autoimmune tings Flashcards
what is systemic lupus erythematous
cells become damaged usually from UV
cells die and release DNA and histones, nuclear antigens, immune cells recognise these and B cells create anti-nuclear antibodies forming immune complexes that can deposit anywhere in the body causing local inflammation and reducing self tolerance
what causes SLE
genetics
what are risk factors for SLE
female
pregnancy
how can SLE present
fever rash joint pain serotitis ulcers photosensitivity rash discoid rash renal disease raynaud's butterfly rash neurological symptoms anaemia alopecia arthritis autoantigens, ANA ANA Anti-smith
what investigations would you do for SLE
urine dipstick - check for renal impairment
renal biopsy
C3 and C4 levels
FBC
CRP and ESR
antibody tests -antinuclear-antibodies, anti-dsDNA antibodies
how can you manage SLE
prevent flare ups - suncream corticosteroids - prednisolone hydroxychloroquine immunosuppressants - methotrexate NSAIDS biologic agents in severe disease
what is systemic sclerosis
connective tissue disease
fibrosis of skin and internal organs
changes in microvasculature
what are the types of sclerosis
diffuse cutaneous
limited cutaneous/CREST
what causes sclerosis
environment
genetics
what are risk factors for sclerosis
female
30-50
how does localised sclerosis present
only skin distal to the elbows and knees is involved CREST calcinosis raynaud's oesophageal dysmotility sclerodactyly telangiectasias should plateau in 5 years Positive ANA
how does diffuse sclerosis present
fast progression
interstitial lung disease
cardiac and renal involvement
how can you manage sclerosis
no cure
try and slow progression with immunosuppressants
manage symptoms
for localised reassure patient it won’t progress to systemic and refer to dermatology
what is sjogren’s syndrome
autoimmune infiltration of salivary and lacrimal glands
glandular tissue is replaced by lymphocytes so the glands can no longer secrete as much saliva/tears
what can cause sjogren’s syndrome
usually follows infection
usually women
Anti Lo, Anti La.
what are the symptoms of sjogrens
dry mouth dysphagia dry eyes sore red eyes feeling of sand in eyes difficulty tasting dryness of skin and vagina neuro symptoms ILD arthralgia
what are the two types of sjogren’s disease
primary - occurs in isolation
secondary - related to SLE or RA
what investigations would you do for sjogrens
saliva test - should produce 15ml/min
sjogrens/schirmir test (tears) - less than 10mm is significant 15mm is normal
biopsy
need occular and oral dryness for at least three months for diagnosis
how would you manage sjogrens
no treatment
manage with eye drops and moisturiser etc
hydroxychloroquine - slows progress
what is giant cell arteritis
autoimmune infiltration of arteries usually temporal arteries
systemic vasculitis
causes hyperplasia and occlusion
what are risk factors for GCA
female
white
70+
polymyalgia rheumatica
how does GCA present
sudden onset painful headache scalp tenderness jaw claudication blurred or double vision painless blindness fatigue muscle ache peripheral oedema tongue necrosis
what investigations could you do for GCA
temporal artery biopsy
doppler
CT or MRI angiogram
ESR blood test
how do you manage GSA
immediate high dose prednisolone 40-60mg
aspirin
PPI to protect stomach from steroids
bisphosphonate to protect bone
what is wegener’s syndrome/granulomatosis with polyangiitis
Small blood vessel vascilutus
ANCA-associated disease
necrotising granulomatous inflammation usually in the lungs (respiratory system and kidneys)
how does wegener’s present
haemoptysis - from ulcers in lungs sinusitis epistaxis wheeze bloody mucous hearing loss rapidly progressing glomerulonephritis.
what are clinical signs of wegeners
collapsed nasal bridge
can effect kidneys and cause glomerulonephritis and patient will have oliguria and hypertension
what investigation can you do for wegener’s
x-ray - look for cavitating lesions in lungs
how do you manage wegeners
corticosteroids
cyclophosphamide (immunosuppresant)
what is churg-strauss syndrome/eosinophilic granulomatosis with polyangiitis
Small and medium vessel vasculitis.
ANCA-associated
inflammation of blood vessels with white blood cells and necrotising granulomas usually in respiratory tract
it can affect lungs, skin or kidneys
how does churg-strauss syndrome present
similar to late onset asthma nasal polyps sinusitis GI involvement neuro involvement lung and kidney damage
what investigations could you do for churg-strauss syndrome
bloods - WBCs
biopsy
may be anti-MPO +ve
how can you manage churg-strauss syndrome
no cure
try manage symptoms
what is polymyalgia rheumatics
inflammatory condition
associated with giant cell arteritis
what age group is most likely to have polymyalgia rheumatics
50+
caucasian
female
how does polymyalgia rheumatics present
symptoms must be > 2 weeks. shoulder or pelvic girdle most commonly effected - bilateral stiffness muscle ache joint pain malaise weight loss fever anaemia
what investigations could you do for polymyalgia rheumatics
rule out giant cell arteritis
ESR > 50
DEXA for patients over 65 with osteoporosis risks and all patient under 65
how do you manage polymyalgia rheumatica
be aware of GCA
give steroids - prednisolone
bone protection with bisphosphonates if over 65 with risk factors
IF its polymyalgia rheumatica - there will be a good response to steroids. should be reduced gradually.
What is the key investigation done for GCA?
ESR - will be >50mm/hour.
Ultrasound - will show halo sign.
Biopsy - will show multinucleate giant cells.
What is the main complication of GCA?
painless vision loss - permanent
cerebrovascular stroke
relapses of condition
steroid side effects
aortitis
What is elevated in churg Strauss syndrome?
raised eosinophils.
How is SLE characterised?
Anti-nuclear antibodies.
