MRCP2 Flashcards
Associations of adhesive capsulitis?
Diabetes
What movements are affected in adhesive capsulitis?
Active and passive movements
External rotations > internal rotation
Phases of a frozen shoulder?
Freezing
Adhesive
Recovery
Management of adhesive capsulitis?
- NSAID
- Oral corticosteroid
- Intra-articular steroids
What are the ANCA positive vasculitis?
Granulomatosis with polyangiitis
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
Microscopic polyangiitis
First line investigations in ANCA positive vasculitis?
Urine analysis: Haematuria + proteinurea
Blood:
urea and creatinine for renal impairment
full blood count: normocytic anaemia and thrombocytosis may be seen
CRP: raised
ANCA testing (see below)
CXR: nodular, fibrotic or infiltrative lesions may be seen
cANCA?
granulomatosis with polyangiitis
pANCA?
eosinophilic granulomatosis with polyangiitis + others (see below)
Target of cANCA?
Serine proteinase 3 (PR3)
What is cANCA positive in?
Wegners - 90%
Microscopic polyangitis - 40%
Churg strauss - low
What is pANCA positive in ?
Churg struass - 50 % !!
Microscopic polyangitis - 75%
Wegners - 25%
What are the associations of pANCA?
Ulcerative colitis (70%)
Primary sclerosing cholangitis (70%)
Anti-GBM disease (25%)
Crohn’s disease (20%)
What is the HLA association for ankylosing spodylitis?
HLA - B27
Clinical findings of ankylosing spondylitis?
Reduced lateral flexion
Reduced forward flexion - schober’s test
Reduced chest expansion
What is a positive schobers test?
a line is drawn 10 cm above and 5 cm below the back dimples (dimples of Venus).
The distance between the two lines should increase by more than 5 cm when the patient bends as far forward as possible
What are the associations of ankylosing spondylitis?
(the begin with A)
Apical fibrosis
Anterior uveitis
Aortic regurgitation
Achilles tendonitis
AV node block
Amyloidosis
and cauda equina syndrome
peripheral arthritis (25%, more common if female)
Can anklyosing spondylitis be ruled out if CRP and ESR not increased?
No
Findings on radiograph of ankylosing spondylitis spine?
sacroiliitis: subchondral erosions, sclerosis
squaring of lumbar vertebrae
‘bamboo spine’ (late & uncommon)
syndesmophytes: due to ossification of outer fibers of annulus fibrosus
chest x-ray: apical fibrosis
First line management of ankylosing spondylitis?
NSAIDs
When should DMARDs be started in ankylosing spondylitis?
only really useful if there is peripheral joint involvement
Associations of antiphospholipid syndrome?
SLE
other autoimmune disorders
lymphoproliferative disorders
phenothiazines (rare)
Features of antiphospholipid syndrome?
venous/arterial thrombosis
recurrent fetal loss
livedo reticularis
other features: pre-eclampsia, pulmonary hypertension
Antibodies found in antiphospholipid syndrome?
anticardiolipin antibodies
anti-beta2 glycoprotein I (anti-beta2GPI) antibodies
lupus anticoagulant
FBC + coag for antiphospholipid syndrome?
Thrombocytopaenia
Prolonged APTT
Primary prophylaxis in antiphopholipid syndrome?
Aspirin
Secondary prophylaxis in antiphospholipid syndrome?
initial venous thromboembolic events: lifelong warfarin with a target INR of 2-3
recurrent venous thromboembolic events: lifelong warfarin; if occurred whilst taking warfarin then consider adding low-dose aspirin, increase target INR to 3-4
arterial thrombosis should be treated with lifelong warfarin with target INR 2-3
Mechanism of azathioprine?
purine analogue that inhibits purine synthesis
Adverse effects of azathioprine?
bone marrow depression
- consider a full blood count if infection/bleeding occurs
nausea/vomiting
pancreatitis
increased risk of non-melanoma skin cancer
Interaction: Azathioprine + Allopurinol?
Bone marrow suppression
Triad of bechet syndrome?
Oral ulcers
Genital ulcers
Anterior uveitis
Other features:
thrombophlebitis and deep vein thrombosis
arthritis
neurological involvement (e.g. aseptic meningitis)
GI: abdo pain, diarrhoea, colitis
erythema nodosum
HLA association of bechets?
HLA b51
Test for bechets?
