MedEd AI and vasculitides Flashcards
what is sarcoidosis?
chronic multisystem granulamatous disorder of unknown cause
who is more likely to get sarcoidosis?
afro caribbeans
20-40 y/o females
what cells are present in sarcoidosis and what do they form?
T lymphocytes and macrophages which form non caseating granulomas
what granulmoas do you get in sarcoidosis? why
non caseating
there is no necrosis
how do granulomas differ between tb and sarcoidosis and explain why
tb= caseating because theres necrosis sarcoidosis= caseating because there is no necrosis
how does acute sarcoidosis present?
fever erythema nodosum polyarthralgia bilat hilar lymphadenopathy dry cough with prgressive dyspnoea
what type of cough do you get in sarcoidosis?
dry cough with progressive dyspnoea
what are extrapulmonary features of sarcoidosis?
organomegaly uveitis lupus pernio subcut nodules arrhythmia symptoms of hypercalcaemia
what colour is lupus pernio and where is it found?
violet macular rash found on the face
in what resp condition is lupus pernio seen?
sarcoidosis
out of lupus pernio and lupus vulgaris which is present in tb and which in sarcoidosis?
lupus pernio= sarcoidosis
lupus vulgaris= tb
what ix are done for sarcoidosis and what will you see? which ix is diagnostic
bloods- high esr, lymphopenia, hypercalcaemia, raised ACE
cxr- bilateral hilar lymohadenopathy
diagnostic- bronchoscopy with biopsy
what are the cxr stages of sarcoidosis?
stage 1= BHL
stage 2= BHL with infiltrates
stage 3= only infiltrates
stage 4= extensive fibrosis
how is sarcoidosis managed?
conservation- most patients with stage 1 will spontaneously resolve
may give steroids- indications inc parenchymal lung disease, uveitis, hypercalcaemia, neurologic or cardiac involvement
what are some indication for giving steroids in sarcoidosis?
parenchymal lung involvement
uveitis
hypercalcaemia
neurologic or cradiac involvement
what tb abx induces lupus?
isoniazid
what is sle? describe what happens
a multisystemic relapsing remitting AI disease where a wrongful response to host antigens leads to immune complex formation. a lack of clearance of these complexes then causes tissue damage and inflammation
what is formed in SLE?
immune complexes
what type of hypersensitivity reaction is present in SLE?
type 3
what genes are associated with SLE?
HLA DR3
also HLA B8/DR2
what is the the trigger for formation of autoimmune complexes in SLE?
host antigens
how does SLE present?
malar rash discoid rash oral ulcers arthritis serositis renal disorder hematologic disorders immunological disorders antinuclear disoders
what drugs cause SLE?
sulfalazine hydralazine isoniazid procainamide penicillamine
what ix are done for SLE and what will you see?
bloods- anaemia, lymphopenia, thrombocytopenia
ESR high
CRP normal
autoantibodies: ANA, anti ds-DNA, anti histone
there may be lupus nephritis on renal biopsy
what are ESR and CRP levels in SLE?
ESR= raised CRP= normal
how is SLE managed?
maintanence= NSAIDs and hydrocychloroquine for joint and skin symptoms
mild flare= low dose steroids
moderate flaer= DMARDs or mycophenolate
severe flare= high dose steroids, mycophenolate, cyclophosphamide, rituximab
what is the difference between CRP and ESR and therefore when will either of them be raised?
CRP= rises when there is acute inflammation, ischaemia or thromboembolism ESR= increases in a slower manner and then remains raised so with be high in chronic conditions
what does high CRP normal ESR indicate?
acute infection, inflammation or ischaemia
what does high ESR normal CRP mean?
chronic systemic inflammatory or autoimmune process
what autoantibodies are associated with SLE?
ANA
anti dsDNA
what is sjogrens syndrome?
chronic inflammatory disorder where lymphocytic infiltration destroys exocrine gland and causes extensive fibrosis
what glands are affected in sjogrens syndrome?
exocrine- mainly lacrimal and salivary
who is more likely to get sjogrens syndrome?
middle aged women
how does sjogrens syndrome present?
dry eyes- keratoconjucntivis sicca dry mouth- xerostomia parotid swelling polyarthritis raynauds phenomenon vasculitis peripheral neuropthy
what is keratoconjunctivits sicca? what condition is it seen in
dry eyes
seen in sjogrens syndrome
what is xeroztomia? what condition is it seen in
dry mouth
seen in sjogrens syndrome
what ix are done for sjogrens syndrome? describe how they are carried out and what is positive
schrimers test- place a piece of paper under the eyelid for 5 mins and if less than 5mm of paper is wet in less than 5 mins this is positive
autoantibodies- anti ro and anto la (are not specific enough)
can do salivary gland biopsy
what is schrimmers test? how is it carried out and which condition is it used for?
place a piece of paper under the eyelid for 5 mins and if less than 5mm of paper is wet in less than 5 mins this is positive for sjogrens syndrome
how is sjogrens syndrome managed?
dry eyes= hypromellose (fake tears) and eye drops
dry mouth= salivary substitutes (sugar free gum)
severe disease= immunosupressants (steroids)
what is systemic sclerosis? describe what happens
multisystemic autoimmune disease where there are functional and structural abnormalities of non inflammatory blood vessels, fibrosis of the skin and internal organs
what is limited systemic sclerosis and how does it present?
