Wegener granulomatosis with phlyctenular conjunctivitis

An 11 year old female presented with a history of sinusitis and of swelling of the right knee for which she was seen at an outlying hospital. She was treated with various antibiotics and then developed a nonpurulent conjunctivitis. About two weeks later she was found to have hematuria and proteinuria. When first seen at the Schneider Children's Hospital, the patient appeared ill and had photophobia. She had severe bilateral conjunctival injection but had no purulent discharge. This was found to be due to phlyctenular conjunctivitis. The rest of the physical examination was normal. ANCA came back positive with a high liter with cytoplasmic staining confirming the clinical impression of Wegener granulomatosis. Wegener granulomatosis used to be uniformly fatal, then was found to respond to aggressive treatment with oral daily cyclophosphamide and high doses of steroids for prolonged periods of time. However, the morbidity of the treatment was a serious problem. This has now been alleviated by the equally effective use of oral methotrexate and steroids. This patient has done well on this regimen.