Results. The mean ؎ SD followup was 56.2 ؎ 31.7 months. Among the 72 patients studied, 93% achieved remission with CS therapy alone, and 35% relapsed, mainly during the first year of treatment. Among the 19 patients randomized to additional immunosuppression because of treatment failure or relapse, 5 of 10 receiving AZA and 7 of 9 receiving pulse CYC achieved remission, but the difference was not statistically significant. Survival rates in all patients at 1 and 5 years were 100% and 97%, respectively. At the end of followup, 79% of the patients whose disease was in remission required lowdose CS therapy, mainly to control respiratory disease. CS-related adverse events were observed in 31% of the 72 patients.Conclusion. In CSS patients with an FFS of 0, survival was excellent, confirming the predictive value of the FFS in this disease. First-line therapy with CS achieved remission in most patients, but relapses were common, and one-third of them required additional immunosuppressive therapy. AZA or pulse CYC was fairly effective in treating CS-resistant disease or major relapses. Over the long term, most patients continued to take oral CS, which might explain the high rate of CS-related adverse events.Churg-Strauss syndrome (CSS), which is also called allergic granulomatosis and angiitis, is characterized by necrotizing eosinophilic vasculitis affecting the small vessels and extravascular granulomas. The major clinical manifestations are asthma, hypereosinophilia, and extrapulmonary manifestations of systemic vasculitis. Antineutrophil cytoplasmic antibodies (ANCAs), ClinicalTrials.gov identifier: NCT00399399.

For patients with PAN or MPA with an FFS of 0, overall 5-year survival was good, but first-line corticosteroid treatment was able to achieve and maintain remission in only about half of the patients, and 40% of the patients required additional immunosuppressive therapy. Azathioprine or pulse cyclophosphamide was fairly effective for treating corticosteroid-resistant disease or major relapses.

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A 36-year-old woman experienced an excruciating right retro-orbital paroxysmal headache after a stroke of the anterior two-thirds of the right hemicord at the C1 level (anterior spinal artery territory) and the dorsal medulla (posterior spinal artery territory). A right vertebral artery dissection was demonstrated. This unusual infarct mimicked a cluster headache attack or paroxysmal hemicrania.

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