Rheum Flashcards
What is Paget’s Disease of the Bone?
excessive bone turnover (reabsorption and formation) due to increased osteoclast and osteoblast activity.
Turnover is not coordinated, so get patchy (high density) sclerosis and (low density) lysis
Paget’s effect on bones
- Enlarged and misshapen bones
- Structural problems
- Increased risk of pathological fractures
- Particularly affects the axial skeleton (the bones of the head and spine).
Presentation of Paget’s
asymptomatic (incidental on xray) or:
- Bone pain
- Bone deformity
- Fractures
- Hearing loss
Paget’s Ix
X-ray findings include:
- Bone enlargement and deformity
- Osteoporosis circumscripta
- Cotton wool appearance of the skull
- V-shaped osteolytic defects in the long bones
Blood tests include:
- Raised ALP (needs monitoring)
- Normal calcium
- Normal phosphate
Paget’s Mx
Bisphosphonates
- calcitonin if can’t have bisphosphonates
- NSAIDs for bone pain
- calcium and vit D
Paget’s complications
- Hearing loss (if it affects the bones of the ear)
- Heart failure (due to hypervascularity of the abnormal bone)
- Osteosarcoma (rare but poor prognosis)
- Spinal stenosis and spinal cord compression
Define osteomalacia
soft bones due to insufficient vit D
low Vit D = low calcium and phosphate
low calcium = high PTH which increases calcium resorption from bones
RF- dark skin, low sunlight exposure, colder climate
Osteomalacia presentation
May be asymptomatic
Fatigue
Bone pain
Muscle weakness
Muscle aches
Pathological or abnormal fractures
Looser zones (fragility fractures partially through bone)
Osteomalacia Ix
- serum 25 hydroxyvitamin D (<25= deficiency, 25-50= insufficiency)
- low serum calcium
- low serum phosphate
- high ALP
- high PTH
- Xray: osteopenia (radiolucent bones)
- DEXA: low bone mineral density
Osteomalacia Mx
colecalciferol (vit D)
loading dose 50,000 IU once a week for 6 weeks or 4000 IU daily for 10 weeks.
then maintenance dose of 800-2000
Define Osteoporosis
reduction in bone density
DEXA <-2.5
-1 to -2.5 = osteopenia
DEXA on femoral neck most important to check
Osteporosis RFs
- older age
- post-menopausal
- reduced mobility
- low BMI
- low calcium or vit D intake
- alcohol and smoking
- long term steroid use (7.5mg and above)
- fracture hx
- CKD, hyperthyroidism, RA
- SSRI, PPI, anti-epileptic, anti-oestrogen
Osteoporosis Ix
QFracture tool
FRAX tool
measures 10 year risk of fracture
above 10%= do DEXA
Osteoporosis Mx
- stop smoking and alcohol
- exercise
- calcium
- Vitamin D
- Bisphosphonates (take on empty stomach to reduce reflux)
- denosumab
- HRT
Side effects of bisphosphonates
- reflux and oesophageal erosions
- atypical fractures
- osteonecrosis of the jaw
- osteonecrosis of external auditory canal
Define Gout
crystal arthropathy
high uric acid
raised serum urate level
joint aspiration: monosodium urate crystals, needle shaped, negative bifringent
Xray- maintained joint space, lytic lesions in bone, punched out eosions
Gout presentation
- hot swollen and tender joint
- gouty tophi in hands elbows and ears (subcut uric acid deposit)
- base of big toe (MTP joint)
- base of thumb (CMC joint)
wrist - larger joint (knee/ankle)
Gout RF
male
FH
alcohol
obesity
high purine diet (meat/seafood)
diuretic
CVD
Kidney disease
Difference between gout and pseudogout
Monosodium urate crystals of gout are needle-shaped and negatively birefringent of polarised light.
The calcium pyrophosphate crystals of pseudogout are rhomboid-shaped and positively birefringent.
Gout Mx
acute:
naproxen
colchicine
prednisolone
Chronic
allopurinol
febuxostat
use NSAID alongside allopurinol at beginning as it can trigger gout flare
- lose weight and hydrate
- no alcohol and purines
Define pseudogout
calcium pyrophosphate crystals
Pseudogout presentation
Many asymptomatic
chronic pain and stiffness in joints
>65 y/o
hot swollen stiff and painful knee
or shoulders, hips, wrists
Diagnosing pseudogout
Need joint aspirate to exclude septic arthritis: rhomboid positive birefringent
Xray: chrondrocalcinosis - calcium deposits in joint cartilage.