Positive Pathergy test
puncture site following needle prick becomes inflamed with small pustule forming
Calcium (normal) + Phosphate ( normal) + ALP (normal) + PTH (normal)
Osteoporosis
/ Osteopetrosis
Calcium (decreased) + Phosphate ( decreased) + ALP (increased) + PTH (increased)
Osteomalacia
Calcium (increased) + Phosphate ( decreased) + ALP (increased ) + PTH (increased)
Primary hyperparathyroidism
Calcium (decreased) + Phosphate ( increased) + ALP (increased ) + PTH (increased)
Secondary hyperparathyroidism
Calcium (normal) + Phosphate ( normal) + ALP (increased ) + PTH (normal)
Pagets
Double bubble / soap bubble?
Giant cell tumour
Osteoma?
Benign growth
What is osteoma associated with?
Gardner’s syndrome (a variant of familial adenomatous polyposis, FAP)
Ewing sarcoma?
Children
small round blue cell tumour
t(11;22) translocation which results in an EWS-FLI1 gene product
“onion skin appearance”
Most common malignant bone tumour ?
Osteosarcoma
cartilage-capped bony projection on the external surface of a bone
Osteochondroma
Codman triangle (from periosteal elevation) and ‘sunburst’ pattern
Osteosarcoma
Mutation associated with osteosarcoma?
Rb gene significantly increases risk of osteosarcoma
Chondrosarcoma?
malignant tumour of cartilage
most commonly affects the axial skeleton
more common in middle-age
What is a charcot joint?
Neuropathic joint
badly disrupted and damaged secondary to a loss of sensation.
Physical activity ME/ CF?
‘feel ready to progress their physical activity beyond their current activities of daily living’
Management of ME/ CF ?
No other illness found
NICE stress this is ‘supportive’ rather than curative for CFS
Denosumab - inidication and mechanism?
treatment for osteoporosis
prevents the development of osteoclasts by inhibiting RANKL
WHat should be investigated in dermatomyositis?
Underlying malignancy
Underlying connective tissue diseae
Antibodies in dermatomyositis?
ANA positive
Jo1: antibodies against histidine-tRNA ligase
srp: antibodies to signal recognition particle
anti-Mi-2 antibodies
Features of dermatomyositis?
photosensitive
macular rash over back and shoulder
heliotrope rash in the periorbital region
Gottron’s papules - roughened red papules over extensor surfaces of fingers
‘mechanic’s hands’: extremely dry and scaly hands with linear ‘cracks’ on the palmar and lateral aspects of the fingers
nail fold capillary dilatation
proximal muscle weakness +/- tenderness
Raynaud’s
respiratory muscle weakness
interstitial lung disease: e.g. Fibrosing alveolitis or organising pneumonia
dysphagia, dysphonia
What antibody is the most specific for dermatomyositis?
Anti- Mi1
Is Anti-Jo1 common in dermatomyositis?
No - they are more common in polymyositis where they are seen in a pattern of disease associated with lung involvement, Raynaud’s and fever
Management of dermatomyositis?
prednisolone
What is diabetic amyotrophy?
proximal diabetic neuropathy.
Features of diabetic amyotrphy?
pain is usually in the first symptom, often in the hips or buttocks
this is followed by weakness, for example difficulty getting out of a chair
What is Diffuse idiopathic skeletal hyperostosis (DISH) ?
ossification at sites of tendinous and ligamentous insertion of the spine. It tends to be seen in elderly patients.
What is the most common causative organism of discitis?
Bacterial
Staphylococcus aureus is the most common cause of discitis
Aetiology of dscitis?
Bacterial
Staphylococcus aureus is the most common cause of discitis
Viral
TB
Aseptic
What is the best imaging modality for discitis?
MRI spine
What can inform antibiotic of discitis?
CT guided biopsy
Features of discitis?
Back pain
pyrexia,
rigors
sepsis
Neurological features
e.g. changing lower limb neurology
if an epidural abscess develops
What other investigation should be completed for discitis?
Echo
What is discoid lupus?
It is not SLE
It is a rash - haracterised by follicular keratin plugs and is thought to be autoimmune in aetiology
Will cause scarring alopecia
Features of discoid lupus?
erythematous, raised rash, sometimes scaly
may be photosensitive
more common on face, neck, ears and scalp
lesions heal with atrophy, scarring (may cause scarring alopecia), and pigmentation
Management of discoid lupus?
