Flashcards in Vasculitis Deck (17):
What is vasculitis - how is it classified?
Vasculitis is defined as inflammation of blood vessels. It may result in vessel wall thickening, stenosis, and occlusion with subsequent ischemia.
The clinical presentation varies according to the histologic type of inflammation, the size of the involved blood vessel segment, and the distribution of the involved vessels.
The classification is according to the International Chapel Hill Consensus.
What are the different types of vasculitides?
-large vessel vasculitis
-medium vessel vasculitis
-immune complex small-vessel vasculitis
-ANCA-assoc. small vessel vasculitis
What is large vessel vasculitis? What are the two main large vessel vasculitides? how do these differ in age affected?
The term ‘large vessel vasculitis’ applies to primary vasculitis that causes chronic granulomatous inflammation predominantly of the aorta and its major branches
Temporal (giant cell) arteritis and Takayasu arteritis (TA). These arteritides differ in the age of onset, with the former rarely occurring before the age of 50 years and the latter rarely after 50 years.
TA - young women in 20-30's
What are the early features of large vessel vasculitides?
Early features include non specific features such as low-grade fever, malaise, night sweats, weight loss, arthralgia and fatigue. Following this patients can experience claudicant symptoms in both the upper and lower limbs.
What can happen if large vessel vasculitis goes untreated?
vascular stenosis and aneursyms can occur. This results in reduced pulses and bruits.
What is seen on blood tests in large vessel vasculitis?
ESR, PV and CRP and elevated.
What can be seen on imaging in large vessel vasculitis?
Imaging such as MR angiography can detect thickened vessel walls and stenosis.
what is the mainstay of treatment of large vessel vasculitis?
The mainstay of treatment is corticosteroids, starting at 40-60mg prednisolone and gradually reducing. Steroid sparing agents such as methotrexate and azathioprine may be added.
How can small to medium vessel vasculitis be classified?
This group can be divided into ANCA positive and negative conditions.
Small to medium vessel vasculitis:
-what features are similar?
Fever and weight loss
A raised, non blanching purpuric rash
Lung opacities on x-ray
What are the four types of vasculitis assoc. with ANCA?
Granulomatosis with polyangitis (wegners):
-necrotising glomerulonephritis common
-pulmonary capilaritis often occurs
Renal limited vasculitis:
-pauci-immune necrotising glomerulonephritis
Eosinophilic Granulomatosis with Polyangiitis (Chrug-strauss) syndrome:
-eosinophil rich and granulomatous inflammation involving respiratory tract
What symptoms are seen in granulomatosis with polyangitis?
ENT symptoms are common in GPA and include nose bleeds, deafness, recurrent sinusitis and nasal crusting (over time there can be collapse of the nose). Respiratory symptoms such as haemoptysis and cavitating lesions on x-ray are frequently seen.
What symptoms are seen in eosinophilic granulomatosis with polyangitis?
This condition is characterised by late onset asthma, rhinitis and a raised peripheral blood eosinophil count.
Neurological symptoms such as mono neuritis multiplex are common.
What symptoms are seen with microscopic polyangitis?
The most important complication is glomerulonephritis, which occurs in up to 90% of patients.
what is included in the investigation of vasculitis?
ESR, PV and CRP and raised
Anaemia of chronic disease is common.
U+E looking for renal involvement
Anti-neutrophil cytoplasmic antibody (ANCA)
Urinalysis (looking for renal vasculitis)
Biopsy of an affected area e.g. skin or kidney is often helpful in confirming the diagnosis
What is the management of vasculitis?
Most cases of ANCA associated vasculitis require treatment with IV steroids and cyclophosphamide due to their aggressive disease course.
There are some situations e.g. GPA localised to the nose, where other options would be considered.