ANCA-associated small vessel vasculitis Flashcards
what is ANCA?
anti-neutrophilic cytoplasmic antibodies
what immunoglobulin are they associated with?
IgG
what are the 3 types?
granulomatosis with polyangiitis (Wegner’s)
eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
microscopic polyangiitis
what is granulomatosis with polyangiitis (GPA)?
granulomatous inflammation affecting small and medium vessels in upper and lower resp tract, eyes and/or kidneys
which 3 places do symptoms most occur?
nasopharynx
lungs
kidneys
what are the ENT features?
sinusitis
bloody mucus
saddle nose
what are the resp symptoms?
cough
haemoptysis
cavitating nodules on CXR
what are the kidney symptoms?
necrotising glomerulonephritis
decreased urine production
increased BP
which antibodies are associated?
cANCA and anti-PR3
what is eosinophilic granulomatosis with polyangiitis (EPGA)?
eosinophilic granulomatous inflammation affecting small and medium vessels in resp tract and skin
what is it secondary to and why?
asthma due to increased eosinophils
how does it clinically present?
late onset asthma
high eosinophil count
sinusitis
which antibodies are associated?
anti-MPO and anti-PR3
what is microscopic polyangiitis?
necrotising vasculitis of small vessels with few immune deposits, usually affects pulmonary, renal and skin
how does it clinically present?
necrotising glomerulonephritis
