connective tissue disease -rheumatology Flashcards
what is SLE?
chronic autoimmune disease mainly involving skin, joints, kidneys, white blood cells and nervous system
who gets lupus?
more common in woman and black people
fever weight loss fatigue swan neck fingers poor appetite malaise
SLE
muco-cutaneous things of SLE?
malar rash, photosensitivity, discoid lupus, oral/nasal ulcer
msk things for SLE?
myalgia
arthralgia
inflam arthritis
resp thing for SLE?
pleural effusion
pleurisy
PE
pulmonary hypertension
haem things for lupus?
anaemia
leukopenia
thrombocytopenia
neuro things for lupus/
seizures, headaches, depression
heart things for SLE?
pericarditis
ischaemic heart disease
are complement levels high or low in active lupus?
LOW
How do you manage the skin and joint damage in SLE?
DMARDs( hydroxychloroquine)
topical steroids
NSAIDS
what must you do in suspected lupus and why?
urinalysis to check for bloods on urine to check for renal involvement
what is Sjogrens syndrome?
autoimmune condition characterized by lymphocytic infiltrates in exocrine glands
dry eyes
dry mouth
dry vagina
Sjogrens syndrome
increased risk if lymphoma
sjogrens
positive ant-Ro and anti-LA and schirmers test
sjogrens
treatment for Sjorgens
saliva replacement
lubricating eye drops
hydroxychloroquine can help with joint pain and fatigue
what causes systemic sclerosis?
excessive collagen deposition
what can systemic sclerosis be divided into?
limited and diffuse
what is the difference between limited and diffuse sclerosis?
limited- tends to be confined to face, hands, forearms and feet
diffuse-changes devlop more rapidly and trunk is involved. early organ involvement
positive anti-centromere antibody and anti SCL-70
systemic sclerosis
management for systemic sclerosis?
digital ulcer/Raynauds- iloprost, calcium channel blockers
renal involvement-ACEi
GI involvement-proton pump inhibitors
how do manage mixed connective tissue disease?
treat the problem
what is anti-phospolipid syndrome?
a disorder where there is recurrent venous/arterial thrombosis and/or fetal loss
who is usually affected by anti-phospolipid syndrome?
young women
livedo reticularis
anti-phospolipid syndrome
management of anti-phospolipid syndrome?
life long anti-coagulation (low weight heparin if pregnant)
what is gout?
deposition of urate crystals within a joint
who is more likely to get gout?
males
what causes gout/
high serum uric acid levels- caused by too much alcohol, red meat and seafood and being on diuretics
deposition of crystals in a joint is triggered by what?
trauma
surgery
dehydration
what body part is mainly affected in joint?
big toe (first metatarsophalangeal joint)
can gout mimic septic arthritis?
yes
symptoms of gout?
intense pain red hot swollen can happen overnight very sudden onset
what is gouty tophi?
painless white accumulations of uric acid which can occur in soft tissue and can erupt through skin
crystals changing from yellow to blue
gout
prophylaxis for gout?
allopurinol
febuxostat
acute treatment for gout
NAIDS
colchicine
analgesics
steroids
what is pseudogout?
deposition of calcium phyrophosphate crystals in joint
what is pseudogout associated with?
OA
treatment for acute attack pseudogout/
NSAIDS
corticosteroids
occasionally colchicine
Milwaukee shoulder
hydroxyapatite
what is hydroxyapatite?
hydroxyapatite crystal deposition in joint. very rapid deterioration
treatment for hydroxyapatite?
NSAIDS
steroid injection
what is this?
it is often misdiagnosed as polymyositis patients tend to be>50 more common in men insidious onset distal muscle weakness weak wrist, finger flexors quads and anterior tibial CK levels lower than polymyositis
inclusion body myositis
inclusion bodies on muscle biopsy?
inclusion body myositis
what is polymalgia Rheumatica?
chronic inflammatory condition of unknown cause.
proximal myalgia of hip and shoulder girdles
headache
tender
jaw claudication
visual loss
temporal/giant cell arteritis
what is polymyalgia rheumatica associated with>
temporal/giant cell arteritis
tests for polymyalgia rheumatica ?
CRP/ESR/PV
temporal artery biospy
treatment for polymyalgia rheumatica
prednisolone 15mg daily (can use this a diagnostic tool as symptoms respond very fast!)
what is polymyositis?
inflammatory myopathy that causes symmetrical, proximal weakness
is there an increased malignancy risk with polymyositis?
yes
symmeterical, proximal muscle weakness in upper and lower extremities
insidious onset worsening over months difficulty with certain activities myalgia dysphagia may have diabetes or thyroid disease might be on steroids/statins alcohol/illicit drug abuser
polymyositis
definitive test for polymyositis?
muscle biopsy
management for polymyositis?
prednisolone 40mg and maybe methotrexate
what is dermatomyositis?
same as polymyositis but there is skin involvement e.f rashes
Grotten's sign (red joints in hand) Heliotrope rash (red circle around eye)
dermatomyositis
common cancers associated with dermatomyositis?
breast lung colon oesophagus bladder
what is fibromyalgia?
widespread muscle pain and fatigue
is there inflammation in fibromayalgia?
no
associated with IBS, depression and migraine?
Fibromyalgia
what is this?
persistent widespread pain and tenderness worse on exertion fatigue disrupted /shitty sleep pins and needles cognitive difficulties anxiety/depression
fibromyalgia
management for fibromyalgia?
graded exercise programme
antypical analgesia
what is vasculitis?
inflammation of blood vessels
2 main causes of large vessel vasculitis?
temporal (giant cell) arteritis Takayasu arteritis (TA)
who does TA mostly affect?
young women in 20’s/30’s
when might you hear carotid bruits?
giant cell arteritis
how to treat large vessel vasculitis?
prednisolone 40-60mg
methotrexate nay be added
features of small and medium vasculitis?
fever weight loss a raised , non blanching purpuric rash arthralgia/arthritis lung opacities on x ray
which type of small/medium vessel vasculitis involves this?
ENT symptoms- nose bleeds, deafness, recurrent sinusitis and nasal crusting
Granulomatosis with polyangitis (GPA)/ Wegeners
associated with cANCA and PR3
Granulomatosis with polyangitis (GPA)/ Wegeners
which type of small/medium vessel vasculitis involves this?
late onset asthma , rhinitis and raised eosinophil count and some neurological symptoms
Eosinophilic Granulomatosis with polyangitis (EGPA)
most important complication of microscopic polyangiitis?
glomerulonephritis
necrotizing vasculitis
microscopic polyangiitis
management for microscopic polyangiitis?
Iv steroids and cyclophosphamide
what is Henoch-Schonlein purpura?
IgA mediated disorder
who is commonly affected by Henoch-Schonlein purpura
children
what type of small/medium vessel vasculitis is this?
purpuric rash over buttocks and lower limb a history of upper resp tract infection abdominal pain bloody diarrhoea vomiting joint pain without swelling
Henoch-Schonlein purpura
