Multisystem autoimmune diseases Flashcards
List some connective tissue diseases
SLE scleroderma sjogren's autoimmune myositis MCTD
List some types of system vasculitis
giant cell arteritis
granulomatosis polyangiitis
microscopic polyangiitis
eosinophilic granulomatosis polyangiitis
Mimics of multisystem autoimmune disorders
drugs eg cocaine
infection eg HIV, TB
cardiac myxoma
scurvey
SLE epidemiology
disease of the young and females (9:1 with males)
15-50 years and afrocaribbean and Asian affected more than Caucasian
Talk through body systems and the symptoms of SLE
face - butterfly rash and photosensitivity
CNS - fits, ataxia and hemiplegia
alopecia
lungs - pleural effusion, pleurisy and fibrosis
heart - pericarditis, endocarditis and aortic valve lesion
general - fever and depression
skin - vasculitis and urticaria, raynauds
blood - anaemia, thrombocytopenia
myopathy, joint arthritis
How many of the classification criteria are required for an SLE diagnosis?
4
List the 11 classification criteria possibilities for SLE
malar rash discoid rash photosensitivity oral ulcers arthritis renal - proteinuria or cellular casts neurological - seizures serositis haematological eg low WCC immunological eg anti-dsDNA, sm la, Low complement ANA
Epidemiology of scleroderma
female 3: male 1
30-50 years
Name the 3 types of scleroderma
morphea
limited
diffuse
morphea
skin affected - localised
limited scleroderma
does not pass elbows Calcium Raynauds Eosophgeal dysmotility Sausage fingers Telengectasia
complications of limited scleroderma
pulmonary hypertension
Complications of diffuse scleroderma
pulmonary fibrosis
renal crisis - high bp
small bowel bacteria overgrowth
Sjogren’s epidemiology
40-50 years
female 9: male 1
Sjogren’s symptoms
CNS - fits, hemiplegia, cranial nerve lesions
GI - dysphagia and abnormal motility
resp - ILD
joints - arthralgia
kidney - renal tubular acidosis
skin - palpable purpura and reynauds
PNS - sensory neuropathy, mononeuritis simplex
DRY EYES AND MOUTH, parotid enlargement and 1/3 have systemic upset
5 complications of Sjogren’s
lymphoma renal tubular acidosis purpura neuropathy ILD
Incidence of auto-immune myositis
Rare - 6/million
Symptoms of myositis
muscle weakness - symmetrical, proximal, diffuse
2 types of myositis
polymyositis
dermatomyositis
2 skin changes with dermatomyositis
gottrons papules - 80%
heliotrope rash - 30-60%
complications of myositis
cancer and ILD
overlap syndromes (MCTD)
raynauds
soft tissue swelling
myositis
arthralgia
Example of large vessel vasculitides
GCA
example of small vessel vascultides
ANCA associated
giant cell arteritis - 3 of the following…
age of onset >50 new headache temporal artery tender/reduced pulsation ESR>50 abnormal temporal artery biopsy
3 types of ANCA associated vasculitis
- granulomatosis with polyangiitis
- microscopic polyangiitis
- eosinophilic granulomatosis with polyangiitis
GPA
necrotising granulomatous inflammation
URT and LRT
small and medium vessels
necrotising glomerulonephritis common
MPA
pulmonary capillaritis
necrotising glomerulonephritis, arteritis and vasculitis
few or no immune deposits
EGPA
eosinophilic rich and necrotising granulomatous inflammation - resp tract
necrotising vasculitis and ANCA
associated with asthma and eosinophilia
5 year damage with AAV
nerve damage, hearing loss, malignancy, lung and renal damage
condition and %ANA
MCTD and drug induced lupus - 100%
SLE - 99%
systemic sclerosis - 97%
sjogrens and myositis rarer
Conditions where positive ANA is not helpful
normal, infection, RA and MS
SLE ANA profiles
dsDNA, Ro, Sm
scleroderma ANA profiles
SCI-70, centromere
polymyositis ANA
jo-1
sjogrens ANA
Ro, La
6 classes of lupus nephritis and briefly describe
1- minimal mesangial 2- mesangial proliferation 3- focal 4- diffuse 5- membranous 6- advanced sclerosing
organ threat and treatment used
mild = hydroxychloroquine moderate = methotrexate,mycophenolate, azathioprine severe = cyclophosphamide, Rituximab