First line: topical steroid cream
Second line: oral antimalarials may be used second-line e.g. hydroxychloroquine
avoid sun exposure
Features of drug induced lupus?
arthralgia
myalgia
skin (e.g. malar rash) and pulmonary involvement (e.g. pleurisy) are common
Antibodies in drug induced SLE?
ANA positive in 100%, dsDNA negative
anti-histone antibodies are found in 80-90%
anti-Ro, anti-Smith positive in around 5%
Most common causes of drug induced lupus?
procainamide
hydralazine
Less common causes
isoniazid
minocycline
phenytoin
What is the defect in ehlers danlos syndrome?
affects type III collagen.
Features of ehlers danlos syndrome?
elastic, fragile skin
joint hypermobility: recurrent joint dislocation
easy bruising
aortic regurgitation, mitral valve prolapse and aortic dissection
subarachnoid haemorrhage
angioid retinal streaks
Inheritence of familial mediterian fever?
Autosomal recessive
Features of familial mediterrian fever?
Features - attacks typically last 1-3 days
pyrexia
abdominal pain (due to peritonitis)
pleurisy
pericarditis
arthritis
erysipeloid rash on lower limbs
Features of familial mediterian fever?
Colchicine
Features of fibromyalgia?
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
Synovial fluid analysis of gout?
negatively birefringent monosodium urate crystals under polarised light
When should uric acid levels be checked in gout?
acute episode has settled down (typically 2 weeks later) as may be high, normal or low during the acute attack
Radiological features of gout?
joint effusion is an early sign
well-defined ‘punched-out’ erosions with sclerotic margins in a juxta-articular distribution, often with overhanging edges
relative preservation of joint space until late disease
eccentric erosions
no periarticular osteopenia (in contrast to rheumatoid arthritis)
soft tissue tophi may be seen
Management of gout?
- Colchicine
- oral steroids may be considered if NSAIDs and colchicine are contraindicated.
a dose of prednisolone 15mg/day is usually used - Intra-articular steroid
Acute gout + already on allopurinol?
Keep allopurinol going
Who should get uric acid lowering therapy in gout?
all patients after their first attack of gout
When can colchicine not be used?
avoid if eGFR < 10 ml/min
When to start urate lowering therapy in gout?
‘Commencement of ULT is best delayed until inflammation has settled as ULT is better discussed when the patient is not in pain’
How long should a patient remain on colchine for gout?
6 months
Urate lowering therapy
First line : allopurinol
Second line: Febuxostat
Urate lower therapy in refractory cases?
Use in persistent symptoms of severe gout
pegloticase (polyethylene glycol modified mammalian uricase)
Hypertension + gout ?
Consider losartan
Drugs that precipitate gout?
Thiazide diuretics
Predisposing factors for gout?
Decreased excretion of uric acid
drugs*: diuretics
chronic kidney disease
lead toxicity
Increased production of uric acid
myeloproliferative/lymphoproliferative disorder
cytotoxic drugs
severe psoriasis
Gout predisposition: Causes that reduce excretion of uric acid?
drugs*: diuretics
chronic kidney disease
lead toxicity
Gout predisposition: Causes that increase production of uric acid?
myeloproliferative/lymphoproliferative disorder
cytotoxic drugs
severe psoriasis
Genetic condition that increases risk of gout?
Lesch-Nyhan syndrome
Features of lesch nyhan syndrome?
x-linked recessive therefore only seen in boys
features: gout, renal failure, neurological deficits, learning difficulties, self-mutilation
Unequal skin folds + positive ortolani/Barlow?
Development dysplasia of hip
Viral infection + acute hip pain
Transient synovitis
Features of Perthes disease?
hip pain: develops progressively over a few weeks
limp
stiffness and reduced range of hip movement
x-ray: early changes include widening of joint space, later changes include decreased femoral head size/flattening
Features of slipped upper femoral epiphysis?
knee or distal thigh pain is common
loss of internal rotation of the leg in flexion
More common in obese children and boys
Displacement of the femoral head epiphysis postero-inferiorly
Bilateral slip in 20% of cases
May present acutely following trauma or more commonly with chronic, persistent symptoms
Features of juvinile idiopathic arthritis?
joint pain and swelling: usually medium sized joints e.g. knees, ankles, elbows
limp
ANA may be positive in JIA - associated with anterior uveitis
Acute hip pain associated with systemic upset e.g. pyrexia. Inability/severe limitation of affected joint
Septic arthritis
Features of chondromalacia paterllae?
