Successful Treatment of a Patient with Severe Churg-Strauss Syndrome by a Combination of Pulse Corticosteroids, Pulse Cyclophosphamide, and High-Dose Intravenous Immunoglobulin

Abstract

A 24-year-old woman with a 4-year history of bronchial asthma suffered from bloody sputum, numbness of the extremities, elevated eosinophil count, and hypoxemia. A diagnosis of alveolar hemorrhage was made by bronchoalveolar lavage. Echocardiogram revealed severe hypokinesis of the left ventricular wall. Her respiratory condition deteriorated despite administration of pulse corticosteroids. A second pulse corticosteroids and pulse cyclophosphamide followed by high-dose intravenous immunoglobulin brought about a dramatic improvement of alveolar hemorrhage, cardiac impairment, and peripheral neuropathy. Levels of antimyeloperoxidase-antineutrophil cytoplasmic antibodies, soluble thrombomodulin, soluble interleukin-2 receptor, eosinophil cationic protein were elevated and returned to the normal range in remission. The combination of pulse corticosteroids, pulse cyclophosphamide, and high-dose intravenous immunoglobulin seemed effective for the acute phase of severe Churg-Strauss syndrome.

• Churg-Strauss syndrome
• alveolar hemorrhage
• cardiac failure
• neuropathy
• immunoglobulin

Abbreviations
CSS:	=	Churg-Strauss syndrome
IVIG:	=	intravenous immunoglobulin
MPO-ANCA:	=	antimyeloperoxidase-antineutrophil cytoplasmic antibodies
PSL:	=	prednisolone
EF:	=	ejection fraction
LV:	=	left ventricle
BAL:	=	bronchoalveolar lavage
CMAP:	=	compound muscle action potential
CVRR:	=	coefficient of variation of electrocardiogram R-R interval
IL:	=	interleukin