In February, 2007, a 70-year-old man visited his general practitioner after 6 weeks of weight loss, shortness of breath on slight exertion, and night sweats. 3 weeks previously, he had had a tooth extracted, and subsequently developed sinusitis, left-sided tinnitus, and reduced hearing in the left ear. In the week before his appointment, the tinnitus and hearing loss had become bilateral. On examination, the patient was pale and looked tired. He had mouth ulcers, a palpable left supraclavicular lymph node, and a hard mass on the right side of the abdomen. Blood tests showed a microcytic anaemia (haemoglobin concentration 50·9 mmol/L), and an ESR and C-reactive-protein concentration of more than 120 mm/h and 250 mg/L, respectively. Renal and liver function tests gave normal results. Blood, but no protein, was found in the urine. Chest radiography revealed shadows consistent with metastases (fi gure). An otolaryngologist ascribed the tinnitus to temporomandibular dysfunction caused by dental extraction, and the hearing loss to adenoid enlargement; he also postulated that the mouth ulcers represented paraneoplastic pemphigus. CT of the abdomen showed cancer in the ascending colon. Histopathological examination of a biopsy sample obtained by colonoscopy revealed a moderately diff erentiated adenocarcinoma. We therefore told the patient that his life was threatened by disseminated cancer. However, CT of the chest then indicated that the lesions in the lungs were likely to be vasculitic rather than metastatic. Further blood tests showed a high concentration of antineutrophil cytoplasmic antibody specifi c for serine proteinase 3 (PR3-ANCA) (0·08 U/L, normal <0·006 U/L). We realised that a diagnosis of Wegener’s granuloma tosis, as well as explaining this result, could account for the vasculitis, sinusitis, tinnitus, hearing loss, and even lymphadenopathy. Moreover, fi breoptic rhinos copy revealed nasopharyngeal ulcers, consistent with Wegener’s granulomatosis. Bronchoscopy, to confi rm the origin of the pulmonary lesions, was not done because the patient was deemed too frail. The patient was treated for Wegener’s granulomatosis with immunoglobulin and methylprednisolone, and then with oral prednisolone. Since he had cancer, we did not prescribe cytotoxic drugs. Within 3 weeks, the pulmonary lesions resolved. The colonic tumour was subsequently resected, and staged as T4 N0 M0; the patient was relieved to learn of his revised prognosis. After the resection, we treated Wegener’s granulomatosis with cyclo phosphamide. However, the patient developed pulmonary aspergillosis, so treatment with cyclophosphamide was stopped. The patient then developed ulcers in the mouth and nasopharynx, digital infarctions, and mononeuritis multiplex. The PR3-ANCA concentration increased to 0·152 U/L. Plasma exchange substantially reduced the symptoms; the patient was then treated with rituximab. When last seen, in November, 2007, the patient continued to improve, albeit slowly; his cancer had not recurred. Wegener’s granulomatosis causes infl ammation of small blood vessels, characteristically aff ecting the upper respiratory tract, lungs, and kidneys. Other features can include mononeuritis multiplex, polyneuropathy, skin nodules, purpura, and infl ammation of the eye. Although high levels of PR3-ANCA can be found in Churg-Strauss syndrome or microscopic polyangiitis, this fi nding is more strongly associated with Wegener’s granulomatosis, being present in over 80% of people at the time of diagnosis. Unlike other causes of pulmonary vasculitis, Wegener’s granulomatosis causes nodular or cavitating lesions, which can be mistaken for metastases. Ironically, Wegener’s granulomatosis, and other causes of smallvessel vasculitis, are associated with an increased risk of cancer.
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