systemic sclerosis that is limited to the hands, face and feet
presents with calcinosis cutis, raynauds, oesophageal dysmotility, sclerdactyly and telangiectasia
what is diffuse systemic sclerosis and how does it present?
systemic sclerosis with widespread organ involvement
there is rapid progression and early visceral involvement and BP needs to be meticulously monitored
how does systemic sclerosis present?
puffy taut skin with no wrinkles and finger tip pitting
renal- scleroderma hypertensive crisis
pulmonary- interstitial fibrosis, PH
GI- dysmotility, reflux and watermelon stomach
cardio- pericarditis or effusion
what autoantibodies are associated with systemic sclerosis?
anti centromere in limited type
anti scl 70 and anti RNA polymerase III in diffuse type
what type of systemic sclerosis are anti centromere antibodies associated with?
limited
what type of systemic sclerosis are anti scl 70 and anti RNA polymerase III antibodies associated with?
diffuse
how is systemic sclerosis managed?
immunosupression and treat individual symptoms
in renal crisis ACE-I or ARB with agressive hydration
what are poly/dermatomyositis?
insidious onset progressive symmetrical proximal muscle weakness (quads, triceps, biceps) due to autoimmune mediated inflammation of straited muscle
what muscles are weakened in poly/dermatomyositis?
proximal muscles like quads, biceps, triceps etc
what cancer is dermatomyositis associated with?
ovarian
what type of inflammation is present in polymyositis?
endomysial inflammation
what type of inflammation is present in dermatomyositis?
perimysial
what cells are involved with polymyositis?
CD8+ T cells
what cells are involved with dermatomyositis?
CD4+ T cells
what muscle group does polymyositis often affect?
shoulder girdle
how does polymyolitis present?
progressive symmetrical muscle weakness
how does dermatomyositis present?
progressive symmetrical muscle weakness grottons papules facial erythema shawl rash mechanics hands
what are grottons papules?
rough red papules across knuckles, knees and elbows
what is shawl rash?
shoulders and chest darkening
what are mechanics hands in medicine?
dark and thick irregular patches
what ix are done for poly/dermatomyositis and what will you see?
CK and adolase enzymes are elevated due to muscle breakdown
EMG shows fibrillation potentials
msucle biopsy to confirm diagnosis and visualise inflammation
autoantibodies; anti srp, anti mi2, anti jo1
how are poly/dermatomyositis managed?
corticosteroids
what is giant cell arteritis?
Large vessel granulomatous vasculitis
what is polymyalgia rheumatica?
inflammatory condition which manifests as pain and morning stiffness in the neck, shoulder and hip girdle
what vessel is commonly affected in giant cell arteritis?
external carotid
who usually gets giant cell arteritis?
elderly females
how does giant cell arteritis present?
unilateral headache sclap tendrness tongue/jaw claudication amaurosis fugax unilateral blidness
what is amaurosis fugax?
vision gradually going away
how does polymyalgia rheumatica present?
weakness is not a feature subacute onset (2 weeks) of bilateral aching, tenderness and morning stiffness fever weight loss anorexia
what is not a feature of polymyalgia rheumatica and helps distinguish it from other conditions?
weakness
what ix are done for giant cell arteritis and what do you see/
raised ESR
raised CRP
normocytic anaemia
temporal artery biopsy but only after management- will show focal granulomtous inflammation
what ix are done for polymyalgia rheumatica and what will you see?
raised ESR
raised CRP
in giant cell arteritis what do you do first and second?
first give steroids
second do ix like bloods
how is giant cell arteritis managed?
high dose methylprednisolone if blindness is starting
prednisolone if suspected
how is polymyalgia rheumatic managed?
give steroids immediately and there should be a response within a week
also give bone protection as steroids will be needed for at least 2 years
in giant cell arteritis when is methyprednisolone given and when is normal prednisolone given?
methylprednisolone= given if blindness has started
normal prednisolone= give if disease is suspected
what is bechets disease?
systemic inflammatory vasculitis
what gene is bechets disease associated with?
B51
where is bechets disease found and who is most likely to have it?
old silk road along mediterrean to china
most likely a turkish male
how does bechets disease present?
recurrent oral ulcers genital ulcerations erythema nodosum thrombophlebitis uveitis
what ix are done for bechets disease and what will you see?
usually diagnosis is clinical
can also perform pathology testing by sticking a needle into their arm and a papule will form in 48 hrs
how is bechets disease managed?
topical steroids for orogenital ulceration
prednisolone plus immunosupressant (azathioprine) if there is organ involvement
what is polyarteritis nodosa?
necrotising inflammation of medium sized arteries
aneurysms lead to thrombosis and infarction
what virus can cause polyarteritis nodosa?
HBV
how does polyarteritis nodosa present?
systemic features
skin- rash, punched out ulcers, nodules
renal- artery narrowing, ischaemia and hypertension
livedo reticularis
what is livedo reticularis? what does it look like? in what condition is it seen?
a skin symptom where there is mottled appearance of blood vessels often on the leg
can be seen in polyarteritis nodosa
what ix are done for polyarteritis nodosa and what is seen?
FBC- high WCC
HBV serology
ESR and CRP high
MRA- shows string of pearls appearance due to micoraneurysms and spasm
renal biopdy might show transmural inflammation and necrosis
how is polyarteritis nodosa managed?
prednisolone and if its severe then a DMARD
