Wegeners Granulomatosis

(WG)

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Features

-  Medium & small venuole & arteriole vasculitis

-  Intra or extravascular granulomas

-  Peak incidence 40-60 yrs

-  Male > female

-  May be associated w Microscopic Polyangitis (only small vessels)

 

Associated S/S

-  Fever, weight loss, anorexia

-  Respiratory tract granulomatous inflammation

-  Acute alveolitis

-  Haemoptysis, dyspnoea, cough

-  Nasal ulceration

-  Pauci-immune necrotising glomerulonephritis

-  Haematuria, proteinuria, renal insufficiency

-  Neurological involvement

-  30% pts

-  Stroke, seizure

-  Cranial nerve palsies: II, VI, VII

-  Opthalmoplegia

-  Peripheral neuropathy: acute mononerutis multiplex

 

Dx

-  2+ of (92% specific, 88% sensitive)

-  Chronic sinusitis: nasal or oral inflammation w discharge (may be purulent)

-  CXR: nodules, infiltrates, cavities

-  Urinalysis: microscopic haematuria, RBC casts

-  Biopsy: lung, nerve, nasal mucosa

 

-  Ix

-  FBE: anaemia, thrombocytosis

-  Autoimmune screen: c-ANCA, RF

-  ESR, CRP

-  CXR

-  Urinalysis

-  Biopsy

-  Elevated CK

-  LP: pleocytosis, elevated protein

-  EMG, MRI, CSF: localise neuro lesion

 

-  Mx

-  Cyclophosphamide: oral pulsed therapy