| From Sjögren syndrome to Sjögren disease: rethinking classification and diagnostic complexity |
Jennifer Jooha Lee1, Howook Jeon2, Hyun-Sook Kim3, Hae-Rim Kim4, Seung-Ki Kwok1, Yoon-Kyoung Sung5, Jisoo Lee6, Shin-Seok Lee7, Sung-Hwan Park1, Yun Jong Lee8,9,10
, and on behalf of the Korean Society of Sjogren Disease Research (KCR-SjD) |
1Division of Rheumatology, Department of Internal Medicine, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
2Division of Rheumatology, Department of Internal Medicine, Uijeongbu St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
3Division of Rheumatobiology, Department of Internal Medicine, Soonchunhyang University Seoul Hospital, Seoul, Korea
4Division of Rheumatology, Department of Internal Medicine, Research Institute of Medical Science, Konkuk University School of Medicine, Seoul, Korea
5Department of Rheumatology, Hanyang University Hospital for Rheumatic Diseases and Hanyang University Institute for Rheumatology Research, Seoul, Korea
6Division of Rheumatology, Department of Internal Medicine, Ewha Womans University College of Medicine, Seoul, Korea
7Department of Rheumatology, Chonnam National University Hospital, Chonnam National University Medical School, Gwangju, Korea
8Division of Rheumatology, Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam, Korea
9Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea
10Department of Medical Device Development, Seoul National University Graduate School, Seongnam, Korea |
Corresponding Author:
Yun Jong Lee , Tel: +82-31-787-7049, Fax: +82-31-787-4051, Email: yn35@snu.ac.kr
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Received: October 7, 2025; Accepted: March 20, 2026. |
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| Abstract |
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Sjögren syndrome (SS), traditionally regarded as a sicca-predominant condition, is now recognized as a systemic autoimmune disease characterized by type I interferon activation, B-cell hyperactivity, and periepithelial lymphocytic infiltration. This conceptual shift has prompted changes in terminology, with “SS” increasingly replaced by “Sjögren disease (SjD)” and “secondary SS” by “associated SjD,” reflecting the concept of polyautoimmunity and ensuring equal clinical standing. In this review, we trace the historical evolution of SS classification criteria and examine their limitations in real-world clinical practice. Current classification criteria employ a weighted scoring system incorporating objective measures, including anti-Ro antibodies, salivary gland histopathology, and exocrine function tests. However, their diagnostic application in routine care is constrained by spectrum bias, clinical heterogeneity, the presence of mimickers, and shared clinical features with other autoimmune diseases. Over-reliance on serological markers, driven by their accessibility, may further contribute to misdiagnosis. We highlight that accurate diagnosis requires flexible clinical reasoning and contextual interpretation beyond rigid adherence to classification criteria. Individualized diagnostic strategies should integrate demographics, laboratory findings, salivary ultrasonography, and histopathology. Practical challenges in diagnosing and evaluating disease activity in associated SjD are also discussed. |
| Keywords:
Sjögren’s syndrome ; Classification ; Diagnosis ; Diagnostic errors |
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