Skip to main content

Clinically isolated syndrome in collagen diseases; approaches and treatments

Background

Acute isolated neurological syndromes, such as optic neuropathy or transverse myelopathy, may cause diagnostic problems since they can be the first presentations in a number of demyelinating disorders including multiple sclerosis (MS) and collagen diseases. However, clinical presentation and lesions evidenced by magnetic resonance imaging may be similar. Collagen disease coexists in demyelinating disorders and frequently various collagen disease related autoantibodies are positive in daily practice.

Hence, the algorithm to overcome these diagnostic and therapeutic issues should be clarified.

B cell immunity in demyelinating disorders

In primary demyelinating disease, MS, a renewed interest in the role of humoral immunity in the pathophysiology has been investigated because oligoclonalIgG band in the CSF and increased intrathecalIgG synthesis are used as an auxiliary diagnosis measure. Moreover, in the secondary progressive MS, meningeal B-cell follicles are associated with early onset of the disease and severe cortical pathology. B cell but not plasma cell depletion therapy with single treatment by Rituximab in MS showed reduced inflammatory brain lesions and clinical relapses.

Oligodendropathy and astrocytopathy in demyelinating disorders

Neuromyelitisoptica (NMO) was previously considered to be a variant of MS but is now recognized as an astrocytopathy and secondary demyelinating event mimicking MS characteristics occurring due to autoantibody mediated mechanisms. Advancement of molecular biology makes it possible to differentiate MS by measuring abnormal autoantibody to aquaporin 4 (water channel). Interestingly, collagen diseases coexist more frequently with NMO than with MS. B cell depletion therapy with Rituximab has showed the same benefits, although, plasma exchange therapy is more effective with NMO than with MS.

TNF therapy and demyelinating event

A report indicates that adverse events such as the demyelinating lesion in the brain, optic neuritis, and neuropathy occurred after treatment with anti-TNF alpha therapy in collagen disease, and TNF antagonizing therapy showed worsening in a clinical trial with MS. Pathogenesis of these events such as primary or secondary demyelination are still in enigma.

In this presentation, I will decode the temporal and spatial demyelinating processes in collagen diseases and show practical approaches and treatments.

References

  1. 1.

    Franciotta D, Salvetti M: B cells and multiple sclerosis. Lancet Neurol. 2008, 7: 852-8. 10.1016/S1474-4422(08)70192-3.

    CAS  Article  PubMed  Google Scholar 

  2. 2.

    Bernatsky S, Renoux C: Demyelinating events in rheumatoid arthritis after drug exposures. Ann Rheum Dis. 2010, 69: 1691-3. 10.1136/ard.2009.111500.

    CAS  Article  PubMed  Google Scholar 

Download references

Author information

Affiliations

Authors

Corresponding author

Correspondence to Kazumasa Yokoyama.

Rights and permissions

This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

Reprints and Permissions

About this article

Cite this article

Yokoyama, K., Hattori, N. Clinically isolated syndrome in collagen diseases; approaches and treatments. Arthritis Res Ther 14, O4 (2012). https://doi.org/10.1186/ar3559

Download citation

Keywords

  • Multiple Sclerosis
  • Optic Neuritis
  • Secondary Progressive Multiple Sclerosis
  • Collagen Disease
  • Plasma Exchange Therapy