rheum Flashcards
osteoarthritis
at synovial joints (hips, knees, DIP, MCP)
cartilage worn down
presntation OA
joint pain and stiffness worse with activity
weakness
decreased ROM
XR OA
loss of joint space
osteophytes
subarticular sclerosis
subchondral cysts
hand signs OA
haberdens nodes at DIP
bouchards nodes at PIP
squaring at CMC thumb
diagnosis OA
> /= 45
pain on activity
morning stiffness < 30m
mx OA
wt loss
PT/OT
analgesia: paracetamol, topical nsaids, orial nsaids, opiates
intra-articualr steroid injection
joint replacement
RA
AI
chronic inflammation of synovial lining, tendon sheaths and bursa
genes associated with RA
HLA DR4
DLA DR1
Ab associated with RA
rheumatoid factor
anti-citrullinated cyclic peptid Ab (more snesitive and specific)
presentation RA
symmetrical distal polyarthropathy small joints (PIP, MCP, MTP, wrist, ankle, c-spine)
pain, swelling, morning stiffness, improves with acitvity
systemic sx
XR features RA
joint destruction and deformity
soft tissue swelling
periarticualr osteopenia
bony erosions
palindromic rheumatism
inflammatory arthritis lasting 1-2d
antlantoaxial subluxation
can occur due to RA
can cause cord compression
RA signs in hands
feel boggy
z shaped thumb
swan neck (hyperextended PIP and flexed DIP)
boutonnieres (hyperextended DIP with flexed PIP)
ulnar deviation MCP
swan neck deformity
hyperextended PIP and flexed DIP
boutonnieres deformity
hyperextended DIP with flexed PIP
extra-articular manifestations RA
pulmonary fibrosis with pulmonary nodules (caplans)
bronchiolitis obliterans
feltys (RA, neutropenia, splenomegaly)
secondary sjogrens (sicca)
anaemia
CVD
eyes: scleritis, episcleritis, keratitis, keratoconjunctivitis)
rheumatoid nodules
lymphadenopathy
carpal tunnel
amyloidosis
feltys syndrome
(RA, neutropenia, splenomegaly
ix RA
RF and anti CCP
CRP, ESR
XR
DAS-28 or HAQ
mx RA
- monotherapy: methotrexate, leflunomide, sulfasalazine, hydroxychloroquine
2.combine - methotrexate and biological therapy (infliximab)
- methotrexate and rituximab
steroids for flare ups
consider surgery
methotrexate
affects folate metabolism so need folate supplements
SE methotrexate
mouth ulcers
liver toxicity
pulmonary fibrosis
leukopenia and BM suppression
teratogenic
leflunomide
interferes with production pyrimidine
leflunomide se
mouth ulcers
HTN
rash
peripheral neurpathy
liver toxicity
leukopenia and BM suppression
teratogenic
sulfasalazine
immunosuppressive and anti-inflammatory
sulfasalazine SE
temporary M infertility
BM suppression
hydroxychloroquine
interferes with toll like receptors
SE hydrpxychloroqeuin
nightmares
decreased visual acuity
liver toxicity
skin pigmentation
types anti-TNF
biologics: monoclonal Ab e.g. infliximab
proteins: etanercept
SE anti-TNFs
more severe infections
reactivation TB/hep B
rituximab
monoclonal Ab to CD20
SE rituximab
more severe infections
night sweats
thrombocytopenia
peripheral neuropathy
liver and lung toxicity
psoriatic arthritis
seronegative spondyloarthropathy
psoriasis and arthritis
features psoriatic arthritis
symmetrical, asymmetrical, spondylitis
nail pitting, oncholysis, dactylitis, enthesitis
conjunctivitis, anterior uveitis,
aortitis
amyloidosis
screening for psoriatic arthritis
PEST
mx psoriatic arthritis
nsiads
dmards
anti-tnfs
ustekinumab (targets Il12 and 23)
XR in psoriatic arthritis
periostitis
ankylosis
osteolysis
dactylitis
‘pencil in cup’ appearance
arthritis mutilans
severe psoriatic arthritis
osteolysis in phalanxes->shortening =telescopic finger
reactive arthritis
reiters: acute monoarthritis triggered by infection
common infections triggering reactive arthritis
gastroenteritis
STI - chlamydia
gene assicaited with reactive arthritish