Characteristically anterior knee pain on walking up and down stairs and rising from prolonged sitting
Usually responds to physiotherapy
Pain, tenderness and swelling over the tibial tubercle
Osgood sclatter
Pain after exercise
Intermittent swelling and locking
Osteochondritis dissecans
More common in athletic teenage boys
Chronic anterior knee pain that worsens after running
Tender below the patella on examination
Patellar tendonitis
bone pain, typically in the skull or proximal femur
cutaneous nodules
recurrent otitis media/mastoiditis
tennis racket-shaped Birbeck granules on electromicroscopy
Langerhans cell histiocytosis
What is lateral epicongylitis commonly called ?
Tennis elbowq
Features of lateral epicondylitis?
pain and tenderness localised to the lateral epicondyle
pain worse on wrist extension against resistance with the elbow extended or supination of the forearm with the elbow extended
episodes typically last between 6 months and 2 years. Patients tend to have acute pain for 6-12 weeks
Management of lateral epicondylitis?
advice on avoiding muscle overload
simple analgesia
steroid injection
physiotherapy
Red flags for back pain?
age < 20 years or > 50 years
history of previous malignancy
night pain
history of trauma
systemically unwell e.g. weight loss, fever
Features of spinal stenosis?
Unilateral or bilateral leg pain (with or without back pain), numbness, and weakness which is worse on walking. Resolves when sits down. Pain may be described as ‘aching’, ‘crawling’.
Relieved by sitting down, leaning forwards and crouching down
Clinical examination is often normal
Best imaging for spinal stenosis?
MRI spine
Features of ankylosing spondylitis?
Typically a young man who presents with lower back pain and stiffness
Stiffness is usually worse in morning and improves with activity
Peripheral arthritis (25%, more common if female)
Motor function of femoral nerve?
Knee extension, thigh flexion
Sensory function of femoral nerve ?
Anterior and medial aspect of the thigh and lower leg
Mechanism of injury for femoral nerve?
Hip and pelvic fractures
Stab/gunshot wounds
Motor function of obturator nerve?
Thigh ADDuction
Sensory function of obturator nerve?
Medial thigh
Mechanism of injury of obturator nerve?
Anterior hip dislocation
Sensory function of lateral cutaneous nerve of thigh?
Lateral and posterior surfaces of the thigh
Mechanism of injury of lateral cutaneous nerve of thigh?
Compression of the nerve near the ASIS → meralgia paraesthetica, a condition characterised by pain, tingling and numbness in the distribution of the lateral cutaneous nerve
Motor function of tibial nerve?
Foot plantarflexion and inversion
Sensory function of tibial nerve?
Sole of foot
Motor function of common peroneal nerve ?
Compression of the nerve near the ASIS → meralgia paraesthetica, a condition characterised by pain, tingling and numbness in the distribution of the lateral cutaneous nerve
Sensory function of common peroneal nerve?
Dorsum of the foot and the lower lateral part of the leg
Motor function of superior gluteal nerve?
Hip abduction
Injury to what nerve gives a postivie trandelburg test?
Superior gluteal nerve
Difficulty rising from seated position - injury to what nerve?
Inferior gluteal nerve
Sitting is better than standing
Walking uphill better than walking down hill
Lumbar spinal stenosis
Marfan’s syndrome - mutation?
Defect in the FBN1 gene on chromosome 15 that codes for the protein fibrillin-1
Features of marfans syndrome?
tall stature with arm span to height ratio > 1.05
high-arched palate
arachnodactyly
pectus excavatum
pes planus
scoliosis of > 20 degrees
Heart features of Marfains syndrome?
dilation of the aortic sinuses (seen in 90%) which may lead to aortic aneurysm, aortic dissection, aortic regurgitation
mitral valve prolapse (75%),
Eye features of marfans syndrome?
upwards lens dislocation (superotemporal ectopia lentis)
blue sclera
myopia
Dural ectasia
(ballooning of the dural sac at the lumbosacral level)
Marfans syndrome
Features of McArdle’s disease?
muscle pain and stiffness following exercise
muscle cramps
myoglobinuria
low lactate levels during exercise
Features of McArdle’s disease?
autosomal recessive type V glycogen storage disease
caused by myophosphorylase deficiency
this causes decreased muscle glycogenolysis
Mechanism of methotrexate?
