Journal List > J Korean Rheum Assoc > v.16(4) > 1003694

Cho, Chang, Chae, and Kim: Churg-Strauss Syndrome Presenting as Transverse Myelitis

Abstract

Churg-Strauss syndrome (CSS), also known as allergic granulomatous angiitis, is a rare disorder characterized by the presence of asthma, eosinophilia and small-to-medium-sized vessels vasculitis. The peripheral nervous system is frequently involved in CSS, but central nervous system (CNS) involvement is rare. Furthermore, transverse myelitis (TM) as a presenting symptom in patients with CSS is extremely rare. We report here on a 60-year-old female who presented peripheral eosinophilia, lung eosinophilic infiltration, lung vasculitis, and TM. She was diagnosed as CSS based on clinical manifestation, pathologic findings, and the use of magnetic resonance imaging (MRI).

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Fig. 1.
Lung setting computed tomography (CT) scan of the chest reveals a well defined nodule in the right upper lobe (0.7 cm in size, arrow).
jkra-16-328f1.tif
Fig. 2.
MRI findings. (A) Sagittal T2 weighted image (T2WI) of the T spine reveals diffuse swelling and increased signal intensity in T2–5 spinal cord (arrow), and focal increased signal intensity lesion in T6 spinal cord (arrowhead). (B) A four-month follow-up sagittal T2WI of the T spine reveals more resolution in the previous lesions, but there are focal residual signal intensity lesions in T4 and 6 spinal cord (arrow and arrowhead).
jkra-16-328f2.tif
Fig. 3.
Localized eosinophilic pneumonia (H&E, ×100, A) with perivascular eosinophilic infiltration in lung tissue (H&E, ×200, B).
jkra-16-328f3.tif
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