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 journalArticle

29 Citations (Scopus)

Abstract

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
Volume34
Issue number4
DOIs
StatePublished - Jun 2010

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Peripherins
Autoimmunity
Nervous System
Immunoglobulin G
Primary Dysautonomias
Primary Ovarian Insufficiency
Intermediate Filament Proteins
Thyroiditis
Rhombencephalon
Inbred NOD Mouse
Sympathetic Ganglia
Type 1 Diabetes Mellitus
Autoantibodies
Epitopes
Pancreas
Ovary
Healthy Volunteers
Thyroid Gland
Epithelium
Biomarkers

Keywords

  • Autoimmune endocrinopathy
  • Dysautonomia
  • NOD mice
  • Peripheral neuropathy

ASJC Scopus subject areas

  • Immunology
  • Immunology and Allergy

Cite this

Peripherin-IgG association with neurologic and endocrine autoimmunity. / Chamberlain, Jayne L.; Pittock, Sean J; Oprescu, Anna Maria; Dege, Carissa; Apiwattanakul, Metha; Kryzer, Thomas J.; Lennon, Vanda A.

In: Journal of Autoimmunity, Vol. 34, No. 4, 06.2010, p. 469-477.

Research output: Contribution to journalArticle

Chamberlain, JL, Pittock, SJ, Oprescu, AM, Dege, C, Apiwattanakul, M, Kryzer, TJ & Lennon, VA 2010, 'Peripherin-IgG association with neurologic and endocrine autoimmunity', Journal of Autoimmunity, vol. 34, no. 4, pp. 469-477. https://doi.org/10.1016/j.jaut.2009.12.004
Chamberlain JL, Pittock SJ, Oprescu AM, Dege C, Apiwattanakul M, Kryzer TJ et al. Peripherin-IgG association with neurologic and endocrine autoimmunity. Journal of Autoimmunity. 2010 Jun;34(4):469-477. https://doi.org/10.1016/j.jaut.2009.12.004
Chamberlain, Jayne L. ; Pittock, Sean J ; Oprescu, Anna Maria ; Dege, Carissa ; Apiwattanakul, Metha ; Kryzer, Thomas J. ; Lennon, Vanda A. / Peripherin-IgG association with neurologic and endocrine autoimmunity. In: Journal of Autoimmunity. 2010 ; Vol. 34, No. 4. pp. 469-477.
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