A case of Crow-Fukase syndrome with elevated soluble interleukin-6 receptor in cerebrospinal fluid. Response to double-filtration plasmapheresis and corticosteroids.
We report the case of a 33-year-old Japanese male who presented with thrombocytosis, lower limb edema, severe polyneuropathy with elevated cerebrospinal fluid (CSF) protein level and serum IgA lambda monoclonal component, fulfilling the manifestations of Crow-Fukase syndrome. A high level of soluble interleukin-6 receptor in the CSF was also found, which fluctuated in parallel with the clinical course. Initial treatment with double-filtration plasmapheresis (DFPP) reduced the serum IgA paraprotein level with improvement of the sensory component of the polyneuropathy and decrease of soluble interleukin-6 receptor in the CSF. The remaining clinical features waned off after steroid treatment. The possible role of interleukin-6 in the pathogenesis of the Crow-Fukase syndrome and the utility of DFPP treatment are discussed.
D000328:Adult; D003131:Combined Modality Therapy; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D015850:Interleukin-6 / Q000134:cerebrospinal fluid* / Q000378:metabolism; D008297:Male; D016878:POEMS Syndrome / Q000134:cerebrospinal fluid* / Q000378:metabolism / Q000628:therapy*; D010956:Plasmapheresis; D018123:Receptors, Interleukin / Q000378:metabolism*
D005938:Glucocorticoids; D015850:Interleukin-6; D018123:Receptors, Interleukin