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Anti-RuvBL1/2 autoantibodies in patients with systemic sclerosis or idiopathic inflammatory myopathy and a nuclear speckled pattern
  1. Jean-Baptiste Vulsteke1,2,
  2. Yves Piette3,4,
  3. Carolien Bonroy5,6,
  4. Patrick Verschueren1,2,
  5. Daniel Blockmans7,8,
  6. Steven Vanderschueren7,8,
  7. Kristl G Claeys9,10,
  8. Petra De Haes11,12,
  9. Jan Leo Lenaerts2,
  10. Wim A Wuyts13,14,
  11. Takashi Matsushita15,
  12. Vanessa Smith4,16,
  13. Ellen De Langhe1,2,
  14. Xavier Bossuyt17,18
  1. 1 Development and Regeneration, Skeletal Biology Engineering and Research Center, KU Leuven, Leuven, Belgium
  2. 2 Rheumatology, KU Leuven University Hospitals Leuven, Leuven, Belgium
  3. 3 Internal Medicine, Ghent University, Ghent, Belgium
  4. 4 Rheumatology, Ghent University Hospital, Ghent, Belgium
  5. 5 Diagnostic Sciences, Ghent University, Ghent, Belgium
  6. 6 Laboratory Medicine, Ghent University Hospital, Ghent, Belgium
  7. 7 Microbiology, Immunology and Transplantation, Laboratory for Clinical Infectious and Inflammatory Disorders, KU Leuven, Leuven, Belgium
  8. 8 General Internal Medicine, KU Leuven University Hospitals Leuven, Leuven, Belgium
  9. 9 Neurosciences, Laboratory for Muscle Diseases and Neuropathies, KU Leuven, Leuven, Belgium
  10. 10 Neurology, KU Leuven University Hospitals Leuven, Leuven, Belgium
  11. 11 Microbiology, Immunology and Transplantation, KU Leuven, Leuven, Belgium
  12. 12 Dermatology, KU Leuven University Hospitals Leuven, Leuven, Belgium
  13. 13 Chronic Diseases and Metabolism, Laboratory of Respiratory Diseases and Thoracic Surgery, KU Leuven, Leuven, Belgium
  14. 14 Respiratory Diseases, KU Leuven University Hospitals Leuven, Leuven, Belgium
  15. 15 Dermatology, Kanazawa University, Kanazawa, Japan
  16. 16 Internal Medicine; Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Ghent University, Ghent, Belgium
  17. 17 Microbiology, Immunology and Transplantation, Clinical and Diagnostic Immunology, KU Leuven, Leuven, Belgium
  18. 18 Laboratory Medicine, KU Leuven University Hospitals Leuven, Leuven, Belgium
  1. Correspondence to Dr Xavier Bossuyt, Microbiology, Immunology and Transplantation, Clinical and Diagnostic Immunology, KU Leuven, Leuven, Flanders, Belgium; xavier.bossuyt{at}

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The International Consensus on ANA Patterns (ICAP) initiative recently described the clinical relevance and associated autoantibodies of HEp-2 indirect immunofluorescence (HEp-2 IIF) patterns.1 We here contend that autoantibodies to RuvBL1/2 are associated with a nuclear speckled HEp-2 IIF pattern, potentially conjointly with a cytoplasmic pattern, an association not included in ICAP so far.

Anti-RuvBL1/2 autoantibodies have recently been described in patients with systemic sclerosis (SSc) and SSc-myositis overlap syndrome, primarily in patients without other known autoantibodies.2–6 Thirteen of 15 anti-RuvBL1/2-positive patients (87%) with description of HEp-2 IIF results in literature had a nuclear speckled pattern. In this study, we evaluated the presence of anti-RuvBL1/2 autoantibodies in patients with SSc or idiopathic inflammatory myopathy (IIM) with a nuclear speckled pattern on HEp-2 IIF without other known associated autoantibodies from two tertiary referral centres in Belgium, the University Hospitals Leuven and Ghent University Hospital.

We performed immunoprecipitation (IP) of HeLa nuclear extract with human sera, followed by western blotting with rabbit polyclonal anti-RuvBL1 and anti-RuvBL2 antibodies in 51 patients classifiable as SSc according to the 2013 European Alliance of Associations for Rheumatology/American College of Rheumatology (EULAR/ACR) classification criteria or early SSc according to the Leroy criteria (n=41), IIM according to the 2017 EULAR/ACR classification criteria (n=8), or both (n=2).7–9 All patients had a nuclear fine or coarse speckled pattern (ICAP AC-4 or AC-5) with …

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  • Handling editor Josef S Smolen

  • Twitter @JBVulsteke

  • EDL and XB contributed equally.

  • Contributors J-BV, EDL and XB designed the study, analysed the data and drafted the manuscript. J-BV performed the immunoprecipitation-western blot experiments. YP, CB, PV, DB, SV, KC, PDH, JLL, WAW, VS and EDL collected patient data and revised the draft manuscript. TM provided reference material and revised the draft manuscript.

  • Funding JB Vulsteke holds a Research Foundation—Flanders (FWO) SB Fellowship (1S62419N).

  • Competing interests WAW has received research grants from Roche, Boehringer-Ingelheim and Galapagos. VS has received research grants and support from Boehringer-Ingelheim and Janssen-Cilag, and research support from Accord Healthcare, UCB, and Celgene. XB was part of a scientific advisory committee for Inova Diagnostics and Thermo Fisher (outside the scope from the submitted work).

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

  • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.

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