Inhibits difolate reducatase
Adverse effects of methotrexate?
pulmonary fibrosis
liver fibrosis
mucositis
myelosuppression
pneumonitis
How long after use of methotrexate should a women become pregnant?
6 months
Drugs to avoid in methotrexate?
Trimethoprim
Co-trimoxazole
What is the treatment for methotrexate toxicity?
Folinic acid
Causes of myopathies?
inflammatory: polymyositis
inherited: Duchenne/Becker muscular dystrophy, myotonic dystrophy
endocrine: Cushing’s, thyrotoxicosis
alcohol
Side effects of methotrexate?
mucositis
myelosuppression
pneumonitis
malaise, fever
pulmonary fibrosis
liver fibrosis
Mechanism of NSAID?
activity of cyclooxygenase enzymes (COX-1 and/or COX-2) thus reducing the production of key mediators involved in inflammation such as prostaglandins.
Adverse effects of methotrexate?
pulmonary fibrosis
liver fibrosis
mucositis
myelosuppression
pneumonitis
COX2 inhibitors examples?
Concerns of this use?
celecoxib and etoricoxib
Concerns regarding cardiovascular safety
Red flags in hip pain?
rest pain
night pain
morning stiffness > 2 hours
Osteoarthritis xray changes?
Loss of joint space
Osteophytes forming at joint margins
Subchondral sclerosis
Subchondral cysts
Defect in osteogenesis imperfecta?
type 1 collagen due to decreased synthesis of pro-alpha 1 or pro-alpha 2 collagen polypeptides
Features of osteogenesis imperfecta?
presents in childhood
fractures following minor trauma
blue sclera
deafness secondary to otosclerosis
dental imperfections are common
Calcium biochemistry results in osteogeneiss imperfecta?
usually normal in osteogenesis imperfecta
What is osteomalacia?
Softening of bone related to lack of vitamin D
Causes of vitamin D deficiency?
Causes
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
Xray findings for osteomalacia?
x-ray
translucent bands (Looser’s zones or pseudofractures)
Osteomyelitis + Sickle cell ?
Salmonella
Osteomyelitis most common organism?
Staph
Imaging modality of choice for osteomyelitis?
MRI
Management of osteopetrosis?
stem cell transplant and interferon-gamma have been used for treatment
Fragility fracture + Age > 75 ?
Osteoporosis is assumed
No need for dexa
Fragility fracture + Age <75?
DEXA arranged
entered into a FRAX assessment (along with the fact that they’ve had a fracture) to determine the patients ongoing fracture risk.
Medications notes to worsen osteoporosis?
SSRIs
antiepileptics
proton pump inhibitors
glitazones
long term heparin therapy
aromatase inhibitors e.g. anastrozole
Older male + isolated ALP?
Pagets
Findings of pagets disease?
procollagen type I N-terminal propeptide (PINP)
serum C-telopeptide (CTx)
urinary N-telopeptide (NTx)
urinary hydroxyproline
Management of pagets disease ?
indications for treatment include
bone pain
skull or long bone deformity
fracture
periarticular Paget’s
Treatment:
bisphosphonate (either oral risedronate or IV zoledronate)
calcitonin is less commonly used now
Complicatiosn of pagets disease?
deafness (cranial nerve entrapment)
bone sarcoma (1% if affected for > 10 years)
fractures
skull thickening
high-output cardiac failure
hip pain: develops progressively over a few weeks
limp
stiffness and reduced range of hip movement
x-ray: early changes include widening of joint space, later changes include decreased femoral head size/flattening
Perthes disease
Diagniosis of Perthes disease?
plain x-ray
technetium bone scan or magnetic resonance imaging if normal x-ray and symptoms persist
What is polyarteritis nodosa?
vasculitis affecting medium-sized arteries with necrotizing inflammation leading to aneurysm formation
What is an assocation of polyarteritis nodosa?
