期刊
INTERNATIONAL IMMUNOPHARMACOLOGY
卷 45, 期 -, 页码 13-15出版社
ELSEVIER SCIENCE BV
DOI: 10.1016/j.intimp.2017.01.025
关键词
EGPA; Churg-Strauss syndrome; IVIG; Heart failure; Cardiac involvement
Glucocorticoids and immunosuppressive drugs represent the first-line treatment of eosinophilic granulomatosis with polyangiitis (EGPA, former Churg-Strauss syndrome), even though the combined therapy is not successful in achieving the disease remission in some patients with neurological or cardiac involvement. We describe a case of an EGPA male patient with impaired left ventricular function not responsive to glucocorticoid and immunosuppressive therapy. We observed that high-dose (2 g/kg/4 weeks) intravenous immunoglobulin (IVIG) therapy significantly improved cardiac function, which was deteriorated after reducing MG dose at 0.5 g/kg/4 weeks, and was restored increasing again IVIG dose to 2 g/kg/4 weeks. The finding highlights the relevance of IVIG as treatment of choice in EGPA patients with cardiac involvement not responsive to the standard glucocorticoid and immunosuppressive therapy. Moreover, at a follow-up of 24 months, the continuance of high dose MG therapy was required to maintain a sustained remission of the heart failure. (C) 2017 Elsevier B.V. All rights reserved.
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