Vasculitis Flashcards
What is vasculitis?
inflammation of the blood vessels (arteries, arterioles, veins, venules and capillaries)
What does primary vasculitis result from?
results from an inflammatory response that targets the vessel walls, can be autoimmune
What does secondary vasculitis result from?
triggered by an infection, drug or toxin and can occur as part of an inflammatory disorder or cancer
What is large vessel vasculitis?
primary vasculitis that causes chronic granulomatous inflammation predominantly of the aorta and its major branches
What are the two subtypes of LVV?
Giant cell arthritis
Takayasu arthritis
What populations does Takayasu arthritis affect?
East Asian populations - some western
Middle aged women - 20-30yrs
What populations does Giant cell arthritis affect?
over 50s
What does Giant cell arthritis cause?
temporal arteritis
What are the main signs of LVV?
Bruit claudication headaches BP difference of extremities unilateral temperal headache jaw claudication scalp tenderness risk of blindness polymyalgia rheumatica
What are the investigations for LVV?
ESR, plasma viscosity increased CRP temporal artery biopsy angiogram PET
What is the management for LVV?
40-60mg prednisolone then gradually decrease
use steroid sparing agent (methotrexate) if necessary
What is the histological sign of LVV?
granulomatous infiltraiton of the walls of the large vessels
What are the different subgroups of Small vessel vasculitis?
ANCA positive
ANCA negative
What are the ANCA positive SVV?
GPA - granulomatosis with polyangitis
EGPA - eosinophillic granulomatosis with polyangitis
Microscopic polyangitis
What is EGPA also called?
Chaug-Strass Syndrome
What does GPA comprise of?
granulomatous inflammation of the respiratory tract, small and medium vessels
necrotising glomonephritis is also common
What is GPA associated with?
cANCA and PR3
What are the symptoms of GPA?
nose bleeds deafness recurrent sinusitis nasal crusting (over time there can be collapse of the nose) haemoptysis cavitating lung lesions on x-ray
What is EGPA characterised by?
same as GPA, but with late onset asthma, rhinitis, and increased eosinophil count
mono neuritis multiplex can also occur
What is EGPA associated with?
pANCA
What is Microscopic polyangitis characterised by?
necrosing vasculitis
90% get glomornephritis
What is MP associated with?
pANA and MPO
How do you test for an ANCA?
immunofluresence
What can cause ANCA positive conditions?
HLA genese, CD226 and IL-10
silica
Staph. aureus, E.coli, K. pneumoniae
Drugs - propylthiouracil, hydralazine, minocycline
How is EGPA diagosed?
eosinophilia of more than 10% of blood
What is the pathophysiology of ANCA positive conditions?
associated with immune complexes so complement is consumed meaning C3/C4 may fall
What is the management for ANCA positive conditions?
IV steroids and cyclophosphamide when aggressive
if localised/early - methotrexate and steroids
if creatinine is >500 - plasma exchange
if refractory - IV immunoglobulins and Rituximab
What are the investigations for ANCA positive conditions?
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)
CXR
Biopsy of an affected area e.g. skin or kidney is often helpful in confirming the diagnosis
What is the ANCA negative condition?
Henoch-Schönlein purpura (HSP)
Desribe Henoch-Schönlein purpura (HSP)?
IgA mediated disorder of generalised vasuclitis affecting the small vessels of the skin, GI tract, kidneys, joints, lungs and CNS
What populations does HSP commonly affect?
children ages 2-11
75% patients have a proceeding URTI, pharangeal infection or GI infection (normally staph aureus)
What are the common symptoms for HSP?
purpuric rash over the buttocks and lower limbs abdominal pain
vomiting
joint pain
What is the management of HSP?
symptoms resolve within 8 weeks normally
need to do renal analysis to check for renal involvement
