The Dept of Anaesthesia & Intensive Care, CUHK thanks

for an unrestricted education grant
Home Feedback Contents

Wegener's

Up Antiphospholipid syn Polyarteritis nodosa SLE Wegener's


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

Further reading

http://vasculitis.med.jhu.edu/wg.htm


© Charles Gomersall December 1999

 

©Charles Gomersall, January, 2018 unless otherwise stated. The author, editor and The Chinese University of Hong Kong take no responsibility for any adverse event resulting from the use of this webpage.
Copyright policy    Contributors