Hepatitis B
Features of polyarteritis nodosa?
fever, malaise, arthralgia
weight loss
hypertension
mononeuritis multiplex, sensorimotor polyneuropathy
testicular pain
livedo reticularis
haematuria, renal failure
perinuclear-antineutrophil cytoplasmic antibodies (ANCA) are found in around 20% of patients with ‘classic’ PAN
hepatitis B serology positive in 30% of patients
Features of polymyalgia rheumatica?
typically patient > 60 years old
usually rapid onset (e.g. < 1 month)
aching, morning stiffness in proximal limb muscles
weakness is not considered a symptom of polymyalgia rheumatica
also mild polyarthralgia, lethargy, depression, low-grade fever, anorexia, night sweats
Management of polyarteritis rheumatica?
prednisolone e.g. 15mg/od
patients typically respond dramatically to steroids, failure to do so should prompt consideration of an alternative diagnosis
Mechanism behind polymyositis?
T-cell mediated cytotoxic process directed against muscle fibres
Features of polymyositis?
proximal muscle weakness +/- tenderness
Raynaud’s
respiratory muscle weakness
interstitial lung disease
dysphagia, dysphonia
e.g. fibrosing alveolitis or organising pneumonia
seen in around 20% of patients and indicates a poor prognosis
Investigation findings in polymyositis?
elevated creatine kinase
other muscle enzymes (lactate dehydrogenase (LDH), aldolase, AST and ALT) are also elevated in 85-95% of patients
EMG
muscle biopsy
anti-synthetase antibodies
anti-Jo-1 antibodies
Management of polymyositis?
high-dose corticosteroids tapered as symptoms improve
azathioprine may be used as a steroid-sparing agent
Risk factors for pseudogout?
haemochromatosis
hyperparathyroidism
low magnesium, low phosphate
acromegaly, Wilson’s disease
Joint aspiration of pseudogout?
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
Patterns seen in psioratic arthopathy?
symmetric polyarthritis
asymmetrical oligoarthritis: typically affects hands and feet (20-30%)
sacroiliitis
DIP joint disease (10%)
arthritis mutilans (severe deformity fingers/hand, ‘telescoping fingers’)
Xray findings in psoriatic arthopathy?
often have the unusual combination of coexistence of erosive changes and new bone formation
periostitis
‘pencil-in-cup’ appearance
Treatment of psoriatic arthopathy?
- Mild disease –> NSAID
- monoclonal antibodies such as ustekinumab (targets both IL-12 and IL-23) and secukinumab (targets IL-17)
- apremilast: phosphodiesterase type-4 (PDE4) inhibitor → suppression of pro-inflammatory mediator synthesis and promotion of anti-inflammatory mediators
Secondary causes of raynoids?
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
Features of raynoids that suggest another diagnosis?
onset after 40 years
unilateral symptoms
rashes
presence of autoantibodies
features which may suggest rheumatoid arthritis or SLE, for example arthritis or recurrent miscarriages
digital ulcers, calcinosis
very rarely: chilblains
Management of raynoid ?
first-line: calcium channel blockers e.g. nifedipine
IV prostacyclin (epoprostenol) infusions: effects may last several weeks/months
may need to stop propranolol
Features of reactive arthritis?
time course
typically develops within 4 weeks of initial infection - symptoms generally last around 4-6 months
around 25% of patients have recurrent episodes whilst 10% of patients develop chronic disease
arthritis is typically an asymmetrical oligoarthritis of lower limbs
dactylitis
symptoms of urethritis
eye
conjunctivitis (seen in 10-30%)
anterior uveitis
skin
circinate balanitis (painless vesicles on the coronal margin of the prepuce)
keratoderma blenorrhagica (waxy yellow/brown papules on palms and soles)
What is the time onset from infection to reactive arthritis?
4 weeks
HLA assocation for reactive arthritis?
HLA B 27
Features of relapsing polychondritis?
Ears: auricular chondritis, hearing loss, vertigo
Nasal: nasal chondritis → saddle-nose deformity
Respiratory tract: e.g. hoarseness, aphonia, wheezing, inspiratory stridor
Ocular: episcleritis, scleritis, iritis, and keratoconjunctivitis sicca
Joints: arthralgia
Less commonly: cardiac valcular regurgitation, cranial nerve palsies, peripheral neuropathies, renal dysfunction
Management of relapsing polychondritis?
