Vasculitis Flashcards
What is vasculitis?
Histological term decribing inflammation in the vessel wall.
- Systemic inflammatory vasculitidies
- Multi-system / organ involvement
- Systemic symptoms and signs
- There are 2 features helpful in classifying vasculitis:
- ANCA
- Size of vessel
What are the types of systemic vasculitis affecting small vessels?
- Microscopic polyangitis
- Granulomatosis and polyangitis
- Eosinophilic granulomatosis with polyangitis
- Henoch Schonlein Purpura
What are the types of systemic vasculitis affecting medium vessels?
- Polyarteritis nodosa
- Kawasaki disease
What are the types of systemic vasculitis affecting large vessels?
- Giant cell arteritis
- Takayasu’s arteritis
What are the systemic clinical features of vasculitis?
- Weight loss
- Fatigue
- Night sweats
- Arthralgia
- Fever
What are the clinical features of vasculitis found in the skin?
- Rash
- Purpura
- Ulcers
What are the clinical features of vasculitis found in the kidneys?
- Haematuria
- Proteinuria
- AKI /CKD
What are the neurological clinical features of vasculitis?
- Nerve involvement
- Motor or sensory
What are the respiratory clinical features of vasculitis?
Haemoptysis
What are the ENT clnical features of vasculitis?
- Epistaxis
- Deafness
- Sinusitis
Describe the epidemiology of polymyalgia rheumatica.
- Usually diagnosed in patients >65. Rare in patients <50.
- 20 / 100,000 >50years old.
- Peak incidence 70-80.
- Women:Men 2:1.
- Familial cases recognised.
- Geographical variation - higher in Scandanavian and Northern European countries.
Describe the relationship between PMR and GCA.
- PMR more common than GCA (2-3x).
- PMR can precede or follow a diagnosis of GCA, or occur at the same time.
- 15% of PMR patients develop GCA.
- PMR seen in 50% of patients with GCA.
Describe the pathophysiology of PMR.
- Unknown cause
- HLA association: HLA-DR4
- Proinflammatory cytokine interleukin (IL)-6 implicated
- Muscle is histopathologically normal, structures around the joint are mainly affected.
- ? infectious trigger.
Describe the typical history of a patient presenting with PMR.
- Aching / stiffness in muscles / joints.
- Onset is often acute.
- Distribution:
- Proximal and symmetrical
- In shoulder / hip girdle
- Can become distal involvement
- Timing - stiffness worse in the morning or after inactivity >30 minutes.
- Systemic features - fatigue, weight loss, depression.
- Can affect daily activities:
- Getting out of a chair
- Getting dressed
What are the differentials for a patient presenting with ?PMR?
What would you likely find on examination of a patient with PMR?
- Limited shoulder abduction
- Distal synovitis
- Reduced ROM
- Normal muscle strength
What are the initial investigations to carry out in a patient with ?PMR?
- Bloods:
- ESR / CRP - hallmarks of the disease; usually elevated.
- FBC - normocytic / normochromic, thrombocytosis.
- LFTs - raised ALP.
- CK - normal.
- Anti-CCP/ANA/RF - negative.
- Imaging (these are not required for diagnosis):
- USS
- MRI
- PET
How is PMR diagnosed?
- There are no formal diagnostic criteria.
- Diagnosis is based on typical clinical features and lab findings.
Describe the treatment for PMR.
- Steroids are the mainstay of treatment - prompt response.
-
ADULT (by mouth)
- 10-15mg daily until remission of disease activity; maintenance 7.5-10mg daily, reduce gradually to maintenance dose.
- Many patients require Rx for at least 2 years and in some patients it may be necessary to continue long term low-dose corticosteroid Rx.
- Prednisolone
- High dose
- Slow tapering
- Careful monitoring
- Steroid-sparing agents
- Methotrexate
Describe the pathology of giant cell arteritis (GCA).
- AKA cranial arteritis or temporal arteritis.
- Symptoms and signs of GCA result from involvement of the cranial branches of arteries that originate from the aortic arch.
- The name reflects the type of inflammatory cell involved.
Describe the typical history of a patient with GCA.
- Headaches - temporal.
- Tenderness of the scalp or temples - unable to brush hair.
- Diplopia / vision loss - transient painless monocular visual loss.
- Dizziness or problems with coordination and balance.
- Jaw claudication.
Describe the likely examination findings of a patient with GCA.
Which initial investigations should be carried out in a patient with ?GCA?
- Bloods
- ESR / CRP - hallmark of the disease, usually elevated.
- FBC - normocytic / normochromic, thrombocytosis.
- LFTs - raised ALP.
- Imaging
- USS - increased diameter of the superficial temporal artery and hypoechoic wall thickening (halo sign).
- Temporal artery biopsy
- Infiltration of T lymphocytes, macrophages and giant cells in the vessel wall.
- Granulomatous inflammation of intima and media.
- Breaking up of internal elastic lamina with giant cells and plasma cells.
Describe the treatment for GCA.
- Corticosteroid
-
FOR ADULT (by mouth)
- 40-60mg daily until remission of disease activity; the higher dose being used if visual symptoms occur; maintenance 7.5-10mg daily, reduce gradually to maintenance dose.
- Many patients require treatment for at least 2 years and in some patients it may be necessary to continue long term low-dose corticosteroid treatment.
What is the prognosis for GCA patients?
- 18-24 months of steroid often required.
- Relapse rate of 25-50%.