HLA B27
sx reactive arthritis
bilateral conjunctivitis
anterior uveitis
arcinate balanitis
mx reactive arthritis
need to rule out septic arthritis
nsaids
steroid injections
dmards
where is ankylosing spondylitis
sacroiliac joints and vertebral column
test for ankylosing spondylitis
schobers
main sx ankylosing spondylitis
back pain
gene associated with ankylosing spondylitis
HLA B27
associations with ankylosing spondylitis
wt loss
chest pain
enthesitis: plantar fasciitis
dactylitis
anaemia
anterior uveitis
aortitis, heart block
restrictive lung disease, pulmonaery fibrosis
IBD
XR ankylosing spondylitis
bamboo spine
squared vertebral body
subchondral sclerosis
syndesmophytes
ossification
joint fusion
mx ankylosing spondylitis
nsaids
steroids
anti-tnf
secukinumab
who is SLE more common in
F
asian
what is SLE
inflammatory AI connective tissue disease
sx SLE
non specific
photosensitive rash
fatigue
arthralgia
mouth ulcers
raynauds
ix SLE
ANA
anti dsDNA
anaemia
decreased C3 and 4
increased CRP and ESR
Ig
proteine:creatinine urine ratio
mx SLE
nsaids
pred
hydroxychloroquine
methotrexate
rituximab
complications SLE
CVD
infection
anaemia
pericarditis
pleuritis
interstitial lung disease
lupis nephritis
neuropsychiatric SLE
recurrent miscarriage
VTE
discoid lupus erythematosus
photosensitive erythematous skin lesions on face, ears, scalp
associated with scarrin galopecia
ix discoid lupus erythematosus
skin biopsy
mx discoid lupus erythematosus
suncream
steroids
hydroxychloroquine
systemic sclerosis
AI inflammatory and fibrotic connective tissue disorder
types systemic sclerosis
limited cutaneous
diffuse cutaneous
mx systemic sclerosis
steroids and immunosuppresants
auto ab in all types systemic sclerosis
ANA
sx limited cutaneous systemic sclerosis
CREST syndrome:
calcinosis, raynauds, oesophageal dysmotility, sclerodactyly, telangiectasia
auto ab in limited cutaneous systemic sclerosis
anti centromere ab
featuers diffuse cutaneous systemic slcerosis
CREST syndrome (calcinosis, raynauds, oesophageal dysmotility, sclerodactyly, telangiectasia)
CV: HTN, CVD
lung: pulmonary HTN, fibrosis
kindey: glomerulonephritis, scleroderma renal crisis
auto ab in diffuse cutaneous systemic sclsrosis
anti-scl-70
what is polymyalgia rheumatic associated with
GCA
features polymyalgia rheumatica
2w bilateral shoulder pain to elbow, bilateral pelvic girdle pain, 45m morning stiffness, worse with movement
systemic sx
pitting oedema
increased inflammatory markers
mx polymyalgia rheumatic
steroids (15mg pred) then wean down - can take 1-2y
GCA
temporal arteritis
systemic vasculitis of medium and large arterires
features GCA
severe unilateral temporal headache
scalp tenderness
jaw claudication
blurred/loss of vision
fever
ache
ix GCA
increased ESR]
temporal artery biopsy=multinucleated giant cells
normocytic anaemia
increased ALP, CRP
USS=hypoechoic halo
mx GCA
40-60mg pred
aspirin
biopsy
complications GCA
early=visual loss, stroke,
late=relapses, stroke, aortitis (->aneurysm/dissection)
polymyositis
inflammation muscles
anti-Jo-1 Ab
dermatomyositis
inflammation muscles and skin
anti-mi-2, ANA
features specific to dermatomyositis
gottron lesions on knucles, elbows and knees
photosensitive erythematous rash
purple rash
periorbital oedema
subcutaneous calcinosis
features polymyositis and dermatomyositis
increased CK
bilateral muscle pain and weakness-often shoulder and pelvic girdle
mx polymyositis and dermatomyositis
corticosteroids
antiphospholipid syndrome
hypercoagulable state
often secondary to SLE
auto ab in antiphospholipid syndrome
antiphospholipid ab
lupus anticoag
anticardiolipin
anti-B-2-glycopreotein
features antiphospholipid syndrome
VTE
MI
