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Bronchoscopy yielded a transbronchial specimen showing necrotizing granulomatous inflammation including distinctive granulomatous microsabscesses. Stains for fungal and AFB organisms were negative. Several days later, ANCA and proteinase 3 antibody were positive. The diagnosis is granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis. It caused scleritis and secondary globe rupture, the nasopharyngeal/intracranial mass with mononeuritis multiplex (cranial nerves 7-11), and lung lesions. He was treated with prednisone 60 mg daily and rituximab 1 gram every 2 weeks for 2 doses. One month later, proteinase 3 antibody index was negative and chest CT showed interval decrease in the multiple mass-like opacities. Dedicated skull base and neck CT re-demonstrated the nasopharyngeal/intracranial mass relatively unchanged. The diagnosis of GPA appears obvious in retrospect! But there were many confounding factors in this case. The patient was cachectic because of cocaine abuse and difficulty swallowing. The nasopharyngeal/intracranial mass was large and eroding bone, suggesting neoplasm. Although GPA can cause this, intracranial lesions are rare and one of this size is extremely rare (1). The cavitary lung lesions certainly occur in GPA, but because of the cranial findings, neoplasm and infection were the primary considerations. The ruptured globe, which in retrospect probably resulted from scleritis, was superinfected and scarred, making the diagnosis of GPA difficult. The nasopharyngeal biopsy was interpreted as chronic inflammation, although in retrospect it did show small zones of "dirty" necrosis. Nasopharyngeal biopsies in GPA are known to be often falsely negative (2, 3). Even the bronchoscopic biopsy was not classic for GPA, missing a convincing vasculitis.