Vasculitis Flashcards
What is systemic vasculitis and its cause?
It is an autoimmune disease affecting lots of blood vessels which therefore affect many organs.
It has a mixture of genetic factors and environmental factors (exposure or infection)
What are some types of large vessel vasculitis?
- Takayasu Arteritis,
- Giant Cell Arteritis
What are some types of medium vessel vasculitis?
- Polyarteritis nodosa,
- Kawasaki Disease,
What are some types of immune complex small vessel vasculitis?
- Cryoglobulin vasculitis,
- IgA vasculitis (Henoch-Schonlein)
- Hypocomplementemic urticarial vasculitis
What are some types of ANCA-Associated small vessel vasculitis
- Microscopic Polyangiitis,
- Granulomatosis with polyangiitis (Wegner’s)
- Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss)
What is the presentation of Giant Cell Arteritis
- Prevalence increases with advancing age.
- Presents with a constant temporal headache and temporal artery thickening/tenderness with little relief from analgesia.
- Visual symptoms
- Scalp tenderness
- Jaw claudication (occurs when eating/talking)
- Polymyalgia rheumatic symptoms (pain around shoulders, neck and hips) it has a strong association with polymyalgia rheumatica,
- Constitutional upset
What are the complications of giant cell arteritis?
- Irreversible visual loss (sudden painless loss of vision)
- Large vessel vasculitis which can cause vascular stenosis and aneurysms.
- Cerebral vascular accident.
Explain the diagnosis of giant cell arteritis
- Clinical presentation (Typical headache with associated features, appropriate age with no alternative diagnosis.)
- Clinical exam findings (temporal artery asymmetry, thickening and loss of pulsatility)
- Acute phase response (Raised CRP/ESR with no signs of infection)
- Gold standard is temporal artery biopsy to look for inflammation. However temporal artery USS, MRI and PET CT can also be used.
What is the treatment for suspected giant cell arteritis?
If strong clinical suspicion treat while you await results with prednisolone (1mg/kg/day) and 75mg Aspirin (unless contraindicated)
What is the treatment of confirmed giant cell arteritis?
Maintain 60mg of prednisolone for 1 month then aim to taper to 15mg by 12 weeks with discontinuation by 12-18months.
If patients has relapsing disease on steroids then use: Mycophenolate, methotrexate or tocilizumab (anti IL6)
What is the differential diagnosis for cutaneous (small vessel) vasculitis/leucocytoclastic vasculitis and an example?
- Differentials include idiopathic, drugs, infections (meningococcal), secondary to other autoimmune disease, malignancy.
- Example of a cutaneous/leucocytoclastic vasculitis is Henoch Schonlein Purpura,
What is the presentation of Henoch Schonlein Purpura
- Commonly occurs in children but can occur in adults. It is a self limiting illness lasting 4-16 weeks. Commonly triggered by streptococcal sore throat.
- It presents with: purpuric rash on buttocks and thighs, urticarial rash, confluent petechiae, ecchymoses (bruise), ulcers and arthralgia/arthritis
What are the complications of Henoch Schonlein purpura
- Renal: IgA nephropathy
- Urological: Orchitis
- CNA very rare to get complications.
- GI disease and renal disease are the most significant complications
Explain the diagnosis and treatment of Henoch Schonlein Purpura
Diagnosis - Exclude secondary causes via history (any recent upper respiratory infections?), examination, immunology and virology. Assess the extent of involvement via urinalysis/urine PCR
Treatment - Often none but may use steroids with complications (testicular torsion, GI disease, Arthritis)
What is the classic triad of Henoch Schonlein Purpura?
Purpuric rash on extensors of lower limbs, abdominal pain and arthritis.
