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This review highlights the 30 plus years research progress since the discovery of autoantibody to Ro52/TRIM21 in patients with systemic lupus erythematosus (SLE) and Sjögren's syndrome (SjS). After the initial expression cloning of the Ro52 cDNA, it has taken many years to the current understanding in the interesting biological function of Ro52 as an E3 ubiquitin ligase and its role in innate immune clearance of intracellular IgG-bound complex. Early observations show that anti-Ro52, mostly associated with anti-SS-A/Ro60 and/or anti-SS-B/La, is commonly found in SLE (40-70%), SjS (70-90%), neonatal lupus erythematosus (NLE, 75-90%), and subacute cutaneous lupus erythematosus (50-60%). Anti-Ro52 has long been postulated to play a direct pathogenic role in congenital heart block in NLE as well as in the QT interval prolongation in some adults. The widespread availability of the anti-Ro52 assay has led to the detection of anti-Ro52 in other diseases including autoimmune hepatitis (20-40%), systemic sclerosis (10-30%), and autoimmune myositis (20-40%). More than ten studies have pointed to an association of anti-Ro52 with interstitial lung disease and, more importantly, correlating with poor outcome and worse survival. Other studies are implicating an interesting role for anti-Ro52 in the diagnosis of certain cancers. Future studies are needed to examine the mechanism in the pathogenesis of anti-Ro52 and carefully documenting its causal relationships in different disease conditions., (© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Results of the anti-nuclear antibodies-indirect immunofluorescence assay (anti-cell antibodies test) on HEp-2 cell substrates should be communicated to clinicians in a standardized way, adding value to laboratory findings and helping with critical clinical decisions. This paper proposes a test report based on the practices informed by 118 laboratories in 68 countries, with recommendations from the International Consensus on ANA Patterns (ICAP) group. Major focus is placed on the report format containing endpoint titers, immunofluorescence patterns together with anti-cell (AC) nomenclature, remarks on follow-up or reflex testing, and possible other autoantibody associations. ISO 15,189 directives were integrated into the test report. Special situations addressed include serum screening dilutions and endpoint titers, relevance of immunofluorescence patterns with special attention to cytoplasmic patterns, mixed and compound patterns, and how to report different titers corresponding to multiple patterns or autoantibodies in the same sample. This paper suggests a subtitle for the HEp-2-IIFA, namely anti-cell antibodies test, which could gradually substitute the original outdated ANA nomenclature. This ICAP pro forma report represents a further step in harmonizing the way relevant clinical information could be provided by laboratories., (© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)