Vasculitis Flashcards
What is vasculitis?
Inflammation of blood vessels
Resulting in
- lumen narrowing
- impaired blood flow
- organ damage
- wall thinning and aneurysm
- perforation and haemorrhage
How are vasculitides classified?
List the causes
Large
- GCA
- Takayasu’s
Medium
- Polyarteritis nodosa
- Kawasaki
Small is split into two:
ANCA +ve:
- Wegner’s (GPA)
- Microscopic polyangiitis
- Churg-Strauss
ANCA -ve
- HSP
- Goodpasture’s
- Autoimmune disease associated
What is ANCA?
Types, associations?
Anti-neutrophil cytopasmic antibodies
Two types
cytoplasmic ANCA
- Assoc with MPO antigen
- Wegner’s (GPA)
peri-nuclear ANCA
- Assoc with PR3 antigen
- Churg Strauss
Two types of GCA?
Describe
Temporal: as in other flashcards
Systemic GCA: can affect any large vessels in the body
- mesenteric
- subclavian
How does systemic GCA present?
Often just fever, malaise, myalgia
If mesenteric - abdo pain
What is Takayasu’s?
Who is affected?
Vasculitis of aorta and major branches
Granulomatous inflammation resutling in
- stenosis
- thrombosis
- aneurysms
20s-40s
Asian origin
Women
Features of Takayasu’s?
Cerebral: dizzy
Ophthalmological: visual changes
Upper limb: weak arm pulses
Plus systemic: fever, malaise, myalgia
Management of Takayasu’s?
High dose steroids
Methotrexate
Cyclophosphamide
What is polyarteritis nododa?
Presentation?
Medium vessel vasculitis
Necrotising
Aneurysms
Thrombosis
Which can lead to organ damage
Severe systemic symptoms (fever, maliase, myalgia)
Skin: livedo reticularis, ulcers, petechial rash
Renal damage (due to hypertension not glomerulonephritis)
Epidymitis
Cardiac, GI, GU
Investigations of polyarteritis nodosa?
Raised white cells
Mild eosinophilia
Raised ESR, CRP
ANCA -ve
Management of polyarteritis nodosa?
Control BP to protect kidneys
Corticosteroids
Cyclophosphamide
What disease is polyarteritis nodosa associated with?
Hepatitis B
Subtypes of small vessel vasculitides?
ANCA +ve
- GPA granulomatosis with polyangiitis (Wegener’s)
- EPGA eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
ANCA -ve
- HSP
What’s the new name for Wegener’s ?
Granulomatosis with polyangiitis (GPA)
Pathophysiology of GPA?
Who gets it?
Necrotising vasculitis of arterioles, capillaries, post-capillary venules
Propensity for upper resp tract, lungs, kidneys
Men 25-60
Presentation of GPA?
Upper resp tract: sinusitis, otitis, nasal crusting and bleeding, saddle nose deformity
Lungs: pulmonary nodules +/- cavitation
Renal: nephritic syndrome
Skin: ulcers, purpura
Neuropathies
Eyes: proptosis, uveitis
Investigations of GPA?
cANCA against PR3 antibody
Raised ESR, CRP
CXR: cavitating lesions
Biopsy
Pathophysiology of EGPA?
Who gets it?
Similar to GPA but eosinophilic involvement
30-45yrs
Men
Presentation of EGPA?
Triad of
Adult onset asthma
Eosinophilia
Vasculitis
Affects nerves, skin, lungs
Presents like septic shock
Management of the ANCA +ve small vessel vasculitides?
Induce remission:
- high dose steroids + cylophosphamide or rituximab
- plasma exchange
- or if no organ involvement steroids + methotrexate
Maintain remission:
- methotrextae
- azathrioprine
What is HSP?
Who gets it?
Causes?
Henoch Schonlein purpura
IgA mediated vasculitis of small vessels
Following an URTI
Children usually
Presentation of HSP?
Pupuric rash over buttocks and legs and extensor surfaces
Glomerulonephritis
Arthritis
Abdo pain
What GI disorder are HSP patients at higher risk of?
Intesussception
Management and monitoring of HSP?
None usually needed
Steroids
Monitor renal function closely as can result in CKD
