[Rheum] Vasculitis Flashcards
what is vasculitis?
a group of autoimmune diseases that affect the vessels
what can vasculitis be classified as?
- large vessel disease
- medium vessel disease
- small vessel disease
how does vasculitis present?
multiple organ systems are affected so pts present as polysymptomatic
what should polysymptomatic pts have?
rheumatology work up with:
- ANA (anti-nuclear antibodies)
- ANCA (anti-neutrophilic cytoplasmic antibodies)
what should be done if ANA or ANCA came back +ve?
further rheumatological ix:
- other antibodies
+/- biopsy (gold standard) of kidney/skin
what is the ANCA associated with granulomatosis with polyangiitis (GPA)?
c-ANCA
what are the defining sx of granulomatosis with polyangiitis (GPA)?
- saddle shaped nose
- recurrent epistaxis / sinusitis
what does histology for granulomatosis with polyangiitis (GPA) show?
granulomas
what is the ANCA associated with microscopic polyangiitis?
p-ANCA
what are the defining sx of microscopic polyangiitis?
similar to GPA but less likely to have nasopharyngeal involvement
what does histology for microscopic polyangiitis show?
no granulomas
what is the ANCA associated with eosinophilic granulomatosis with polyangiitis (EGPA)?
p-ANCA
what are the defining sx of eosinophilic granulomatosis with polyangiitis (EGPA)?
asthmatic, quite often requires intense rx for control
what does histology for eosinophilic granulomatosis with polyangiitis (EGPA)?
granulomas
what are the common Sx shared by GPA, microscopic polyangiitis and EGPA?
- renal impairment / rapidly progressive GN (RPGN)
- lung inflammation and haemoptysis
- skin ulceration (purpura in lower limbs)
what are the common Rx shared by GPA, microscopic polyangiitis and EGPA?
- rx remission: cyclophosphamide and corticosteroids
- once remission: low dose steroids + methotrexate / mycophenolate / azathoprine
what is the most common large vessel vasculitis?
Giant Cell Arteritis (GCA)
what arteries does GCA affect?
large arteries in the head, most commonly temporal artery
in whom is GCA rare in?
<50 year olds
what are the sx of GCA?
- single sided temporal headaches
- scalp and jaw tenderness (painful shaving, combing hair)
- visual disturbance: ophthalmic artery involvement [ophthalmic emergency]
what should be given urgently in GCA?
prednisolone (40-60mg)
60-100mg if visual sx
what is the gold standard dx for GCA?
temporal artery biopsy
heavy smoker, recurrent digit ischaemia. dx?
thromboangiitis obliterans (Buerger’s)
haemoptysis, haematuria and anti-GBM. dx?
Goodpasture’s syndrome
new agitation and hallucinations on prednisolone. dx?
steroid induced psychosis
fever >5 days, conjunctivitis, strawberry tongue. dx?
Kawasaki’s disease
transmural inflammation, “beads on a string” angiography. dx?
polyarteritis nodosa
recurrent mouth and genital ulcers not due to infection. dx?
Behcet syndrome
what should all small vessel disease be treated with initially?
cyclophosphamide and corticosteroids
what should be performed if there is renal, lung or skin involvement?
biopsy
what is the common autoantibody present in the following:
- rheumatoid arthritis
- Sjögren’s syndrome?
rheumatoid factor (RF)
what is the autoantibody present in rheumatoid arthritis?
anti-CCP
what is the common autoantibody present in the following?
- SLE
- systemic sclerosis (limited and diffuse)
- Sjögren’s syndrome
- poly-/dermato-myositis
- autoimmune hepatitis
ANA
what are the autoantibodies present in SLE?
anti ds-DNA and anti-Smith (Sm)
what is the autoantibody present in limited systemic sclerosis?
anti-centromere
what is the autoantibody present in diffuse systemic sclerosis?
anti-Scl-70
what are the autoantibodies present in Sjögren’s syndrome?
anti-Ro and anti-La
what are the autoantibodies present in poly-/dermato-myositis?
anti-Jo and anti-Mi2
what is the autoantibody present in drug induced lupus?
anti-histone
what is the autoantibody present in vasculitides?
ANCA
