Peripherin-IgG association with neurologic and endocrine autoimmunity

Jayne L. Chamberlain, Sean J. Pittock, Anna Maria Oprescu, Carissa Dege, Metha Apiwattanakul, Thomas J. Kryzer, Vanda A. Lennon

Research output: Contribution to journalArticlepeer-review

31 Scopus citations


Peripherin-IgG has been reported a pertinent autoantibody in non-obese type 1 diabetic (NOD) mice. However, it has not previously been recognized in any human disease. In blinded evaluation of serum for markers of neurological autoimmunity in a high-volume diagnostic laboratory, we incidentally identified 26 patients (61% female) with an IgG that bound selectively to neural elements in enteric ganglia, sympathetic nerve trunks and discrete nerve tracts in mid-brain and hind-brain. The target antigen was identified as peripherin, a 55. kDa - type III intermediate filament protein. Review of clinical histories revealed that 54% of seropositive patients had dysautonomia (predominantly gastrointestinal dysmotility), 30% had neuropathies with varied sensory symptoms and 35% had clinical or serological evidence of endocrinopathy (type 1 diabetes, thyroiditis or premature ovarian failure). Collectively, 73% had autonomic dysfunction or endocrinopathy. None of 173 healthy subjects was seropositive. Subsequent western blot evaluation of archival sera from patients with small fiber/autonomic neuropathies (with or without endocrinopathy) revealed a 33% seropositivity rate for peripherin-IgG. Our further demonstration that peripherin-immunoreactive autonomic fibers in pancreas, thyroid and ovary are juxtaposed to endocrine epithelium, complement our clinical observations in suggesting that neuronal elements may be a pertinent initial target for immune attack in multiple forms of endocrine autoimmunity (intermolecular epitope spreading). It remains to be determined whether or not peripherin-IgG is predictive for development of small fiber neuropathy (autonomic or somatic).

Original languageEnglish (US)
Pages (from-to)469-477
Number of pages9
JournalJournal of Autoimmunity
Issue number4
StatePublished - Jun 2010


  • Autoimmune endocrinopathy
  • Dysautonomia
  • NOD mice
  • Peripheral neuropathy

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology


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