C - Immune related multisystem disorders (E- Amir Sam) Flashcards
Sx of SLE
skin - malar rash / discoid
oral ulcers
joints
neuroloigcal - psychosis / depression
serositis
renal - glomerularnephritis
haematological - pancytopaenia
immunological
AB for SLE
anti dsDNA
anti smith
anti histone
which SLE pts gets anti histone ABs
drug induced SLE
what % of SLE pts have anti smith ABs
30% (but its very specific for SLE)
what protazoa is used to help diagnosis of SLE and how
crithidia luciliae
- has double stranded DNA so the SLE pt’s ABs bind to it
why do SLE pts get a malar rash
lymphocytic infiltration of basal epidermis
immune complex deposition at epidermis-dermis junction
kidney histology in SLE
Y loop capillaries - thickening of glomerular capillary walls
immune complex deposition in BM of capillary wall
what type of endocarditis can you get in SLE
Libman - Sacks (non infective endocarditis caused by immune complex deposition)
what is scleroderma (systemic sclerosis)
fibrosis and excess collagen deposition in skin
2 forms of scleroderma
diffuse
limited
ABs in diffuse scleroderma
DNA topoisomerase (Scl70)
ABs in limited scleroderma
anti centromere
Sx of limited scleroderma
CREST
calcinosis
raynauds
eosophageal dysmotility
sclerodactyly
telangiectasia
difference between diffuse and limited scleroderma clinically
involvement of trunk
- tight skin over trunk = diffuse
- no tight skin over trunk = limited
pattern of immunoflurescence in scleroderma
nucleolar
histology of artery in scleroderma
onion skinning - intimal proliferation (narrowed lumen)
mixed connective tissue disease has features of which other disorders
SLE
scleroderma
polymyositis
dermatomyositis
immunoflurescence pattern of mixed connective tissue disease
speckled pattern
finger sign of dermatomyositis
Gottron’s papules
- erythema over distal IPJ
Sx of sarcoidosis
joints - arthralgia
skin - nodules and papules, Lupus pernio, erythema nodosum
lung - fibrosis, BHL
lymphadenopathy
heart - pericarditis, valvular lesions
eyes - uveitis, conjunctivitis
neuro - meningitis, CN
liver - hepatitis, cirrhosis
bilateral parotid enlargement
pathological hallmark of sarcoidosis
non caseating granulomas
what is lupus pernio
erythematous lesion involving nose
causes of erythema nodosum
infection
inflammation - IBD, Behcets, sarcoidosis
what is a granuloma
ball of lymphocytes
what type of cell is seen in granuloma that is a fusion of mutlinucleated lymphocytes
giant cells of langhan
what does non caseating mean
no necrosis
3 metabolic imbalances in sarcoidosis
high Ca
high ACE
hypergammaglobulinaemia
why do you get high calcium in sarcoid
granulomas in lung hydroxylate (activate) Vit D
2 types of large vessel vasculitis
takayasau’s
giant cell arteritis
2 types of medium vessel vasculitis
polyarteritis nodosa
Kawasaki’s
vessels in which parts of the body are affected by polyarteritis nodosa
gut
kidney
which infection is polyarteritis nodosa associated with
hep B
3 types of ANCA associated small vessel vasculitis
microscopic polyangitis
granulomatosis with polyangitis (Wegners)
eosinophilic granulomatosis with polyangiitis (Churg Strauss)
what clinical sign is characteristic of vasculitis
palpable purpuric rash
Tx for GCA
steroids
Ix for GCA
ESR
Sx of Kawasaki
fever
erythema / desquamation of soles / palms
conjunctivitis
lymphadenopathy
coronary arteries may be affected (MI/aneurysms)
what is polyarteritis nodosa
necrotising arteritis
what cells are high in polyarteritis nodosa
polymorphs, lymphocytes, eosinophils
what does the angiography of polyarteritis nodosa look like
nodular apperance - due to small aneurysms
triad of hallmark organs that are affected in granulomatosis with polyangiitis
ENT
lungs
kidneys
AB in granulomatotsis with polyangiitis
c-ANCA (against proteinase 3)
what is c-ANCA
cytoplasmic ANCA
triad of hallmark signs in eosinophilic grnaulomatosis with polyangiitis
asthma
eopsinophillia
vsaculitis
AB in eosinophilic granulomatosis with polyangiitis
p-ANCA (directed against myeloperoxidase)
what is p-ANCA
perinuclear ANCA
