what minority does SLE commonly affect?
more common in women + asians/afro-caribbean
usually young - middle aged
systemic lupus erythematosus
inflammatory autoimmune connective tissue disorder
effects multiple organs + systems
relapsing-remitting course - result of chronic inflammation
shortened life expectancy
pathophysio of SLE
complexes of antigens + auto-antibodies form + circulate - deposition of immune complexes in basement membrane which activates complement + leucocytes
when immune system is activated by anti-nuclear antibodies it generates an inflammatory response - chronic inflammation causes necrosis + scarring and is what causes symptoms
what part of the pathphysio of SLE drives the disease?
immune complexes of antigens + auto-antibodies in basement membrane which activates complement + leucocytes
presentation of SLE
photosensitive malar rash - butterfly rash weight loss, fatigue, fever joint + muscle pain lymphadenopathy + splenomegaly SOB, pleuritic chest pain mouth ulcers, hair loss, raynauds delirium, psychosis, seizures
SLE investigations
autoantibodies
- ANA - not very specific
- anti-double stranded DNA - specific to SLE
FBC - normocytic anaemia
C3 + C4 levels decreased in active
how do anti-dsDNA levels change with the activity of SLE?
rises with activity
treatment of SLE
NSAIDS
steroids - prednisolone
hydroxychloroquine = first line for mild SLE
suncream/sun avoidance
resistant/severe SLE = methotrexate
–> biologics if unresponsive
Sjogren’s syndrome
autoimmune condition affecting exocrine glands
F:M = 9:1
increased risk of lymphoma
primary or secondary (to SLE or rheumatoid arthritis)
Sjogren’s presentation
dry mucous membranes
- dry mouth
- dry eyes
- dry vagina
Sjogren’s investigations
- schirmer test - paper in eyelid + distance of tears measured after 5 mins
- – normal = 15mm, bad <10mm
- anti-Ro + anti-La
antibodies associated with Sjogren’s syndrome?
Anti-Ro
Anti-La
management of Sjogren’s
hydroxychloroquine to halt disease progression
artificial tears + saliva, vaginal lubricants
complications of Sjogren’s
- eye infections - conjuntivitis, corneal ulcers
- oral problems - dental cavities, thrush
- vaj problems - candidiasis + sexual dysfunction
lymphoma
pneumonia + bronchiectasis
systemic sclerosis presentation
limited cutaneous (face + below elbows + knees only) - CREST C- calcinosis R - raynaud's E - oEsphageal dysmotility S - sclerodactyly T- Telangiectasia
diffuse cutaneous (whole body) - CREST + many internal organs causing -
- cardio probs - hypertension + CAD
- lung probs - fibrosis
- kidney probs - glomerulonephritis, scleroderma renal crisis
systemic sclerosis presentation
limited cutaneous (face + below elbows + knees only) - CREST C- calcinosis R - raynaud's E - oEsphageal dysmotility S - sclerodactyly T- Telangiectasia
diffuse cutaneous (whole body) - CREST + many internal organs causing -
- cardio probs - hypertension + CAD
- lung probs - fibrosis
- kidney probs - glomerulonephritis, scleroderma renal crisis
what is sclerodactyly?
skin changes in hands
-> as skin, restricts normal range of motion of joints - skin can break + ulcerate, patient can’t make fist/straighten hand properly
autoantibodies associated with systemic sclerosis
antinuclear antibodies(ANA) - positive in most patients, not specific
anti-centromere - limited cutaneous
anti-Scl-70 - diffuse cutaneous (more severe)
systemic sclerosis investigations
nailfold capillaroscopy
anti-centromere/anti-Scl-70 autoantibodies
what is nail capillaroscopy and what is it used to investigate?
nailfold is magnified + examined to see peripheral capillaries
abnormal capillaries, avascular area, micro-haemorrhages = systemic sclerosis
patients with primary raynauds without systemic sclerosis = normal capillaries
treatment for raynauds
nifedipine
management of systemic sclerosis
MDT - occupational, physio
stop smoking
steroids
immunosuppressants
-> no standardised + proven treatment
what makes polymyositis + dermatomyositis paraneoplastic syndromes?