Induce remission: steroids
Maintenance: azathioprine, methotrexate, cyclosporin, cyclophosphamide
Complications of rheumatoid arthritis?
respiratory: pulmonary fibrosis, pleural effusion, pulmonary nodules, bronchiolitis obliterans, methotrexate pneumonitis, pleurisy
ocular: keratoconjunctivitis sicca (most common), episcleritis, scleritis, corneal ulceration, keratitis, steroid-induced cataracts, chloroquine retinopathy
osteoporosis
ischaemic heart disease: RA carries a similar risk to type 2 diabetes mellitus
increased risk of infections
depression
Felty’s syndrome (RA + splenomegaly + low white cell count)
amyloidosis
What type of antbody is rheumatoid factor?
IgM
Tests to detect RF?
Rose-Waaler test: sheep red cell agglutination
Latex agglutination test (less specific)
What is sensitivity of RF?
RF is positive in 70-80% of patients with rheumatoid arthritis,
What can be deduced by a RF factor/
high titre levels are associated with severe progressive disease
Not a marker of diseas activity
Best test for diagnosis of rheumatoid arthritis?
Anti-cyclic citrullinated peptide antibody
heumatoid factor (around 70%) with a much higher specificity of 90-95%.
What is the ideal treatment for rheumatoid arthritis?
DMARD monotherapy
+/- short course of prednisolone
How to monitor disease response in RA?
CRP
How should flares of RA be managed?
RA are often managed with corticosteroids - oral or intramuscular
When should TNF inhibitors be considered in RA?
When two DMARDS have failed
Examples of DMARDS in RA?
Methotrexate
sulfasalazine
leflunomide
hydroxychloroquine
Presentation of RA?
swollen, painful joints in hands and feet
stiffness worse in the morning
gradually gets worse with larger joints becoming involved
presentation usually insidiously develops over a few months
positive ‘squeeze test’ - discomfort on squeezing across the metacarpal or metatarsal joints
Most common organism for septicarthritis?
Staph aureus
Investigation for septicarthritis?
Synvocial joint aspirate
done prior to the administration of antibiotics if necessary
may need to be done under radiographic guidance
Management of septic arthritis?
flucloxacillin or clindamycin if penicillin allergic
Painful arc of abduction between 60 and 120 degrees
Tenderness over anterior acromion
Rotator cuff injury
Supraspinatus tendonitis
(Subacromial impingement,
painful arc)
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
Management of still’s disease?
NSAID
Steroids
if symptoms persist, the use of methotrexate, IL-1 or anti-TNF therapy can be considered
Features of sjorgen’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)
Investigation findings of sjorgen’s syndrome?
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
Management of sjorgen’s syndrome?
- Artifical tears
- Pilocaripine - tear stimulation
What is the most specifc antibody in lupus?
anti-Smith: highly specific (> 99%), sensitivity (30%)
anti-dsDNA: highly specific (> 99%), but less sensitive (70%)
How to monitor lupus ?
ESR is generally used
during active disease the CRP may be normal - a raised CRP may indicate underlying infection
anti-dsDNA titres can be used for disease monitoring (but note not present in all patients)
Management of SLE?
Hydroxychloroquine
the treatment of choice for SLE
Basics
NSAIDs
sun-block
Problems of SLE and pregnancy?
neonatal complications include congenital heart block
strongly associated withanti-Ro (SSA) antibodies
Difference between limited and systemic sclerosis?
Anti-centrmere –> limited
Anti-SCL70 –> diffuse
CREST syndrome –> limited
Lung involvement –> diffuse
Features of diffuse systemic sclerosis?
scleroderma affects trunk and proximal limbs predominately
interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH)
Management of diffuse systemic sclerosis?
ACEi
Antibodies in systemic sclerosis?
ANA positive in 90%
RF positive in 30%
anti-scl-70 antibodies associated with diffuse cutaneous systemic sclerosis
anti-centromere antibodies associated with limited cutaneous systemic sclerosis
Features of temporal arteritis/
typically patient > 60 years old
usually rapid onset (e.g. < 1 month)
headache (found in 85%)
jaw claudication (65%)
anterior ischemic optic neuropathy a
What happens to creatinine kinase and ESR in temporal arteritis?
They are normal
How does anterior iscaemic optic neuropathy cause visual impairment?
occlusion of the posterior ciliary artery (a branch of the ophthalmic artery) → ischaemia of the optic nerve head. Fundoscopy typically shows a swollen pale disc and blurred margins
Erb’s palsy?
C5C6
Klumpe’s plasy?
