A Case of the Wegener's Granulomatosis Presenting with Cluster Headache

Background: Wegener's granulomatosis (WG) is a disorder of the upper airway, lungs, and kidneys, characterized histologically by necrotizing granulomas and vasculitis and, occasionally, headaches. Some clinical and histological characteristics of WG are not well known because WG is a rare disorder. We report a case of WG complicated with Cluster Headache (CH). Patient: A 41-year-old man complained about left hemifacial pain. Previously, he was treated as left maxillary sinusitis and had received analgesics and antibiotics systemically, but symptoms had not improved significantly. The pain onset every midnight for about 2 hours, and was very intense in the left temporal region. During that period, ipsilateral red eye and blepharoptosis appeared. Left cluster headache and myofacial pain disorder syndorome on left m. temporalis were diagnosed and treatments with sumatriptan (50 mg/day) and lomerizine (10 mg/day) systemically and trigger point block on left m. temporalis were started. However, his symptoms improved after applications of sumatriptan and trigger point block. He subsequently complained of onset of dyspnea, and arthritis of the fingers. A chest radiograph showed patchy infiltrates in the lung. From laboratory data showed abnormally below: WBC 9,610/mm ³, ESR 98 mm/h, CRP 9.2 mg/dl. Furthermore, RA and C-ANCA became positive. WG was diagnosed at the internal medicine and started pulse treatment with prednisolone and cyclophosphamide (methyl prednisolone 1 g/day for 3 days, and prednisolone 250 mg/day. cyclophosphamide 50 mg/day for 10 days). The pulse treatment has induced complete remission. Conclusion: It is still unknown whether there is some relationship between cluster headache and topical inflammation diseases such as Wegener's granulomatosis. Some reports indicated this relationship, but we need more clinical investigation about cluster headache. It is difficult to diagnose prolonged orofacial pain. We need careful observation.