Screening for lipoprotein receptor-related protein 4-, agrin-, and titin-antibodies and exploring the autoimmune spectrum in myasthenia gravis

Isabell Cordts, Nicolas Bodart, Kathi Hartmann, Katerina Karagiorgou, John S. Tzartos, Lin Mei, Jens Reimann, Philip van Damme, Michael H. Rivner, Alain Vigneron, Joachim Weis, Jörg B. Schulz, Socrates J. Tzartos, Kristl G. Claeys

Research output: Contribution to journalArticle

21 Scopus citations

Abstract

In autoimmune myasthenia gravis (MG), the identification of antibodies and characterization of serological subgroups is of great importance for diagnosis and management of the disease. Our aims were to study the frequency of antibodies against lipoprotein-related protein 4 (LRP4), agrin, and titin using the most recent techniques, and to characterize corresponding clinical features and autoimmune diseases (AID) in 100 MG-patients. The antibody frequencies in the 55 AChR-antibody positive patients were 7% LRP4, 5% agrin, 53% titin, and in the 45 AChR-antibody negative patients 2% MuSK, 2% LRP4, 2% agrin, and 27% titin. LRP4-MG presented late-onset age, mild symptoms, good therapeutic response, and no thymic changes. Agrin-MG showed early onset age, mild-to-severe symptoms, and moderate treatment response. The phenotype of titin-MG depended on AChR-antibodies: AChR-antibody negative patients presented with mostly mild limb muscle weakness, whereas AChR-antibody positive patients showed more frequently severe symptoms, including myasthenic crisis, bulbar predominance, and thymoma. Additional AID were detected in 32% of MG-patients, most frequently Hashimoto’s thyroiditis (21%). Based on our data, we recommend the detection of LRP4-antibodies for at least AChR-antibody negative MG-patients and titin-antibodies for all MG-patients. We propose taking an accurate medical history for typical symptoms of Hashimoto’s thyroiditis in MG-patients.

Original languageEnglish (US)
Pages (from-to)1-11
Number of pages11
JournalJournal of Neurology
DOIs
StateAccepted/In press - May 17 2017

Keywords

  • Antibodies
  • Autoimmune diseases
  • LRP4
  • Seronegative
  • Thyroid
  • Titin RIPA

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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    Cordts, I., Bodart, N., Hartmann, K., Karagiorgou, K., Tzartos, J. S., Mei, L., Reimann, J., van Damme, P., Rivner, M. H., Vigneron, A., Weis, J., Schulz, J. B., Tzartos, S. J., & Claeys, K. G. (Accepted/In press). Screening for lipoprotein receptor-related protein 4-, agrin-, and titin-antibodies and exploring the autoimmune spectrum in myasthenia gravis. Journal of Neurology, 1-11. https://doi.org/10.1007/s00415-017-8514-z