Similar xray changes to OA
L: LOJS
O: osteophytes
S: subarticular sclerosis (increased bone density on joint line)
S: subchondral cysts (fluid filled holes in bone)
Pseudogout Mx
resolve spontaneously
symptomatic mx:
NSAID
colchicine
joint steroid injection
oral steroids
Define Ehlers-Danlos Syndrome
Genetic conditions involving collagen defects
Causes hypermobility and connective tissue abnormality of the skin, bones etc.
Types of EDS
Hypermobile EDS
Classical EDS
Vascular EDS
Kyphoscoliotic EDS
what is hypermobile EDS
most common
least severe
joint hypermobility and stretchy skin
autosomal dominant
what is classical EDS
stretchy skin that feels smooth and velvety
severe hypermobility, joint pain and abnormal wound healing
lumps over pressure points
prone to hernias, prolapses, MR and aortic root dilatation
autosomal dominant
What is vascular EDS
most severe and dangerous
blood vessels prone to rupture
thin translucent skin
gastrointestinal perforation
spontaneous pneumothorax
autosomal dominant
Kyphoscoliotic EDS
hypotonia as neonate/infant
kyphoscoliosis as they grow
joint hypermobility
joint dislocation
autosomal recessive
What can occur with HEDS
postural orthostatic tachycardia syndrome (tachy on sitting/standing)
how to score hypermobility
Beighton score
EDS Mx
no cure
physio to stabilise the joints
OT
What is Behcet’s disease
affects BVs and tissues
recurrent oral and genital ulcers >3x a year (red halo)
can affect eye, skin, GIT, lungs, MSK and CNS
HLA B51 gene
Signs of Behcet’s disease
- halo red ulcer
- erythema nodosum
- anterior uveitis
- arthralgia
- ulceration of ileum, caecum
- aseptic meningitis
- aneurysms
- DVT and Budd-chiari syndrome
Test for Behcet’s
pathergy test
positive test indicates Behcet’s, sweet’s syndrome or pyoderma gangrenosum
Behcet’s disease Mx
topical steroids for ulcers
prednisolone
colchicine
topical anaesthetics
azathioprine
biologic: infliximab
Behcet prognosis
relapsing-remitting
normal LE
Define vasculitis
inflammation of BVs
Types of vasculitis
Henoch-Schonlein Purpura
Microscopic Polyangiitis
Granulomatosis with Polyangiitis
Eosinophilic Granulomatosis with Polyangiitis
Polyarteritis Nodosa
Kawasaki Disease
Giant Cell Arteritis
Takayasu’s Arteritis
HSP features
- Small Vessel
- Purpura (non-blanching rash)
- IgA nephritis (renal involvement)
- children
- joint and abdo pain
Mx- supportive
Microscopic Polyangiitis features
- Small vessel
- p-ANCA
- Glomerulonephritis causing renal failure
- Diffuse alveolar haemorrhage
Granulomatosis with Polyangiitis features
- Small vessel
- c-ANCA
- Nasal symptoms
- Respiratory symptoms
- Glomerulonephritis
- saddle shaped nose due to bridge collapse
Eosinophilic Granulomatosis with Polyangiitis features
- Small vessel
- p-ANCA
- Raised eosinophils
- Late-onset severe asthma
- Sinusitis and rhinitis
Polyarteritis Nodosa features
- Medium vessel
- idiopathic or due to Hep B
- Renal impairment
- Hypertension
- Cardiovascular events
- Tender skin nodules
Kawasaki Disease features
- Medium vessels
- children <5
- High fever (more than 5 days)
- Widespread rash
- Bilateral conjunctivitis
- Strawberry tongue
- Desquamation of palms and soles
- Coronary artery aneurysms is a complication
Mx- aspirin and IVIG
Giant Cell Arteritis features
- Large vessels
- Raised ESR
- Unilateral headache
- Scalp tenderness
- Vision loss
Mx steroids
Takayasu’s Arteritis features
- Large vessel
- Aortic arch affected
- “Pulseless” disease
- claudication sx
Need CT/MRI angio to diagnose
General vasculitis features
Joint and muscle pain
Peripheral neuropathy
Renal impairment
