Vasculitis Flashcards
What are the different categories of vasculitis?
- immune complex small vessel vasculitis
- ANCA-associated small vessel vasculitis
- medium vessel vasculitis
- large vessel vasculitis
What types of vasculitis is immune complex small vessel vasculitis?
- cryoglobulinemic vasculitis
- IgA vasculitis
- hypocomplementemic urticarial vasculitis
- anti-GBM disease
What type of vasculitis is in ANCA-associated small vessel vasculitis?
- microscopic polyangiitis
- granulomatosis with polyangiitis
- eosinophilic granulomatosis with polyangiitis
What vasculitis is in medium vessel vasculitis/
- polyarteritis nodosa
- Kawasaki disease
What vasculitis is in large vessel vasculitis?
- takayasu arteritis
- giant cell arteritis
What sign might you see on examination in giant cell arteritis?
thickened, non-pulsatile temporal artery
Define giant cell arteritis
systemic vasculitis that affects the aorta and its major branches
Describe a typical clinical presentation of giant cell arteritis
- headache (temporal with pain on palpation, subacute onset, constant with little relief from analgesics)
- visual symptoms
- jaw claudication (pain when eating/talking)
- polymyalgia reumatica symptoms (shoulder/pelvic girdle pain)
- constitutional symptoms
What are the complications of giant cell arteritis?
- visual loss (irreversible loss, acute ischaemic neuropathy, sudden painless loss - can be preceded by amaurosis fugal)
- large vessel vasculitis (stenosis and aneurysm - stroke risk)
- CVA (obstruction/occlusion of internal carotid a./vertebral a.
What investigations would you want to do in giant cell arteritis?
- temporal artery biopsy (gold standard)
- temporal artery US
- MRI
- PET CT (good to check if large vessel involvement - aneurysm)
How would you medically treat giant cell arteritis?
- prednisolone 1mg/kg/day (discontinue by 12-18months)
- aspirin 75mg (if risk for stroke)
- methotrexate/ mycophenolate mofetil/ tocilizumab (steroid-sparing therapy) for relapses
What are the causes of cutaneous vasculitis?
- idiopathic
- drugs
- infection (HCV, HBV, gonococcus, meningococcus, HIV)
- secondary RA/CTD/PBC/UC
- malignancy (esp lymphoma)
- manifestation of small/medium ANCA vasculitis
What important tests should you do if you suspect vasculitis?
- CRP/ESR
- urinalysis (kidney function)
- FBC, LFTs, Us and Es
Describe the signs of Henoch Schonlein purpura
- purpuric rash on buttocks and thighs
- urticarial rash, petechiae, ecchymoses, ulcers
- arthralgia/arthritis (lower limb)
What are the complications of Henoch Schonlein purpura?
- GI: pain, bleeding, diarrhoea, intussusception (rare)
- renal: IgA nephropathy
- urinary: orchitis
- CNS (rare)
What is the management and prognosis for Henoch Schonlein purpura?
- often no treatment
- corticosteroids for certain complications (testicular torsion, GI, arthritis)
- self-limiting with small minority relapsing within 12 months
Describe granulomatosis with polyangiitis (GPA)
granulomatous necrotising inflammatory lesions of the upper and lower respiratory tract and often a pauci-immune glomerulonephritis (no immune deposit in glomeruli)
What is the classical triad of symptoms and signs of GPA?
- upper airway symptoms: rhinitis, chronic sinusitis, chronic otitis media, saddle nose deformity, nasal septal perforation
- renal symptoms: pauci-immune glomerulonephritis
- lower respiratory symptoms: parenchymal nodules and cavitation, alveolar haemorrhage (haemoptysis)
What factors would increase your clinical suspicion of vasculitis?
- classical presentation
- constitutional symptoms
- multi-system disease
- repeat GP/hospital visits
- disease ‘not behaving’ as it should
Describe the immunology of ANCA vasculitis
- autoantibodies against cytoplasmic constituents of neutrophils and monocytes
- cANCA with PR3 (neutrophils) very suggestive of GPA
- pANCA with strong MPO suggestive of MPA (or EGPA)
- positive not always indicative of ANCA
How useful is ANCA detection?
- not helpful as diagnostic tool (not all positive ANCE = ANCA vasculitis)
- useful prognostic information (increased ANCA = increased chance of relapse)
- can monitor for for early signs of relapse)
What is the treatment for vasculitis?
- remission induction (prednisolone + cyclophosphamide/rituximab or methotrexate/mycophenolate - milder drugs)
- switch off vasculitis activity
- higher dose = higher toxicity
- 3-6m - remission maintenance (azathioprine/ methotrexate/ rituximab)
- prevent relapse
- lower drug toxicities
- more prolonged therapy
What are the risks of cyclophosphamide treatment?
- cytopenias
- malignancy
- infertility
- rituximab is safer and no risk of infertility
