Vasculitis Flashcards
What is vasculitis?
- Inflamamation of blood vessels
What are the categories for vasculitis?
They are categorised by size of blood vessels
- Large
- Giant cell arteritis
- Takayasu’s arteritis
- Medium
- Polyarteritis nodosa
- Kawasaki disease
- Small
- ANCA-associated
- Granulomatosis with polyangitis
- Eosinophilic granulomatosis with polyangitis
- Immune complex vasculitis
- Goodpastures disease
- Variable vessel vasculitis
What are the symptoms of vasculitis?
- Systemic features
- Fever
- Weight loss
- Respiratory symptoms
- Nose bleeds
- Shortness of breath
- Renal symptoms
- Neurological symptoms
- Stroke
- Fits
- Chorea
- Psychosis
- Confusion
- GI
- Cardiac
- Eyes
How does giant cell arteriosus present?
- New onset headache
- Typically in the elderly
- Temporal artery/scalp tenderness
- Systemic symptoms
- Jaw claudication (pain with eating)
- Extra cranial symptoms
- Weight loss
- Malaise
- Morning stiffness
- 50% of cases are associated with Polymyalgia Rheumatica so the symptoms of joint pain and stiffness may be present
What are the complications of GCA?
- Amaurosis fugax can occur where there is initial blurring of vision then visual loss in the effected eye, only 10% recover
- This is irreversible
How is GCA investigated for diagnosis?
- Raised CRP and ESR
- ANCA may be +ve
- Biopsy needed to confirm but don’t wait for the results of the biopsy – if strongly suspecting then start steroid treatment stat to avoid visual loss
What is the treatment for GCA?
- Prednisolone 40mg/day (60mg if visual loss) over a 2 year course
- Pts see rapid improvement over 2-3 days
- On long term steroid therapy so be aware of side effects
- Cover with alendronic acid, calcium/vitD and PPI.
- Can add DMARDs to reduce steroid dose if needed)
What is polymyalgia rheumatica?
- Polymyalgia Rheumatica (PMR) is not a true vasculitis
- It is a condition that causes pain, stiffness and inflammation in the muscles around the shoulders, neck and hips.
- It typically presents in females > males and over 50
What causes polymyalgia rheumatica?
Unknown
What are the features of polymyalgia rheumatica?
- Age >50years
- Severe pain and stiffness in the shoulder, neck and hips – usually symmetrical
- Pain is worse in the morning
- o muscle weakness (which excludes the ddx of polymyositis).
- Can also cause systemic sx
- Weight loss
- Anorexia
- Depression
¡Investigations - raised CRP and ESR (unlike the ddx of fibromyalgia), normal CK
¡Treatment – 15mg/day of prednisolone causes rapid improvement of sx within days (if not then ?wrong diagnosis) then on 2 year course
What tests should be carried out to investigate polymyalgia rheumatica?
- Raised CRP and ESR
- Unlike the ddx of fibromyalgia
- Normal CK
How is polymyalgia rheumatica treated?
- 15mg/day of prednisolone
- Causes rapid improvement of sx within days
- If symptoms do not improve then ?wrong diagnosis)
- Some people require a 2 year course of steroids
What is granulomatosis with polyangitis?
- Granulomatosis with polyangiitis (GPA), formerly known as Wegener granulomatosis, is a rare multisystem autoimmune disease of unknown etiology.
- Its hallmark features include necrotizing granulomatous inflammation and pauci-immune vasculitis in small- and medium-sized blood vessels.
What are the symptoms of GPA?
- It causes a classic triad of ENT problems
- Saddle nose
- Epistaxis
- Lung disease
- Cough
- Haemoptysis
- Kidney disease
- Progressive glomerulonephritis
What tests should be carried out to investigate and diagnose GPA?
- Raised cANCA in the blood is strongly associated
- CXR should be performed to check for pulmonary haemorrhage
- Urine dip for proteinuria and haematuria