Purpura (purple-coloured non-blanching spots caused by blood leaking from the vessels under the skin)
Necrotic skin ulcers
Gastrointestinal symptoms (e.g., diarrhoea, abdominal pain and bleeding)
Vasculitis Ix
inflammatory markers (CRP ESR)
ANCA (p and c)
Vasculitis Mx
steroids
Define Sjogren’s syndrome
autoimmune condition affecting exocrine glands (lacrimal and salivary)
Primary- in isolation
Secondary- due to SLE or RA
Sx of Sjogren’s
dry mouth, eyes and vagina (sicca sx)
dry skin
joint pain and stiffness
women
middle age
Antibodies in sjogren’s
anti-Ro
anti-La
Mx of Sjogren’s
eye drops
artificial saliva
vaginal lubricants
pilocarpine to stimulate tear/saliva
hydroxychloroquine
Define Antiphospholipid Syndrome
autoimmune caused by antiphospholipid abs
cause inflammation and increase thrombosis risk
Name the different antiphospholipid antibodies
lupus anticoagulant
anticardiolipin antibodies
anti-beta 2 glycoprotein I antibodies
Mx antiphospholipid syndrome
primary thromboprophylaxis- aspirin
long term warfarin INR 2-3
LMWH and aspirin used in pregnancy
Define polymyositis and dermatomyositis
autoimmune disorders causing muscle inflammation presenting with proximal muscle weakness
Dermatomyositis signs
Gottron papules on back of hands
helitrope rash on eyelids
additional:
Raynaud’s
respiratory muscle weakness
interstitial lung disease: e.g. Fibrosing alveolitis or organising pneumonia
dysphagia, dysphonia
Cause of polymositits or dermatomyositis
can be due to underlying cancer –> paraneoplastic syndromes
viral infection (coxsackie virus or HIV)
HLA genes risk factors
presentation of Polymyositis or dermatomyositis
gradual onset
symmetrical
proximal muscle weakness
difficulty standing from chair
myalgia
no skin changes in poly
skin changes in dermato
- gottron lesions, helitrope rash, periobrital oedema, photosensitive erythematous rash
What is the test for myositis?
creatine kinase in the thousands
Mx of polymyositis and dermatomyositis
steroids
or
immunosuppressants,
IVIG, biologics
Define GCA
medium/large systemic vasculitis
> older white demographic
what is GCA associated with
polymyalgia rheumatica
complication of GCA
vision loss (irreversible)
Presentation of GCA
unilateral headache
jaw claudication
blurred/double vision
temporal artery tender/thickened
assoc sx.
- shoulder/pelvic stiffness
- FLAWS
- muscle tenderness
- carpel tunnel syndrome
- peripheral oedema
Ix for GCA
Raised ESR
Temporal artery biopsy
Duplex USS (halo sign and stenosis of temporal artery)
Mx of GCA
40-60mg prednisolone (with no visual/jaw sx)
500mg-1000mg methylprednisolone (with visual/jaw sx)
can give
aspirin
PPI
bisphosphonates, calcium vitamin D
Define polymyalgia rheumatica
inflammatory condition causing pain and stiffness in shoulders, pelvic girdle and neck
what is PMR associated with
GCA
what demographic is PMR more common in
older white patients
presentation of PMR
rapid onset days-weeks of pain/stiffness in shoulders (radiate to upper arm and elbow), pelvic girdle (radiate to thighs) and neck
type of pain/stiffness in PMR
worse in morning
worse after rest/inactivity
interfere with sleep
takes 45 mins in the morning to ease
improves with activity
can also have
weight loss, fatigue, fever
muscle tenderness
carpal tunnel syndrome
peripheral oedema
how to diagnose PMR
clinical presentation, response to steroids, excluding differentials
inflammatory markers usually raised but could be normal
Mx of PMR
15mg prednisolone daily for 1-2 years
need dramatic improvement in sx within 1 week
Define systemic sclerosis
autoimmune connective tissue disease involving inflammation and fibrosis of connective tissues, skin and internal organs.
