Wegeners granulomatosis

A systemic vasculitis characterised by necrotising granulomas of the upper and lower respiratory tract, glomerulonephritis and small vessel vasculitis. Wegener's granulomatosis is associated with positive core antineutrophil cytoplasmic antibodies (c-ANCA), particularly granular with central attenuation on immunofluorescence. Intensive care admission is usually because of renal and/or pulmonary involvement.

Renal failure

Focal necrotising glomerulonephritis leads to progressive renal failure. Treatment with steroids and cyclophosphamide may give complete remission.

Upper airway disease

Most patients will have nasal symptoms including epistaxis, nasal discharge and septal perforation. Intensive care admission may be required rarely for severe epistaxis. Ulcerating lesions of the larynx and trachea may cause subglottic stenosis. This is usually insidious but may present problems on attempted intubation.

Pulmonary involvement

Usually associated with haemoptysis, dyspnoea and cough with rounded opacities on the CXR. There may be cavitation. Nodules may be solitary. Alveolar haemorrhage may be life threatening. The mainstay of treatment is steroids and cyclophosphamide which may produce complete remission. Plasma exchange may be helpful.

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