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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.
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