GRANULOMATOSIS WITH POLYANGIITIS Flashcards
previously known as
weighers granulomatosis
definition of GPA
auto-immune inflammatory systemic disease of small vessels with associated with c-ANCA and PR3
where does GPA mostly affect
respiratory tract, skin, kidneys
the symptoms which scream GPA in clinical vignette
ENT symtoms
presentation
- refractory chronic sinusitis
- epistaxis (ntdebleeds)
- saddle nose deformity due to destruction of nasal septum
- haemoptysis
- non-blanching purpuric rash
- sudden onset deafness
haematological change in GPA
anaemia of chronic disease
renal involvement ion GPA
microhaematuria and proteinuria
ocular involvement in GPA
keratitis, conjunctivitis, scleritis, uveitis
what do x-rays show in GPA
cavitating granulomas
what does chest CT show in GPA
alveolar haemorrhage
GPA IS A…
granulomatous inflammation of the respiratory tract and a necrotising vasculitis affect small vessels and necrotising glomerulonephritis
initial test when you suspect GPA
c-ANCA TITRE
diagnostic test gor GPA
renal biopsy
if untreated what is the median survival
5 months
management when proven with renal biopsy
cyclophosphamide (or rituximab) and IV steroids