stroke
pregnancy complications
livedo reticularis (purple lace rash)
libmannn sacks endocarditis
thrombocytopenia
mx antiphospholipid syndrome
warfarin
sjogrens syndrome
AI condition exocrine glands leading to dry mucous membranes
can be secondary to SLE/RA
auto ab in sjogrens
anti-Ro
anti-La
test for sjogrens
schirmers
mx sjogrens
artificial tears/saliva
lubriants
hydroxychloroquine
complications sjogrens
conjunctivitis
corneal ulcers
dental cavitis
candida
small vessel vasculitis
HSP
eosinophilic granulomatosis with polyangitis-churg strauss
microscopic polyangitis
granulomatosis with polyangitis=wegners granulomatosis
HSP features
IgA
purpura
joint pain
renal invovlement
abdo pain
<10y
mx HSP
supportive
features eosinophilic granulomatosis with polyangitis
p-ANCA
lung and skin problems-asthma
increased eosinophils
features microscopic polyangitis
renal failure
sob
haemoptysis
p-ANCA
features granulomatosis with polyangitis
c-ANCA
resp tract: epistaxis sinusitis
lungs: cough, wheeze, haemoptysis
glomerulonephritis
medium vessel vasculiits
polyarteritis nodosa
churg strauss
kawasaki
features polyarteritis nodosa
hep B (+hep c and HIV)
renal impairment
stroke
MI
livedo reticularis
features kawasaki
fever >5d
erythematous rash
strawberry tongue
<5y
risk coronary artery aneurysm
mx kawasaki
aspirin
IV Ig
large vessel vasculitis
GCA
takayasus
features takayasus
effects aorta (PA->swell and block=pulselses diseease)
<40y
fever
aches
syncope
mx vasculitis
steroids
immunosuppressants (cyclophosphamide, methotrexate)
what is behcets disease
relapsing remitting inflammatory disease
HLA B51
features behcets disease
mouth and genital ulcers
skin: papules, erythema nodosum
eyes: uveitis, vasculitis
MSK: stiff, arthralgia
GI
CNS: memory, headaches
veins: inflammed, VTE
PA aneurysm
behcets ix
pathergy test
mx behcets
steroids
colchicine
azathioprine
inliximab
gout features
urate crystals in joint (MTP, wrist, CMC) -> pain/red/swollen +/- gouty tophi in DIPgo
gout RF
M
obese
meat
seafood
alcohol
diuretics
CVD
fhx
ix gout
aspirate=needle shaped negatively birefringent
monosodium urate crystals
gout XR
lytic lesions
punched out erosions with sclerotic border
mx gout
acute=nsaids, colchicine
prophylacis-allopurinol (xanthine oxidase i)
features pseudogout
calcium pyrophosphate crystals ->chondrocalcinosis-hot/swollen/stiff knee/shoulder/wrist/hi
ix psuedogout
aspirate=positively birefringent rhomboids
XR pseudogout
chondrocalcinosis (white line in joint space)
loss joint space
osteophytes
subarticular sclerlosis
subchondral cysts
mx pseudogout
nsaids
colchicine
steroids
joint washout
RF osteoporosis
age
F
less mobile
BMI <18.5
RA
alcohol
smoking
long term steroids
SSRI
PPI
anti epileptics
anti oestrogens
post menopausal
risk osteoporosis
FRAX - risk # in nect 10y
osteoporosis ix
DEXA
z-score is age adjusted
t-score is sd below young: -1 to -2.5 is osteopenia, <-2.5 is osteoporosis, <-2.5 and # is severe osteoporosis
mx osteoporosos
vit d and ca (calcichew d3)
bisphosphonates e.g. alendronate, zolendronic acid
denosumab (monoclonal ab)
how to bisphosponates work
decrease osteoclast activity
SE bisphosphonates
reflux
atypical #
osteonecrosis
osteomalaacia
defective bone mineralisation due to vit d deficiency = soft bones
features ostemomalacia
fatigue
pain
weakness
#
what is rickets
osteomalacia before growth plates close
ix osteomalacia
low serum 25-hydroxy vit d
low ca
low phos
high ALP
high PTH
XR=radiolucent bones
mx osteomalacia
vit d -colecalciferol
pagets disease
excessive bone turnover (increase osteoclast and osteoblast activity)=pathy sclerosisand lysis=bone pain, deformity, #
ix pagets
increased ALP
