The occurrence of anti-titin antibodies and thymomas: a population survey of MG 1970-1999

Neurology. 2002 Jul 9;59(1):92-8. doi: 10.1212/wnl.59.1.92.

Abstract

Objective: To estimate the incidence of elevated anti-titin antibodies titers and of thymomas in a population of patients with MG using various statistics and associations.

Methods: Extensive epidemiology, systematic measurement of anti-titin antibodies, and histologic assessment of thymomas according to the new World Health Organization classification.

Results: The mean annual incidence rate of MG per million population was 8.3. The analogous mean rate of thymomas was 2.0, out of which MG was encountered in about 20%. A thymoma was coexistent in 7% of the patients with MG. The finding of titin autoantibodies and the coexistence of thymomas were both associated with age at the appearance of MG. In patients with MG with a thymoma, the frequency of seropositivity was 68%, whereas acetylcholine receptor (AChR) autoantibodies were detected in all such sera. Titin autoantibody-positive sera were also anti-AChR antibodies positive. Further, all serum samples negative for anti-AChR antibodies were devoid of anti-titin antibodies. Titin autoantibodies were not detected in nonthymoma early-onset MG.

Conclusion: Apart from MG with a thymoma, the finding of the titin autoantibodies was observed to be an exclusive feature of late-onset MG, the frequency being 55%. No data were found to suggest that patients with MG were more likely to present with thymic tumors than other patients exhibiting thymic neoplasia. In about 80%, such tumors in MG were composed of cortical cells. The concept of the anti-titin antibodies merely as a paraneoplastic marker in MG was not supported by these data.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Age Distribution
  • Aged
  • Aged, 80 and over
  • Child
  • Connectin
  • Female
  • Humans
  • Incidence
  • Male
  • Middle Aged
  • Muscle Proteins / immunology*
  • Protein Kinases / immunology*
  • Seroepidemiologic Studies
  • Sex Distribution
  • Thymoma / epidemiology*
  • Thymoma / immunology*
  • Thymus Neoplasms / epidemiology*
  • Thymus Neoplasms / immunology*

Substances

  • Connectin
  • Muscle Proteins
  • TTN protein, human
  • Protein Kinases