Granulomatosis with Polyangitis (GPA) Flashcards
pathogenesis
granulomatous inflammation of respiratory tract, small and medium vesselsnecrotizing glomerulonephritis common
who gets it
more common in northern Europe (90%)m:f 1.5:135-55yrs
how does it present?
Constitutional symptoms & arthralgia are common
American college of rheumatology 1990 criteria for the classification of GPA - 2 or more criteria (88.2% sensitivity, 92% specificity)o
Nasal / oral inflammation: painful/painless oral ulcers or purulent/bloody nasal dischargeo
Abnormal chest radiograph: nodules, fixed infiltrates or cavitieso
Urinary sediment: microhematuria (>5 RBC/high power field) or red cell casts in urineo
Granulomatous inflammation on biopsy:
Histologic changes showing granulomatous inflammation within the wall of an artery or in the perivascular / extravascular area (artery or arteriole)
ENT symptoms
o sinusitiso nasal crustingo epistaxiso mouth ulcerso sensorineural deafnesso otitis media and deafnesso “saddle nose” due to cartilage ischaemia
Respiratory symptoms
o pulmonary infiltrateso cougho haemoptysiso diffuse alveaolar hemorrhageo cavitating nodules on CXR
Cutaneous symptoms
o palpable purpurao cutaneous ulcers
renal symptoms
necrotising glomerulonephritis
nervous symptoms
o mononeuritis multiplexo sensorimotor polyneuropathyo cranial nerve palsies
ocular symptoms
o conjunctivitiso episcleritiso uveitiso optic nerve vasculitiso retinal artery occlusiono proptosis
how is it investigated?
ESR, PV, CRP raised anaemia of chronic disease U&E for renal involvement urinalysis CXR biopsy of affected area - skin, kidney immunology - ANCA
how is it classified?
Localized – Upper/Lower respiratory tract disease without any other systemic involvement or constitutional symptomsEarly systemic - any, without organ or life threatening Generalized – renal (creatinine < 500) or other organ threateningSystemic – renal (creatinine > 500) or other vital organ failureRefractory – progressive disease unresponsive steroids + cyclo
How is it managed?
Localized/early systemic – Methotrexate + steroids (?Azathioprine + steroids)Generalised/systemic o Cyclophosphamide + steroids (1st line)o Rituximab + steroids (alternative)o plasma exchange if creatinine > 500o Followed by azathioprine with alternatives being methotrexate. mycophenolate mofetil or leflunomide• Refractory – IV immunoglobulins, Rituximab
