vasculitis Flashcards
what is vasculitis
autoimmune inflam of blood vessels –> thickness and stenosis
what are serious side effects of vasculitis
ischaemia, necrosis and organ inflam
what is the pathogenesis of primary vasculitis
inflam response in vessel walls become leaky and narrow, thrombus and fibrin deposits –> reduces flow
what is secondary vasculitis
triggered by infection, drugs, toxins or another inflam disorder eg cancer
what are the 2 main types of large vessel vasculitis
Takayasu arteritis (TA), giant cell arteritis (GCA)
who commonly gets TA
asian women under 40
what vessels does TA commonly affect
arteries of the aorta
what causes large vessel vasculitis
granulomatous infiltration of large vessel walls
what are common symptoms associated with large vessel vasculitis
bruits (carotid), reduced pulses, low grade fever, malaise, night sweats, BP difference in extremities
what is the most common form of systemic vasculitis
giant cell arteritis
who commonly gets giant cell arteritis
older patients with polymyalgia rheumatica
what is the pathogenesis of giant cell arteritis
inflamm of tunica intima, media and adventitia by lymphocytes + macrophages causing granulomas
what are the symptoms of giant cell arteritis
headache (occipital and temporal), scalp tenderness, jaw claudication, visual loss, tender, non-palpable arteries, fever, malaise, fatigue
what investigations are done for giant cell arteritis and TA
temporal artery biopsy (gold), bloods; PV, CRP. MR angiogram, CT
what is the treatment of giant cell arteritis
40-60mg pred - 60mg if visual disturbance
what subgroups is small vessel vasculitis divided into
ANCA +ive and ANCA -ive
what are the ANCA +ive small vessel vasculitis
granulomatosis polyangiitis (GPA), Eosinophillic granulomatosis with polyangiitis (EGPA), microscopic polyangiitis (MPA)
what are the ANCA -ive small vessel vasculitis
Henoch-Schnlein purpura
who commonly gets GPA
white men 30-50s
what body systems are commonly affected in GPA
nasopharynx, lungs’ kidneys
what symptoms are present in GPA
sinusitis, ulcers, SADDLE NOSE, nose bleeds, otitis media, cough/ haemoptysis, palpable purpura, eye problems
what autoantibodies are present in GPA
cANCA and anti-PR3
what is the GPA diagnostic criteria (5)
nasal/ oral inflamm (ulcers/ discharge), chest imaging with nodules, urinary sediment, granulomatous inflamm on biopsy, cANCA + anti-PR3
what is the main difference between EGPA and GPA
EGPA is symptoms of late onset asthma and high eosinophil count
what is the diagnostic criteria for EGPA (6)
(4+) asthma, eosinophilia, histological proof of eosinophilia, paranasal sinusitis, puml infiltrates, polyneuropathy
what antibodies are present in EGPA
pANCA and anti-MPO
how is MPA different to GPA and EGPA
doesn’t affect nasopharynx, no granulomas present
what does MPA usually cause
glomerulonephritis
what antibodies are present in MPA
pANCA and anti-MPO
what are the investigations of ANCA +ive small vessel vasculitis
bloods: PV, CRP, FBC/ U+E’s, ANCA, CXR
what is the management + criteria of localised/ early ANCA +ive small vessel vasculitis
U/LRTI with non life threatening organ involvement: methotrexate + steroids
what is the management + criteria of generalised/ systemic ANCA +ive small vessel vasculitis
renal/ organ threatening: cyclophosphamide + steroids OR rituximab + steroids
what is the management + criteria of refractory ANCA +ive small vessel vasculitis
progressive/ unresponsive to steroids: IV immunoglobins + rituximab
what is HSP
ANCA -ive, IgA mediated vasculitis
who commonly gets HSP
children
what normally preceeds HSP
URTI, pharyngeal infection or GI infections (GAS organisms)
what are the main symptoms of HSP (4)
purpura, abdo pain, arthritis, renal involvement (50%), lungs
describe the purpura of HSP
purpuric (purple) rash over buttocks and lower limb (punch out legions)
what abdo pain can come with HSP
colicky, bloody diarrhoea, vomiting
what management is there for HSP
normally self-limiting <8 weeks, urinalysis for renal damage
