multi system autoimmune disease Flashcards
what are examples of connective tissue diseases ?
SLE scleroderma Sjorgen's syndrome auto-immune myositis mixed connective tissue disease
what are examples of systemic vasculitis ?
giant cell arteritis
granulomatosis polyangitis - Wegener’s
microscopic polyangitis
eoisinophillic granulomatosis polyangitis - Churg-Strauss
how are autoimmune diseases diagnosed ?
cardinal clinical features immunology imaging tissue exclusion of differentials
what can mimic an autoimmune disease ?
drugs - coke, minocyline, PTU infection - HIV, IE, hep, TB malignancy - lymphoma cardiac myxoma cholesterol emboli scurvy
who gets SLE ?
young people
F9:1M
afro-caribbeans>asians>whites
name any 4 classification criteria for SLE (diagnostic) ?
malar rash - butterfly discoid rash - alopecia, scarring photosensitivity oral ulcers arthritis serositis - pleurisy, pericarditis renal - proteinuria, casts neurological - seizures, psychosis haematology - low stuff, haemolytic anaemia immunity - low shit ANA
who gets scleroderma ?
F3:1M
30-50
what are features of scleroderma ?
morphea - grey blotches abdo raynauds syndrome RA tight skin digital ulcers
what are complications of scleroderma ?
limited - pulmonary hypertension
diffuse - pulmonary fibrosis, renal crisis, small bowel bacterial overgrowth
what are features of Sjogren’s syndrome ?
dry eyes and mouth
parotid gland enlargement
1/3rd have systemic upset - fatigue, fever, myalgia, arthralgia
what are complications of Sjogren’s syndrome ?
lymphoma neuropathy purpura interstitial lung disease renal tubular acidosis
what are features of auto-immune myositis ?
rare
muscle weakness - symmetrical, diffuse, proximal
polymyositis
dermatomyositis - Gottron’s papules, heliotrope rash
what are complications on auto-immune myositis ?
cancer
interstitial lung disease
what are overlap syndromes ?
soft tissue swelling
raynauds
myositis
arthralgia
what are the large vessel vasculitides ?
Takayasu arteritis
giant cell arteritis
what are the medium vessel vasculitides ?
polyarteritis nodosa
kawasaki disease
what are the small vessel vasculitides ?
ANCA - associated
what are the diagnostic criteria for GCA ?
3 of; age 50+ new headache temporal artery tenderness/reduced pulsation ESR 50+ abnormal temporal biopsy
what are features of granulomatosis with polyangitis (Wegener’s) ?
necrotising granulomatous inflammation
involves upper and lower respiratory tract
small to medium vessels
necrotising glomerulonephritis is common
what are features of microscopic polyangiitis ?
necrotising polyangitis, few or no immune deposits, small vessels
necrotising arteritis and small and medium vessels
necrotising glomerulonephritis is very common
pulmonary capillarities common
granulomatous inflammation absent
what are features of eosiniphillic granulomatosis with polyangitis (Churg Strauss) ?
eosinophil rich and necrotising granulomatous inflammation involving respiratory tract
necrotising vasculitis predominately involving small to medium vessels
associated with asthma
ANCA more frequent when glomerulonephritis present
what are common consequences of ANCA associated vasculitides ?
hearing loss nerve damage lung disease end stage renal disease malignancy
what is the specific ANA profile for SLE ?
dsDNA
Ro
Sm
what is the specific ANA profile for scleroderma ?
Scl-70
centromere
what is the specific ANA profile for polymyositis ?
Jo-1
what is the specific ANA profile for Sjogren’s disease ?
Ro
La
what are the different classes of lupus nephritis ?
1 - minimal mesangial 2 - mesangial proliferative 3 - focal 4 - diffuse 5 - membranous 6 - advanced sclerosing
how do you treat mild autoimmune disease ?
hydroxychloroquine
how do you treat moderate autoimmune disease ?
azathioprine
methotrexate
mycophenolate
how do you treat severe autoimmune disease ?
cyclophosphamide
rituximab
