From Sjögren syndrome to Sjögren disease: rethinking classification and diagnostic complexity

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.