Name the types of systemic sclerosis
limited cutaneous systemic sclerosis
diffuse cutaneous systemic sclerosis
Sx of limited cutaneous systemic sclerosis
CREST
calcinosis
raynaud’s
oesophageal dysmotility
sclerodactyly
telangiectasia
Sx of diffuse cutaneous systemic sclerosis
CREST + CVS problems/lung problems/kidney problems
What is scleroderma
hardening of the skin
What is sclerodactyly
tightening of skin in hands
What is nailfold capillaroscopy
magnifies peripheral capillaries at nail bed. microhaemorrhages suggest systemic sclerosis
How to treat Raynaud’s
keep hands warm
CCB (nifedipine)
losartan, ACEi, sildenafil, fluoxetine
Autoantibodies in systemic sclerosis
ANA
Anti-centromere (limited)
ANti-Scl70 (diffuse)
Mx of systemic sclerosis
DMARDS (methotrexate)
biologics (rituximab)
Steroids
Define discoid lupus erythematosus
autoimmune chronic skin condition
F>M
20-50
darker skinned patients
smokers
increased risk of SLE
What can DLE rarely progress to
SCC
Presentation of DLE
photosensitive lesions on face, scalp and ears
scarring alopecia
hyper/hypopigmentation
lesions are inflamed, dry, red, scaling
Mx of DLE
skin biopsy to diagnose
SPF
topical steroids
intralesional steroid injections
hydroxychloroquine
Define SLE
inflammatory autoimmune connective tissue disorder
F>M
young-middle aged
asian, african, caribbean, hispanic
What type of condition is SLE
relapsing-remitting
Complications of SLE
cardiovascular disease and infection. chronic inflammation reduces life expectancy
Autoantibodies in SLE
ANA
Anti dsDNA
Anti-Sm
Antiphospholipid can occur secondary to SLE due to increased risk of VTE
Sx of SLE
- Serositis (pericarditis, pleuritis
- Oral ulcers
- Arthritis (non-erosive, arthralgia, myalgia)
- Photosensitivity
- Blood (all are low, anaemia, leukopenia, thrombocytopenia)
- Renal (proteinuria, glomerulonephritis)
- ANA
- Immunological (dsDNA etc.)
- Neurologic (psuch, seizures)
- Malar rash (photosensitive, sparing nasolabial fold)
- Discoid rash
Fatigue
Weight loss
Lymphadenopathy
Splenomegaly
Hair loss
Raynaud’s phenomenon
Ix for SLE
autoantibodies
CRP/ESR
C3 and C4 decreased in active disease
FBC
urinalysis
renal biopsy
Mx of SLE
SPF
1st line
hydroxychloroquine
NSAIDs
Steroids
more severe:
DMARDs (methotrexate)
biologics (rituximab, belimumab)
Define Ankylosing Spondylitis
inflammatory condition affecting axial skeleton causing progressive stiffness and pain
Name the seronegative spondyloarthropathies
AS
Psoriatic arthritis
Reactive arthritis
Joints affected in AS
sacroiliac joints
vertebral column joints
HLA B27 genes
Psoriatic arthritis
AS
IBD
Reactive arthritis
Sx of AS
20 y/o male
pain and stiffness in lower back
sacroiliac pain in buttock
improves with movement
pain worse at night and in the morning
takes 30 mins for pain to improve
additional
chest pain: costovertebal and sternocostal joints
enthesitis
dactylitis
vertebral fractures
SOB
AS associations
5As
Anterior uveitis
Aortic regurgitation
Atrioventricular block
Apical lung fibrosis
Anaemia of chronic disease
Test for AS
Schober’s test
Ix for AS
CRP, ESR
HLA B27
Xray of spine and sacrum (bamboo spine)
MRI spine
Mx of AS
1st NSAIDs
2nd anti TNF
3rd secukinumab/ixekizumab
3rd upadacitinib
can give steroid injections for specific joints
physio
Define Reactive arthritis
synovitis in one or more joints due to infective trigger (gastroenteritis, chlamydia)
causes acute monoarthritis
Associations of reactive arthritis
bilateral conjunctivitis
anterior uveitis
urethritis
circinate balanitis
“can’t see, pee or climb a tree)
Mx of reactive arthritis
exclude septic arthritis
joint aspiration
treat trigger
NSAIDs
steroid injection into affected joint
most cases resolve within 6 months
Define psoriatic arthritis
inflammatory arthritis assoc with psoriasis
can vary from mild stiffening of joints to arthritis mutilans
extra-articular manifestations of psoriatic arthritis
uveitis
IBD
5 patterns of psoriatic arthritis
- asymmetrical oligoarthritis (1-4 joints on one side of the body)