XR=deformity, osteroporosis, circumscripta, cotton wool skull, v shaped long bones
mx pagets
bisphosphonate
complications pagets disease
osteosarcoma
spinal stenosis and cord compression
where does pagets often effect
axial skeleton
What is inflammatory response driven by
Cytokines
Features inflammatory response and cause
Hot: capillary widening leading to increased blood flow
Red and swollen: Increased permeability leading to fluid release
Tender: leukocytes go to injury site
Pain: systemic response leading to fever and more leukocytes
Normal Inflammation pathway
Macrophages activated (for phagocytosis)
Release IL-1
Activate other macrophages
Response shut down by NK cells and CTL
What happens when the immune system doesn’t switch off in inflammation
Continuous macrophage activation
Cytokine storm/hyperinflammation leading to organ damage
Cytokine storm/HLH triggers
Infection: viral, coronations
Cancer: often haem
AI
immunotherapy
Individual susceptibility
What is HLH
Haemophagocytic lymphohistiocytosis
Immune disregulation
Hyperinflammation
Features HLH
Fever
Falling blood counts
Ferritin highly elevated (>10,000)
Unwell patients and CRP falling
High TG, reduced fibrinogen, abnormal LFTs)
What to do if suspect HLH
Quick screen: FBC, TG, U &E, LDH, LFT, ferritin, fibrinogen, clotting
Review imaging: hepatosplenomegaly , lymphadenopathy
H- score: if over 169 give 1g IV methylpred, MDT, rheum, haem, HDU
Consider triggers
Mx HLH
Steroids: IV methylpred, treats trigger, may be enough to tx if catch early
If no response 48h: anakinra (blocks IL1), etoposode (proapoptotic), tocilizumab/baricitimab (block IL6 and JAC - used in covid 19)
feltys syndrome
(RA + splenomegaly + low white cell count)
SE and risks nsaids
Peptic ulcer
Renal failure
Exacerbate asthma
MI and stroke- particularly diclofenac
Aim urate lowering therapy
Uric acid under 300
Febuxostat
Xanthine oxidase inhibitors
2nd line urate lowering therapy in gout due to cost
SE DMARDS
Suppress bone marrrow: low WCC AND platelets
Abnormal LFTs
Mouth ulcers
Hair loss
Nausea
Diarrhoea
Teratogenic other than sulfasalazine and azathioprine
Monitoring DMARDS
FBC, U AND E, LFT
Initially monthly for 3m, then every 3m
Methotrexate
6-8w to work
Anti folate therefore need folic acid
Restric alcohol
Need 3m off before pregnant
Leflunomide
Less well tolerated than methotrexate due to GI SE
Sulfasalazine
Well tolerated but less effective
Anti TNF drugs
Infliximab
Etanercept
Adalimumab
Certolizumab
Golimumab
How are biological drugs given
INFUSION or injection
Can’t take orally as would destroy
Rituximab
Anti CD20 = ANTI b cell
Abatercept
Anti t cell
Tocilizumab
Anti IL6
JAK i
Baricitinib
Tofacitinib
Oral as work on intracellular pathway
SE anti TNF
Increased risk infection and work infection
Ab against so reduced effect
Infection injection site
Increase risk ca - skin
What is rituximab good for
Ab mediated disorders
Important SE riuximab
Reduces Ab response to vaccines, as given 6m -18mnthly usually give vaccine then wait 6w before give rituximab
SE tocilizumab
Reduces CRP so it won’t rise in infection
Are JAK I biologics
No!
How to biological work
Outside cekk to stop cytokines binding to receptors
JAK i SE
Short half life in comparison to biologics
Increased risk infection - shingles
Increased risk DVT and PE
Possible risk malignancy
sx allopurinol sensitivity
lymphadenopathy
eosinophilia
fever
skin rash
medications causing lymphadenopathy
allopurinol
phenytoin
atenolol
carbamazepine
sx dermatomyositis
unwell
arthralgia
wt loss
heliotrope rash
purple scaly patches on elbows
muscle weakness - particularly proximal upper limbs
blood results dermatomyositis
ANA positive
CK: very high - 50x normal
anti-mi-2 ab
