Churg-Strauss syndrome (CSS), which is characterized by systemic small-vessel
vasculitis of unknown etiology, is associated with a history of
asthma. Although reports of CSS occurring in children are limited, effective treatment of pediatric patients with severe CSS remains challenging. A 10-year-old Japanese boy with a 6-month history of
asthma treated with a
leukotriene modifier,
pranlukast, developed high
fever, pleural infiltration, and
pericarditis that were associated with marked
hypereosinophilia (10,350 eosinophils/µl). Owing to his persistent high
fever,
mononeuritis multiplex, and severe
abdominal pain that was refractory to
prednisolone, his general condition progressively deteriorated thereafter. Although intravenous high-dose
immunoglobulin administration was transiently effective for
mononeuritis multiplex, the recurrent high
fever and severe
abdominal pain remained refractory. An endoscopic study revealed ulcerative lesions of the total colon. In this context, we treated the patient with an aggressive multidrug immunosuppressive regimen consisting of a high-dose
methylprednisolone pulse plus short-course intravenous
cyclophosphamide pulse
therapy, followed by oral
tacrolimus combined with
prednisolone. After the rescue multidrug treatment, his severe clinical signs dramatically subsided within a short time, and the concomitantly administered
prednisolone was successfully tapered without flare. At present, 12 months after the presentation, he is free from CSS signs or
therapy-related toxicity except for an occasional mild
asthma attack. Although further close observation should be needed to draw a long-term outcome in this patient, we believe that aggressive multidrug immunosuppressive treatment should be considered as an alternative rescue treatment in selected patients with severe CSS, even with pediatric-onset disease, that is refractory to
prednisolone.