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Four additional <jats:styled-content style=\"fixed-case\">CIDP<\/jats:styled-content> patients with anti\u2010<jats:styled-content style=\"fixed-case\">NF<\/jats:styled-content>155 antibodies referred from other clinics were enrolled for clinical characterization.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The positivity rate for anti\u2010<jats:styled-content style=\"fixed-case\">NF<\/jats:styled-content>155 antibodies in <jats:styled-content style=\"fixed-case\">CIDP<\/jats:styled-content> patients was 18% (9\/50), who all showed a predominance of IgG4 subclass. No other subjects were positive, except one <jats:styled-content style=\"fixed-case\">GBS<\/jats:styled-content> patient harboring IgG1 anti\u2010<jats:styled-content style=\"fixed-case\">NF<\/jats:styled-content>155 antibodies. No anti\u2010<jats:styled-content style=\"fixed-case\">NF<\/jats:styled-content>155 antibody carriers had anti\u2010<jats:styled-content style=\"fixed-case\">NF<\/jats:styled-content>186 antibodies. Anti\u2010<jats:styled-content style=\"fixed-case\">NF<\/jats:styled-content>155 antibody\u2010positive <jats:styled-content style=\"fixed-case\">CIDP<\/jats:styled-content> patients had a significantly younger onset age, higher frequency of drop foot, gait disturbance, tremor and distal acquired demyelinating symmetric phenotype, greater cervical root diameter on magnetic resonance imaging neurography, higher cerebrospinal fluid protein levels, and longer distal and F\u2010wave latencies than anti\u2010<jats:styled-content style=\"fixed-case\">NF<\/jats:styled-content>155 antibody\u2010negative patients. Marked symmetric hypertrophy of cervical and lumbosacral roots\/plexuses was present in all anti\u2010<jats:styled-content style=\"fixed-case\">NF<\/jats:styled-content>155 antibody\u2010positive <jats:styled-content style=\"fixed-case\">CIDP<\/jats:styled-content> patients examined by neurography. Biopsied sural nerves from two patients with anti\u2010<jats:styled-content style=\"fixed-case\">NF<\/jats:styled-content>155 antibodies demonstrated subperineurial edema and occasional paranodal demyelination, but no vasculitis, inflammatory cell infiltrates, or onion bulbs. Among anti\u2010<jats:styled-content style=\"fixed-case\">NF<\/jats:styled-content>155 antibody\u2010positive patients, treatment responders more frequently had daily oral corticosteroids and\/or immunosuppressants in addition to intravenous immunoglobulins than nonresponders did.<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>Anti\u2010<jats:styled-content style=\"fixed-case\">NF<\/jats:styled-content>155 antibodies occur in a subset of <jats:styled-content style=\"fixed-case\">CIDP<\/jats:styled-content> patients with distal\u2010dominant involvement and symmetric nerve hypertrophy.<\/jats:p><\/jats:sec>","DOI":"10.1002\/acn3.248","type":"journal-article","created":{"date-parts":[[2015,9,11]],"date-time":"2015-09-11T05:44:51Z","timestamp":1441950291000},"page":"960-971","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":197,"title":["Characterization of IgG4 anti\u2010neurofascin 155 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