they can be caused by an underlying malignancy
most common = lung, breast, ovarian, gastric
myositis presentation
muscle weakness + pain
occurs bilaterally + typically proximally
mostly affects shoulders + pelvic girdle
develops over weeks
others organs = interstitial lung, dysphagia, myocarditis, weightloss, raynauds
dermatomyositis skin features
- gottron lesions - scaly erythematous patches on knuckles, elbows + knees
- photosensitive erythematous rash - back, shoulders
- purple rash on face + eyelids (helitope rash)
- periorbital swelling
- subcutaneous calcinosis
antibodies associated with dermatomyositis + polymyositis
anti-Jo-1 antibodies
anti-Mi-2
anti-nuclear
dermatomyositis + polymyositis investigations
muscle biopsy = definitive diagnosis
creatine kinase
anti-Jo-1, anti-Mi-2, ANA
electromyography (EMG) - increased fibrillation, abnormal motor potentials, repetitive discharges
management of dermatomyositis + polymyositis
steroids = first line, if inadequate -
- immunosuppressants - azathioprine
- IV immunoglobulins
- biological therapy - infliximab
new cases should be assessed for underlying cancer
osteoporosis
a reduction in bone mineral density
- less strong bone = prone to fractures
- can be secondary to endocrine (cushings, hyperparathyroidism) + GI (hepatic insuffieciency, vit C + D deficiency) disorders
(osteopenia = less severe reduction in density)
osteoporosis risk factors
old, reduced activity low BMI (<18.5) female, postmenopausal (less oestrogen which is protective against osteoporosis) rheumatoid arthritis alcogol + smoking low vit D + calcium long term corticosteroids
osteoporosis investigations
DEXA scanning - provides measure of bone mineral density
normal serum calcium + phosphate
osteoporosis treatment
bisphosphonates (alendronate) - reduces osteoclastic resorption monoclonal antibody (desunoman) - reduces osteoclast activity
diet, exercise, sun exposure
calcium + vit D supplements
bisphosphonates side effects
- reflux + oesophageal erosions
- atypical fractures - femoral fractures
- osteonecrosis of jaw
- osteonecrosis of external auditory canal
examples: alendronate, risedronate, zoledronic
MoA of bisphosphonates
interfere with osteoclasts + reducing their activity, preventing reabsorption of bone
osteomalacia
qualitative defect
defective bone mineralisation - “soft” bones
results from insufficient vit D
when occurs in kids prior to growth plates closing = rickets
osteomalacia risk factors
darker skin
low sun exposure
spends lots of time indoors
colder climates
malabsorption - IBD
chronic kidney disease - kidneys r essential in metabolising vit D to its active form
osteomalacia treatment
supplementary vit D = colecalciferol
osteomalacia investigations
serum 25-hydroxyvitamin D - <25 = deficiency, 25-50 = insufficiency
LOW serum calcium + phosphate
DEXA shows low mineral density
serum alkaline phosphate may be high
parathyroid hormone may be high - secondary hyperparathyroidism
pathophysio of osteomalacia
- vit D is essential in calcium + phosphate absorption from intestines + kidneys - calcium + phosphate r required for bone construction
- vit D also responsible for regulating bone turnover + promoting bone reabsorption to boost serum calcium level
* low calcium causes secondary hyperparathyroidism - parathyroid gland tries to raise calcium levels by secreting PTH which increases reabsorption from bone (causes further problems)
Paget’s disease
disorder of excessive bone turnover - excessive activity of osteoblast + osteoclasts
–> not coordinated - patchy areas of high density (sclerosis) + low density (lysis)
increase risk of pathological fractures
particularly affects axial skeleton
complications of paget’s disease
osteosarcoma
spinal stenosis + spinal cord compression - deformity leads to spinal cord narrowing, presses on spinal nerve causing symptoms
polymyalgia rheumatica
inflammatory causing pain + STIFFNESS in shoulders, pelvic girdle + neck
occurs exclusively in those over 50
more common in women + Caucasians
strong associations with giant cell arteritis
polymyalgia rheumatica presentation
bilateral shoulder/pelvic girdle pain
worse with movement + interferes with sleep
stiffness for at least 45 mins in morning
muscle strength is normal
symptoms of giant cell arteritis
what blood abnormalities would you expect to see in a patient with fibromyalgia?
no abnormalities/all Ix normal - clinical diagnosis
(widespread pain, unrefreshed sleep, cognitive symptoms, fatigue) for >= 3months
polymyalgia rheumatica investigations
exclude differentials
diagnosis based on clinical presentation + rapid response to steroids
raised inflammatory markers
polymyalgia rheumatica treatment
15mg prednisolone daily (40-60mg if signs of giant cell arteritis)
– start reducing if good response @ 3-4weeks (reduce over 18months - 2yrs)
*if poor response after 1 week - probs not PMR, stop steroids !