C8-T1
WHat antibody causes congenital heart block?
Anti-Ro
Three myositis specifc antibodies?
Transfer ribonucleic acid (tRNA) synthetases: Anti-Jo 1
Nuclear Mi-2 protein: Anti Mi2
Signal recognition peptide (SRP): Anti SRP
If question gives calcium biochemistry. Distinguish between osteomalacia and osteoporosis
Osteomalacia –> give calcium and vitamin D
Osteoporosis –> Bisphosphonates
Treatment for methotrexate pneumonitis?
Prednisolone + Folinic acid
First line bisphosphonate?
Alendronate
DIP joint swelling + morning stiffness that eases ?
Psoriatic arthritis
Choice of vitamin D in oseomalacia?
Mainteance dose
When to swap from 2X DMARD therapy ?
when the patient has been on at least two DMARDs, including methotrexate, reporting two DAS 28 scores of greater than 5.1 at least one month apart
Lupus nephritis + kidney dysfunction ?
Mycophenolate
Dose of steroid in GCA?
Vision complication methylprednisoone 1 g IV
Uncomplicated: 60 mg oral pred
Dose of prednisolone in PMR?
15 mg taper over 2 years
Risk factors of osteoporosis/
history of glucocorticoid use
rheumatoid arthritis
alcohol excess
history of parental hip fracture
low body mass index
current smoking
sedentary lifestyle
premature menopause
Caucasians and Asians
endocrine disorders: hyperthyroidism, hypogonadism (e.g. Turner’s, testosterone deficiency), growth hormone deficiency, hyperparathyroidism, diabetes mellitus
multiple myeloma, lymphoma
gastrointestinal disorders: inflammatory bowel disease, malabsorption (e.g. coeliac’s), gastrectomy, liver disease
chronic kidney disease
osteogenesis imperfecta, homocystinuria
What non -STI infections cause reactive arthritis?
gastrointestinal (GI) infections including Shigella, Salmonella, Campylobacter, and other organisms, as well as with genitourinary (GU) infections (especially with Chlamydia trachomatis).
Drug induced lupus ?
Anti histones
How to treat systemic sclerosis hypertensive crisis?
Oral ACE inhibitor
Gradual reduction required
Liver: when to stop methotrexate?
If ALT or ALP double
Otherwise continue
What can be considered as a steroid sparing agent in giant. cell arteritis?
Methotrexate
mild to moderate pain pathway
Step 1 - paracetamol. Increase to the maximum dose of 1 gram four times a day, before switching to (or combining with) another analgesic.
Step 2 - substitute the paracetamol with ibuprofen and increase the dose of ibuprofen to a maximum of 2.4 grams daily
Step 3 - add paracetamol to ibuprofen
Step 4 - continue with paracetamol 1 gram four times a day. Replace the ibuprofen with an alternative NSAID (such as naproxen)
Step 5 - start a full therapeutic dose of a weak opioid in addition to full-dose
paracetamol (1 gram four times a day) and/or an NSAID
Rash on sole of feet in reactive arthritis?
keratoderma blennhagia
Fever, lethargy, neuropathy, testicular pain and renal dysfunction
polyarteritis nodosa (PAN).
WHat is Jaccoud’s arthorpathy?
Jaccoud’s arthopathy is a chronic non-erosive reversible joint disorder that may occur after repeated bouts of arthritis. It is caused by inflammation of the joint capsule and subsequent fibrotic retraction, causing ulnar deviation of the fingers, through metacarpophalangeal joint (MCP) subluxation,primarily of the ring and little-finger. Joints in the feet, knees and shoulders may also get affected.It is commonly associated with systemic lupus erythematosus (SLE)
A 54-year-old woman presents to the medical clinic with an itchy rash. She says that she has noticed a bluish-purple patchy rash mostly on sun-exposed areas. On examination, she has purple eyelids and rough raised purple areas on her knuckles Her nails show ragged cuticles, and blood vessels are seen on the nail fold. A purple, poorly defined rash is present on both her arms going up to her shoulders. What is the likely diagnosis?
patient has a photosensitive rash, as well as a heliotrope rash around the eyelids, and a description of Gottron’s papules. The distribution is also in keeping with a dermatomyositis rash.
macular erythematous and photosensitive butterfly rash over the face
SLE
Hypomagnesaemia + swollen wrist hot red inflamed
Pseudogout
