Vasculitis Flashcards
What are primary vasculitides?
A group of autoimmune conditions, characterised by inflammation of the blood vessels
Blood vessels in any organ can be affected by primary vasculitides, but particularly. vessels in which organs are affected?
Skin Kidneys Lungs Joints Nerves ENT
Give examples of large vessel vasculitis?
Takaysu arteritis
Giant cell arteritis
Give examples of medium vessel vasculitis?
Polyarteritis nodosa
Kawasaki disease
Give examples of ANCA-associated small vessel vasculitis?
Microscopic polyangitis
Granulomatosis with. polyangitis
Eosinophilic granulomatosis with polyangitis
Give. examples of immune complex. small vessel vasculitis?
Cryoglobulinaemic vasculitis
IgA vasclitis
Hypocomplementemic urticarial vasculitis
Which vessels are affected. by giant cell arteritis?
Aorta and its major branches
What is the peak age of. incidence of giant cell arteritis?
70-79 years
In which groups is giant cell arteritis most common?
Women
Caucasian
What are the symptoms of giant cell arteritis?
Headache - temporal headache with tenderness, subacute onset, constant and little relief with analgesics
Visual symptoms
Jaw claudication - pain on chewing can lead to weight. loss
Polymyalgia rheumatic symptoms
Constitutional upset
How can giant cell arteritis lead to blindness?
Causes ischaemic optic neuropathy due to loss of blood flow in the posterior ciliary. arteries
What. are the clinical examination findings of giant cell arteritis?
Temporal artery. symmetry
Thickening
Loss of pulsatility
What is the gold standard test for giant cell arteritis?
Temporal artery biopsy
What is a positive. finding in a temporal artery biopsy. for. giant cell. arteritis?
Interruption of the internal elastic laminae with mononuclear inflammatory cell infiltrate within the vessel wall
Absence of positive findings on temporal artery biopsy rules out giant cell arteritis. T/F?
True
Other than temporal artery biopsies how can giant cell arteritis be investigated?
Temporal artery US, MRI and PET CT
How is giant. cell arteritis treated?
1mg/kg/day of prednisolone up to a maximum 60mg - should be maintained for 1. month then tapering to 15mg by 12. weeks. The aim is the discontinue corticosteroids by 12-18 months.
IV methylprednisolone can be used if. visual symptoms are present
Corticosteroid sparing therapies - methotrexate, tocilizumab, mycophenolate
75mg aspirin daily to reduce ischaemic complications
What are. the differential diagnoses of IgA vasculitis?
Idiopathic
Drugs
Infection: HCV, HBV, gonococcus, meingococcus, staph
Secondary RA
Malignancy
Manifestation of small/medium vessel ANCA associated vasculitis
In what age group is IgA vasculitis most common?
Children aged 2-11 years
IgA vasculitis is usually a self-limiting illness. T/F?
True
What are the signs of IgA vasculitis?
purpuric rash on the buttocks, thighs and occasionally lower legs
Urticarial rash, confluent petechiae, ecchymosis, ulcers
Arthralgia and arthritis in the lower limb
What are the potential complications of. IgA vasculitis?
GI. pain, bleeding, diarrhoea, intussusception
IgA nephropathy
Orchitis
CNS signs
How can the extent of involvement of IgA vasculitis be assessed?
Urinalysis
Urine PCR
Often no treatment is required for IgA vasculitis. T/F?
True
When can corticosteroids be used in the treatment of IgA. vasculitis?
Testicular torsion
GI. disease
Arthritis
What type of vasuculitis has the most associated morbidity and mortality?
ANCA Associated vasculitis
What cells are ANCA autoantibodies directed against?
Neutrophils
Monocytes
Why is detection of ANCA autoantibodies helpful in vasculitis?
Diagnosis
Assessing response to treatment
Monitoring for early signs of relapse
Gives prognostic information
cANCA with PR3. is very suggestive of…?
Granulomatosis with polyangiitis
pANCA with strong mPO is suggestive of
Microscopic polyangiitis
What treatments can be used to induce remission of ANCA associated vasculitis?
prednisolone with Oral or IV cyclophosphamide or rituximab in severe disease
Prednisolone with methotrexate. or mycophenolate in moderate. disease
What risks are associated with the use of cyclophosphamide in ANCA associated vasculitis?
Infection
Cytopaenia
Malignancy
Infertility
What is the mechanism of action of rituximab?
Anti-B cell biologic
What drugs are used in maintenance of remission of ANCA associated vasculitis?
Azathioprine
Methotrexate
Mycophenolate
What characterises granulomatosis with polyangiitis?
Granulomatous necrotising ifnlammatory lesions of the upper and lower respiratory tract and often paucity-immune glomerulonephritis
Describe the classic triad of disease seen in granulomatosis with polyangiitis?
Upper airway / ENT - rhinitis, chronic sinusitis, chronic. otitis media, saddle nose deformity, nasal/septal perforation
Renal - rapidly progressive pouch-immune glomerulonephritis
Lower respiratory - parenchymal nodules +/- cavitation, alveolar haemorrhage
What constitutional symptoms are there in granulomatosis with polyangiitis?
Fatigue Weight loss Fever/sweats Myalgia/arthralgia Failure to thrive in the elderly
