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Case report
Proteinase 3 (PR3)-antineutrophil cytoplasmic antibody (ANCA)-associated vasculitic neuropathy in diffuse cutaneous systemic sclerosis: a rare duo
  1. Yasser Radwan1,
  2. Sarah Berini2,
  3. Floranne Ernste1 and
  4. Ashima Makol1
  1. 1 Division of Rheumatology, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA
  2. 2 Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA
  1. Correspondence to Dr Ashima Makol; Makol.Ashima{at}mayo.edu

Abstract

Systemic sclerosis (SSc) is characterised by non-inflammatory vasculopathy, autoimmunity and widespread fibrosis. While the presence of antineutrophil cytoplasmic antibodies (ANCAs) has been reported in SSc, their association with ANCA-associated vasculitis is exceedingly rare. Myeloperoxidase ANCA is more common than proteinase-3 ANCA, and glomerulonephritis is the most common clinical presentation of ANCA-associated vasculitis in SSc. ANCAs have been associated with the adverse disease outcomes in SSc, including higher mortality per recent reports. A 65-year-old man with diffuse cutaneous SSc for 6 years presented with new-onset peripheral neuropathy. Workup revealed a positive proteinase-3 and cytoplasmic ANCA, and histopathology confirmed an inflammatory vasculitic neuropathy. The patient was successfully treated with rituximab. Our case highlights the importance of checking ANCA in SSc at baseline, given the risk of disease-related complications, even years later. Tissue biopsy is often warranted for confirmation of vasculitis and prompt treatment can optimise long-term outcomes.

  • vasculitis
  • connective tissue disease
  • peripheral nerve disease

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Footnotes

  • Contributors YR: designed the study, reviewed the literature, drafted the manuscript, formulated the data tables and the flow diagram, and revised the manuscript for the important intellectual content. SB: interpretation of data and contributed to the case presentation. FE: contributed to the discussion. AM: study concept and design, analysis and interpretation of data, critical revision of the manuscript, study supervision and gave approval for the version published.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.