Vasculitis Flashcards
What is vasculitis?
Inflammation of the blood vessel walls which can be seen in many diseases including RA, SLE, polymyositis and some allergic drug reactions
What are the common features of all systemic vasculitides?
Anaemia
Raised ESR
How are the systemic vasculitides classifed?
According to the size of vessel affected
What are the large systemic vasculitides? Which vessels do these affect?
Giant cell arteritis
Takayasu’s arteritis
Affect the aorta and its main branches
What are the medium vasculitides? Which vessels do these affect?
Polyarteritis nodosa
Kawasaki’s disease
Affect the main visceral vessels e.g. renal, coronary
What are the small vasculitides? Which vessels do these affect?
- ANCA positive vasculitis: tends to affect the respiratory tract and kidneys. Includes Microscopic polyangiitis, Wegener’s granulomatosis and Churg-Strauss syndrome
- ANCA negative vasculitis: includes Henoch-Schonlein purpura, Goodpasture’s syndrome and cryoglobulinaemia
How does vasculitis present?
Different vasculitides preferentially affect different organs, causing different patterns of symptoms. Often presentation is only of extreme fatigue with raised ESR/CRP.
N.B. consider vasculitis in any unidentified multisystem disorder
What are the systemic features of vasculitis?
Fever Malaise Weight loss Arthralgia Myalgia
What are the skin features of vasculitis?
Purpura Ulvers Livedo reticularis Nailbed infarcts Digital gangrene
What is livedo reticularis?
Pink-blue mottling caused by capillary dilatation and stasis in skin venules.
Cause may be physiological e.g. cold or vasculitis
What general tests might indicate a vasculitis?
Raised ESR/CRP
ANCA may be positive
Raised creatinine if there is renal failure
What is polymyalgia rheumatica (PMR)?
It is not a true vasculitis but it shares the same demographic characteristics as GCA and, although two separate conditions, the two often occur together
What are the clinical features of PMR?
Age >50 years
Subacute onset (<2 weeks) of bilateral aching
Tenderness and morning stiffness in shoulders, neck, hip and lumbar spine
Muscle weakness is NOT a sign
May be mild polyarthritis, tenosynovitis and carpal tunnel syndrome
May be associated fatigue, fever, weight loss, anorexia and depression
What results might be seen on investigation of a patient with PMR?
Raised CRP
ESR is typically >40 but may not be raised
ALP is increased in 30%
How is PMR distinguised from myositis/myopathies?
Creatinine kinase levels are normal
How is PMR managed?
Prednisolone PO
Expect dramatic response within 1 week and consider alternative diagnosis if not
Reduce dose slowly e.g. by 1mg/month according to symptoms and ESR
Investigate apparent ‘flares’ during the withdrawal
Most need steroids for ≥2 years so give gastric and bone protection
What is giant cell arteritis (GCA)?
Giant cell arteritis (GCA) is a systemic immune-mediated vasculitis affecting medium-sized and large-sized arteries, particularly the aorta and its extracranial branches. Arteritic involvement is most commonly noticed in the superficial temporal arteries
The clinical connections between polymyalgia rheumatica (PMR) and GCA have suggested that they are different manifestations of the same disease process
What are the symptoms of GCA?
Headache
Tenderness over the scalp or temple (often noticed when combing hair)
Claudication of the jaw when eating
What are the complications of GCA?
- GCA affecting the vertebrobasilar and sometimes carotid circulation may result in stroke
- GCA involving the opthalmic artery may cause sudden loss of vision or amaurosis fugax.
How should you act if you suspect GCA?
Do ESR
Start prednisolone 60mg PO immediately to reduce risk of irreversible bilateral blindness
What results might be seen on investigation of a patient with GCA?
Very high ESR and CRP
Often a normochromic, normocytic anaemia
Temporal artery biopsy is performed if GCA is suspected, within a week of started steroids
How is GCA managed?
Prednisolone 60mg daily at first and then gradually reduced by around 5mg a week. Once 10mg dose is reached a reduction of 1mg every 2-4 weeks is sufficient. Dose is titrated against symptoms and ESR. Prophylaxis against steroid-induced osteoporosis should be given.
What is the prognosis in GCA?
Typically a 2 year course of steroids then complete remission. Main cause of death and morbidity is long-term steroid treatment so must consider risk vs benefit and give long term gastric and bone protection
What is the gastric and bone protection given in conjunction with corticosteroids in GCA?
Gastric: PPI
Bone: Bisphosphonate
What is Takayasu;s arteritis?
A vasculitis involving the aortic arch and other major arteries causing hypertension, absent peripheral pulses, strokes and cardiac failure. It is very rare except in Japan but should be considered in patients presenting with features of CGA who are under 55 years old. Treatment is with corticosteroids
What is polyarteritis nodosa? (PAN)
A necrotizing vascultis that causes aneurysms and thrombosis in medium sized arteries leading to infarction in affected organs
What demographic is most commonly affected by PAN?
Middle aged men
What infection is PAN associated with?
Hepatitis B
What is the paediatric variant of PAN?
Kawasaki’s syndrome
What are the clinical features of PAN?
Fever Malaise Weight loss Mononeuritis multiplex Abdomial pain (resulting from visceral infarcts) Renal impairment Hypertension Lungs are rarely involved
How is PAN diagnosed?
Angiography- microaneurysms in the hepatic, renal or intestinal vessels
OR
biopsy of an affected organ, often the kidney
WCC is often increased
ESR and CRP are increased
ANCA negative
Anaemia
How is PAN treated?
Control BP meticulously
Control PAN with corticosteroid usually in combination with an immunosuppressive drug- cyclophosphamide
What is microscopic polyangiitis?
A necrotising vasculitis affecting small- and medium-size vessels
What are the symptoms of microscopic polyangiitis?
Mainly affects lungs and kidneys Causes: -Haemoptysis -Haematuria -Proteinuria -Progressive renal failure
Other features include:
- Arthralgia
- Purpuric rash
How is microscopic polyangiitis diagnosed?
Renal biopsy
Measurement of serum perinuclear-ANCA
How is microscopic polyangiitis treated?
Corticosteroid + cyclophosphamide
What is ANCA?
Anti-neutrophil cytoplasmic antibodies- a group of autoantibodies, mainly IgG, against antigens in the cytoplasm of neutrophil granulocytes (the most common type of white blood cell) and monocytes. They are particularly associated with systemic vasculitis, so called ANCA-associated vasculitides.