- symmetrical polyarthritis (like RA, more than 4 joints)
- DIP predominant pattern (DIP joints)
- Spondylitis (back stiffness and pain, axial skeleton)
- Arthritis mutilans (most severe, affecting phalanges. Osteolysis around joints and telescoping digit)
Signs of psoriatic arthritis
psoriasis plaques
nail pitting
onycholysis
dactylitis
enthesitis
xray changes in psoriatic arthritis
periostitis
ankylosis
osteolysis
dactylitis
pencil in cup in digits
Mx of psoriatic arthritis
NSAIDs
steroids
DMARDs
anti TNF
ustekinumab
define Rheumatoid arthritis
autoimmune condition causing chronic inflammation in synovial lining of joints, tendon sheaths and bursa
inflammatory arthritis
Signs of RA
affect multiple small joints (MCP, PIP, wrist, MTP) symmetrically
F > M
middle age
pain/stiffness/swelling
boggy joints
Gene associated with RA
HLA DR4
autoantibodies in RA
Rheumatoid factors
Anti CCP
Hand signs in advanced RA
Z shaped deformity of thumb
swan neck deformity (hyperextended PIP and flexed DIP_
Boutonniere deformity (hyperextended DIP and flexed PIP)
ulnar deviation at MCP joints
Extra articular manifestations of RA
- Pulmonary fibrosis
- Felty’s syndrome (a triad of rheumatoid arthritis, neutropenia and splenomegaly)
- Sjögren’s syndrome (with dry eyes and dry mouth)
- Anaemia of chronic disease
- Cardiovascular disease
- Eye manifestations
- Rheumatoid nodules (firm, painless lumps under the skin, typically on the elbows and fingers)
- Lymphadenopathy
- Carpel tunnel syndrome
- Amyloidosis
- Bronchiolitis obliterans (small airway destruction and airflow obstruction in the lungs)
- Caplan syndrome (pulmonary nodules in patients with rheumatoid arthritis exposed to coal, silica or asbestos dust)
Eye manifestations related to rheumatoid arthritis and its treatment include:
- Dry eye syndrome (keratoconjunctivitis sicca)
- Episcleritis
- Scleritis
- Keratitis
- Cataracts (secondary to steroids)
- Retinopathy (secondary to hydroxychloroquine)
Xray changes of RA
periarticular osteopenia
bony erosions
soft tissue swelling
joint destruction and deformity
Mx of RA
steroids short term
DMARDs and biologics
hyxroychloroquine can be used in mild disease
NSAIDs for pain relief
Side effects of methotrexate
Mouth ulcers and mucositis
Liver toxicity
Bone marrow suppression and leukopenia (low white blood cells)
Teratogenic and needs to be avoided before conception in both women and men for 3-6 months (ideally 6)
what to take to reduce adverse side effects of methotrexate
folic acid (once a week, can have daily just not on the same day as methotrexate)
Define Osteoarthritis
wear and tear in joints
joints affected in OA
Hips
Knees
Distal interphalangeal (DIP) joints in the hands
Carpometacarpal (CMC) joint at the base of the thumb
Lumbar spine
Cervical spine (cervical spondylosis)
Xray changes in OA
LOSS
L- loss of joints space
O- osteophytes
S- subchondral cysts (fluid holes in bone)
S- Subarticular sclerosis (increased denosity of bone at joint)
Signs of OA
pain/stiffness worse at the end of the day
Bulky, bony enlargement of the joint
Restricted range of motion
Crepitus on movement
Effusions (fluid) around the joint
Signs in the Hands
Heberden’s nodes (in the DIP joints)
Bouchard’s nodes (in the PIP joints)
Squaring at the base of the thumb (CMC joint)
Weak grip
Reduced range of motion
Mx of OA
exercise, weight loss
topical NSAID
Oral NSAID
weak opiate.paracetamol
intr-articular steroid injection
joint replacement
Define drug induced SLE
not all the typical features of systemic lupus erythematosus are seen, with renal and nervous system involvement being unusual. It usually resolves on stopping the drug.
Features of DILE
arthralgia
myalgia
skin (e.g. malar rash) and pulmonary involvement (e.g. pleurisy) are common
ANA positive in 100%, dsDNA negative
anti-histone antibodies are found in 80-90%
anti-Ro, anti-Smith positive in around 5%
Causes of DILE
most common:
procainamide
hydralazine
less common:
isoniazid
minocycline
phenytoin
