Maintaining remission in a patient with vasculitis.

BACKGROUND

A 40-year-old man was referred to a specialist vasculitis center 4 years after being diagnosed with Wegener's granulomatosis. At the time of diagnosis he had presented with skin, ear, nose and throat involvement, pulmonary hemorrhage, and microscopic hematuria. Remission was achieved with plasma exchange and with daily oral prednisolone and cyclophosphamide. The patient was switched to maintenance treatment with azathioprine and prednisolone but suffered a relapse shortly afterwards. Further treatment with cyclophosphamide achieved a second remission, but the patient relapsed again despite remission-maintaining treatment with mycophenolate mofetil.

INVESTIGATIONS: Physical examination, laboratory testing, serological testing, culture of eye swabs and sputum, chest X-ray, chest CT scan, head MRI scan, bronchoscopy and bronchoalveolar lavage, and consultation with ophthalmological and otorhinolaryngological specialists.

DIAGNOSIS: Refractory Wegener's granulomatosis with involvement of the eyes, upper and lower respiratory tracts, and kidneys.

MANAGEMENT: Disease activity was controlled following treatment with deoxyspergualin and oral steroids in addition to aggressive management of intercurrent infections with repeated courses of oral and intravenous antibiotics. Relapses that occurred when deoxyspergualin was discontinued were treated with repeated courses of deoxyspergualin or with pulsed intravenous cyclophosphamide. Remission was achieved with rituximab. Pulmonary disease was closely monitored with repeated bronchoscopy.