Wegener's granulomatosis

A 63-year-old man presented with a 6-month history of recurrent epistaxis, impaired hearing, arthralgia, and constitutional symptoms. He had had a productive cough with scanty streaks of blood in his sputum, and deterioration of hearing. These symptoms were followed by muscle weakness in his finger flexors with acral paraesthesiae. The past medical history was unremarkable and he was not on any medication. On examination, the patient was pale but had no skin lesions or signs of arthritis; the chest was clear on auscultation. Laboratory tests revealed a serum haemoglobin concentration of 6.7 m mol/L (typical range 8.38-10.55 m mol/L) and serum creatinine concentration of 187 mu mol/L (59-104 mu mol/L). Dipstick testing of his urine was strongly positive for haemoglobin and protein, and phase contrast microscopy of a freshly voided sample showed many erythrocyte casts and dysmorphic erythrocytes. Chest radiograph revealed round shadows in both lung fields and a CT scan confirmed bilateral non-cavitating nodules. The nasal mucosa was moderately inflamed and the biopsy sample revealed necrotising granulomatous inflammation. Examination of the ear showed tympanic effusion. Neurological examination and nerve-conduction studies were consistent with mononeuritis multiplex. An immunofluorescence assay for anti neutrophil cytoplasmic antibodies was positive with a cytoplasmic fluorescence pattern, and proteinase-3 antibodies were high in an enzyme-linked immunoassay. Renal biopsy confirmed pauci-immune crescentic glomerulonephritis. A diagnosis of Wegeners granulomatosis with involvement of middle ear, nose, lungs, kidneys, and peripheral nervous system was made. He was treated with high-dose intravenous methylprednisolone (500 mg daily for 3 days), then by monthly pulses of intravenous cyclophosphamide (starting with 750 mg/m(2), later adjusted to leucocyte nadir), and tapering doses of corticosteroids (starting at 1 mg/kg). All pulmonary lesions had resolved within 6 months, and the urinanalysis had returned to normal, with serum creatinine of 96 mu mol/L.