vasculitis Flashcards
large vessel vasculitis
giant cell arteritis
Takaysau’s artertitis
medium vessel vasculitis
polyarteritis nodosa
kawaskis disease
small vessel vasculitis
EPGA, granulomatous polyangitis (GPA), microscopic polyangitis
vasculitis
inflammation of blood vessels
commonly affects joints, lungs, skin, kidneys and nerves
GCA
headache scalp tenderness jaw claudication sudden blindness high dose prednisolone risk of going blind comes on over weeks
symptoms and signs of vasculitis
consider in any unidentified multi-system disorder
systemic: fever, malaise, weight loss, arthralgia, myalgia
skin: rashes, ulcers, nailed infarcts
eyes: episcleritis, slceitis, visual loss
pulmonary: heamoptysis
cardiac: angina or MI, HF, pericarditis
GI: pain, perforation, malabsorption
renal: HTN, haemtauria, proteinuria, renal failure, glomerulonephritis
neurological: stroke, fits, chorea, psychosis, confusion, impaired cognition, altered mood, mononeuritis monoplex - individual nerves, different places, wrist and foot drop (radial and common perineal) most common
management of vasculitis
large vessel: steroids
medium/small: IV cyclophosphamide (not good in pregnancy, can loose fertility avoid in women who haven’t had a family)
6 months of intense treatment to get patient into remission then maintenance therapy
control BP
azathioprine may be used as steroid sparing maintenance
microscopic polyangitis
small vessel
rapidly progressive glomerulonephritis
pulmonary haemorrhage
pANCA
polyarteritis nodosa
necrotising vasculitis
causes aneurysms and thrombosis, leading to infarction in affected organs
systemic features, skin rash and punched out ulcers, renal, cardiac, GI, GU involvement
control BP
corticosteroids and cyclophosphamide
investigations in vasculitis
ESR CPR ANCA MSU, cast, microscopy angiography and biopsy
treatment for active newly diagnosed small vessel vasculitis
rapid induction of immunosuppression in someone with multi organ involvement
start with steroids prednisolone in combination with a second immunosuppressant such as cyclophosphamide or rituximab
once remission has been achieved, switching the patient to a steroid sparing agent is the long term plan: methotrexate, azathioprine and myocphemolate mofetil
EPGA diagnosis and presentation
asthma, eosinophilia and multi-organ involvement - diagnosis
LRT - asthma, haemoptysis, pneumonitis
URT: allergic rhinitis, paranasal sinusitis, nasal polyps
heart: pericardial effusion, MI, myocarditis
skin: rashes and nodules
renal: crescentic glomerulonephritis, HTN, renal failure
NS: mononeuritis monoplex
opthamology: uveitis
GI: mesenteric infarct and perforation
brittle asthma and montelukast worsening asthma symptoms - EPGA
p-ANCA
vasculitis screen
FBC U&Es LFT TFT PV CRP ANA ANCA CXR urine dip
respiratory tract and kidney involvement think
ANCA positive vasculitis
graunlomatosis with polyangiitis
upper airways disease
NASAL involvement - obstruction, ulcer, epistaxis, saddle nose deformity due to destruction of septum
renal involement
c-ANCA