LAUSR

EJD, vol. 29, n◦ 1, January-February 2019 developed a reddish oval patch on her back (figure 1F). Skin biopsy showed thickened and hyalinized collagen, suggestive of localized scleroderma (morphea) (figure 1G, H). At this point, we searched for scleroderma-related autoantibodies. Interestingly, both anti-topoisomerase I Ab (ATA; x16) and anti-RNA polymerase III Ab (RNAPIII; 52), which are associated with diffuse cutaneous systemic sclerosis (dcSSc), were detected. Anti-centromere antibody (ACA), which is associated with limited cutaneous systemic sclerosis (lcSSc), was not detected. No sclerosis was apparent at this time, except for morphea on the back. One year later, while under PSL at 9 mg/day, the patient developed sclerosis in her fingers and forearms (figure 1I). Skin biopsy showed thickened and hyalinized collagen, suggestive of sclerosis (figure 1J, K). Collectively, the patient developed SLE, SS, RA, localized scleroderma, and dcSSc, therefore, she was diagnosed with overlap syndrome. Neither renal dysfunction (nephritis or renal crisis) nor interstitial lung disease has so far developed. In principle, autoantibodies in SSc are mutually exclusive except for anti-U1-RNP Ab in one case [1]. However, the coexistence of ACA and ATA is not very rare. Two studies have addressed the coexistence of ACA and ATA in SSc based on published reports and found coexistence in 0.52% (28/5,423) [2] and 0.6% (29/4,687) [3] of SSc patients, respectively. By contrast, the coexistence of two dcSSc-associated autoantibodies (ATA and RNAPIII) is very rare [4]. To the best of our knowledge, the coexistence of ATA and RNAPIII has been only reported in one patient [5]. In this regard, our case is the second to demonstrate the coexistence of two dcSSc-associated autoantibodies. In addition to the presence of ATA and RNAPIII, several other autoantibodies (such as anti-ssDNA Ab, anti-Sm Ab, anti-SS-A Ab, anti-SS-B Ab, and anti-CCP Ab) were also detected in our patient. This suggests that the production of autoantibodies is abnormally enhanced, possibly reflecting the aberrant B and plasma cell activities. In this regard, we speculate that our case demonstrated overlap between ATA-positive dcSSc and RNAPIII-positive dcSSc, along with other systemic autoimmune rheumatic diseases.