Wegener's

Forthcoming BASIC courses: August - Brisbane, Hawkes Bay, Kuala Lumpur, Bali; September - Hong Kong; October - Sydney, Chennai
Click here for details

Wegener’s granulomatosis

Necrotizing granulomatous inflammation that affects small and medium sized blood vessels, upper and lower respiratory tract, and kidneys

Clinical features

Eyes

- involved in 25-50%
- conjunctivitis, episcleritis, scleritis, uveitis, vasculitis of retina, optic neuritis, occlusion of central retinal artery
- proptosis due to granulomatous pseudotumours of the orbit

ENT

- conductive or sensorineural deafness
- otitis; may lead to mastoiditis
- persistent nasal congestion
- nasal mucosal thickening
- ulceration and crusting of nasal mucosa; may lead to perforation of septum and facial bone destruction
- chondritis of nose leads saddle nose deformity
- subglottic region is most frequently involved area of tracheobronchial tree. Ulcerative lesions may lead to subglottic stenosis causing respiratory distress. May be confused with asthma

Lung

- usually asymptomatic
- single/multiple nodules or masses (+/- cavitation) are most frequent manifestations
- pulmonary infiltrates rare and should alert clinician to the possibility of alveolar haemorrhage
- pleural effusion in 10%

Renal

- usual form of involvement is a focal segmental glomerulonephritis
- if untreated renal involvement may progress to irreversible renal insufficiency

Skin

- urticarial lesions
- papules
- vesicles
- erythema
- petechiae
- ulcerative lesions
- pyoderma gangrenosum

Joints

- migratory arthralgias involving single or multiple joints
- symmetrical polyarthritis

CNS

- mononeuritis multiplex
- cranial nerve palsies

Diagnosis

- requires histological proof for definitive diagnosis
- positive cytoplasmic staining antineutrophil cytoplasmic antibody test has specificity of > 90% for Wegener's
- mild normochromic, normocytic anaemia
- thrombocytosis in about 1/3
- WCC usually normal, occasionally elevated
- ESR and CRP raised
- high titres of rheumatoid factor in 1/2
- urinalysis: red cells and casts in those with renal involvement
- sinus XR
- CXR abnormal in 55-95% of cases

Treatment

- cyclophosphamide and prednisone orally
- IV boluses of cyclophosphamide for those not controlled with oral regime