Current perspectives on the diagnosis, assessment, and management of vasculitic neuropathy

ABSTRACT

Introduction

Vasculitic neuropathy can present associated with both primary and secondary systemic vasculitis as a result from underlying diseases such as rheumatic diseases and infections, Moreover, confined vasculitis in the peripheral nervous system may be present. Thus, the diagnosis and management of vasculitic neuropathy require multidisciplinary approaches.

Areas covered

Current views as well as relevant clinical research on the diagnosis, assessment, and management of vasculitic neuropathy are reviewed to suggest appropriate treatment strategies. We searched PubMed and Google Scholar for reports published between July 2017 and July 2022.

Expert opinion

For the treatment of vasculitic neuropathy, determining the causative primary disease is important and often requires diagnosis by tissue biopsy. Due to the scarce research on the treatment of vasculitic neuropathy, treatment is empirically based on findings from studies of systemic vasculitides involving other organs, particularly antineutrophil cytoplasmic antibody-associated vasculitis. In addition to conventional glucocorticoids and immunosuppressive agents, complement-targeted therapy, anti-B-cell therapy, and disease-specific molecular targeted therapies have recently gained relevance. Future research is needed to develop new patient-specific therapeutic options.

KEYWORDS:

• Vasculitis
• vasculitic neuropathy
• antineutrophil cytoplasmic antibody-related vasculitis
• axonal degeneration
• treatment

Article highlights

• The search for a causative primary disease often requires diagnosis by tissue biopsy; this is important in the treatment of vasculitic neuropathy.

• As most vasculitic neuropathies are linked with systemic diseases, multidisciplinary approaches involving neurologists, rheumatologists, nephrologists, internists, and pathologists are needed for diagnosis and management.

• Despite lacking evidence for their treatment, primary vasculitic neuropathy is treated empirically based on vasculitis involving other organs, particularly antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis.

• Some rheumatic diseases, such as systemic lupus erythematosus, are treated based on their own evidence.

• In addition to conventional glucocorticoids and immunosuppressive agents, complement-targeted therapies, anti-B-cell therapies, and more disease-specific molecular targeted therapies are becoming increasingly important.

• Symptomatic treatment and rehabilitation are important as well, since neuropathic symptoms often negatively affect the activities of daily living and quality of life.

Acknowledgments

We thank Enago for the English-language review.

Author contributions

Drafting/revising the manuscript for content: Y Fukami, H Koike, and M Katsuno. Study concept and design: Y Fukami, H Koike, and M Katsuno.

Declaration of interest

H Koike reports support from the Health and Labour Sciences Research Grant on Intractable Diseases (Neuroimmunological Diseases) from the Ministry of Health, Labour and Welfare of Japan (20FC1030), and from JSPS KAKENHI (20K07882). M Katsuno reports support from JSPS KAKENHI (JP20H00527), grants from the Japan Agency for Medical Research and Development (JP19ek0109221, JP19ek0109359, JP19dk0207027, JP19lk0201101 and JP19dm0107155), and a grant from the Naito Foundation. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This work was supported in part by the Health and Labour Sciences Research Grant on Intractable Diseases (Neuroimmunological Diseases) from the Ministry of Health, Labour and Welfare of Japan (20FC1030) and JSPS KAKENHI (20K07882).