Editing Vasculitis (section)

== '''Large-vessel vasculitis:''' ==

* '''Takayasu arteritis'''
* '''GCA/Temporal arteritis'''
** Epidemiology
*** Primarily >50yo, and increasing with age
*** Mostly caucasians
*** F:M 2:1
** Pathophysiology
*** Exact cause unknown
*** Probably in response to immunologic injury, the artery induces myofibroblasts which leads to new vessel formation and marked thickening of intima
*** This leads to vessel occlusion leading to symptoms of ischaemia
** Clinical
*** New-onset headache
*** Scalp tenderness
*** Reduced pulse and tenderness along temporal artery
*** Jaw and tongue claudication (specific)
*** Diplopia (specific)
*** Blindness
*** Systemic inflammation
*** PMR (although only 10-20% of patients with PMR develop GCA)
*** Check for bilateral pulses and BPs - 20% have large-vessel vasculitis
*** ICA and vertebral arteries can be involved, leading to stroke/seizure/acute hearing loss/vertigo/cerebral dysfunction and depression
** Diagnosis
***
*** Gold standard test if temporal artery biopsies
**** Up to 50% of biopsies show diffuse lymphocytic infiltrate without evidence of granulomatous inflammation or giant cells
**** Fraying of the internal elastic lamina is a non-specific finding of normal aging, and should be ignored in the absence of inflammation
** Medical treatment
*** Corticosteroid therapy - 40-60mg daily, maintaining the dose until inflammatory markers normalise
**** Reduce prednisolone by 5mg every 1-2 weeks down to 30mg/day, then 2.5mg every 1-2 weeks until 15mg per day, then 2.5mg every 4 weeks until 10mg, then 1mg every 4 weeks until the patient is off prednisolone
*** IV methylpred if visual loss is present for 3 days
*** Start on low-dose aspirin to reduce risk of cardiovascular events and blindness
** Surgical treatment
*** Revascularisation is rare required
* '''Idiopathic aortitis'''
** '''Idiopathic retroperitoneal fibrosis'''
*** Presents 40-60yo
*** Pain in lower back, abdomen, flank and scrotum
*** Some have systemic symptoms
*** Most have elevated inflammatory markers
*** CT - homogenous mass around the aorta following the iliac bifurcation, with medialisation of ureters and associated hydronephrosis. Usually, lymphadenopathy is absent.
*** Pathophysiology unknown
*** Treat with high-dose prednisolone
** '''IgG4-related systemic disease'''
** '''Erdheim-Chester disease'''
*** Periaortic fibrosis ('coated aorta')
*** Hairy kidney
*** Main distinguishing factor from RPF/IgG4 is that bones are involved