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
how long should initial treatment be continued for
18 months and then you switch to methotrexate or azathioprine as maintenance
in life-threatening scenarios of GPA what do you use
plasma exchange as well as the cyclophosphamide and iV steroids
what are raised in GPA
ESR, CRP, PV and creatinine
what should everyone getting cyclophosphamide be also prescribed
co-trimoxazole due to the risk of pneumocystis jirovecci
what is the risk associated with long term treatment of cyclophosphamide
bladder cancer