types of vasculitis affecting small vessels
Henoch-Schonlein purpura (HSP)
Eosinophilic granulomatosis with polyangitis (Churg-Strauss syndrome)
Microscopic polyangitis
Granulomatosis with polyangitis (Wegener’s granulomatosis)
types of vasculitis affecting medium vessels
Polyarteritis nodosa
eosinophilic granulomatosis with polyangitis (Churg-Strauss syndrome)
Kawasaki disease
types of vasculitis affecting large vessels
Giant cell arteritis
Takayasu’s arteritis
Henoch-Scholein purpura (HSP)
IgA deposits in blood vessels of affected organs
children <10
purpuric rash on lower limbs/bum
often triggered by infection 1-3 weeks previous
management = supportive, analgesia/rest
– resolve within 8 weeks (may relapse)
eosinophilic granulomatosis with polyangitis (Churg-Strauss)
small + medium vessels
lung + skin
severe asthma in late teenage years
raised eosinophils
microscopic polyangitis
renal failure
SOB + haemoptysis
causes of chest pain in SLE
PE
pericarditis
MI
pleurisy
most appropriate first test in patient with SLE
urinalysis
-> if evidence of blood/protein, renal biopsy can be done
what clinical features are included in the modified New York criteria for diagnosis of ankylosing spondylitis?
back pain > 3 months
back pain worse at rest + relieved with movement
x-ray changes of spondylitis
x-ray finding indicative of pseudogout
chondrocalcinosis
how are uric acid crystals seen under polarised light microscopy?
negatively birefringent, needle shaped crystals
GOUT
which type of crystals are responsible for Milwaukee shoulder?
hydroxyapatite crystals
how do calcium pyrophosphate crystals appear under polarised light microscopy?
positively birefringent, rhomboid/envelope shaped crystals
PSEUDOGOUT
Z-shaped thumb + squaring of thumb base are clinical features of what diseases?
z-shaped thumb = rheumatoid arthritis
squaring of thumb base = osteoarthritis
what are uricosuric agents?
enhance renal clearance (do not affect uric acid production but inhibit reabsorption of uric acid)
contraindicated in renal impairment
examples: probenecid, sulfinpyrazone
what does “golder’s elbow” affect?
medial epicondyle
what does “tennis elbow” affect?
lateral epicondyle
Is ANCA positive in every type of vasculitis?
NO - large + medium vessel vasculitides are generally negative for ANCA
granulomatosis with polyangiitis (Wegener’s granulomatosis)
affects respiratory tract + kidneys
presentation = saddle shaped nose, hearing loss, nose bleeds, crusty nasal secretions, cough, wheeze, haemoptysis
(small vessels)
polyarteritis nodosa
associated with hep B - also hep C + HIV
medium vessels
associated with livedo reticularis rash (mottled, purplish, lace like rash)
which types of vasculitis commonly affect kids?
Henoch-Schonlein purpura (HSP) - <10
Kawasaki Disease - <5
Kawasaki disease
affects young (<5), medium vessels
persistent high fever >5days
erythematous rash, bilateral conjunctivitis
skin peeling of palms + soles
**“strawberry” tongue - red with prominent papillae
treatment = aspirin + IV immunoglobulins complication = CAD
investigations of vasculitis
inflam markers + urine dipstick
immunofluorescence to detect anti-neutrophil cytoplasmic antibodies (ANCA)
- p-ANCA (MPO) = microscopic polyangiitis + Churg-Strauss syndrome
- c-ANCA (PRS antibodies) = Wegener’s granulomatosis
what are p-ANCA (MPO antibodies) associated with?
microscopic polyangiitis (renal failure, SOB+haemoptysis) Churg-Strauss syndrome (severe asthma in late teenager, high eosinophils)
what are c-ANCA (PR3 antibodies) associated with?
Wegener’s granulomatosis (saddle shaped nose, hearing loss)
risk factors for giant cell arteritis
white females over 50
associated with polymyalgia rheumatica
giant cell arteritis presentation
temporal arteries prominent with reduced pulsation
unilateral headaches around temple + forehead
tender scalp
temporary visual disturbances
jaw claudication
how is giant cell arteritis diagnosed?
temporal artery biopsy = “multinucleated giant cells”
presentation
raised ESR
duplex US of temporal artery = hypoechoic halo
Takayasu’s arteritis
mainly aorta + it’s branches - swell + form aneurysms / become narrowed + blocked
under 40, asian, women
Px = claudication, syncope, fever, muscle aches diagnosis = CT or MRI angiography / doppler US in carotid disease
treatment of giant cell arteritis + Takayasu’s arteritis
steroids
