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We also reviewed the literature on SS with AOSD and AOSD with other rheumatic diseases, and scattered case reports were retrieved. So we think that AOSD is not an absolutely exclusive diagnosis and can occur in patients with other rheumatic diseases. To our knowledge, this is the only literature review of a reported AOSD case in a SS patient. We aimed to answer several clinically relevant questions; (1) the interobserver agreement, (2) diagnostic performance of MRI with MRCP for (a) branch duct intraductal papillary mucinous neoplasms (