#2-48E. IIM-SjD Overlap: Recognizing a Distinct Entity and Tailoring Immunosuppression (FIM, 2025)

How should clinicians approach the challenging intersection of Idiopathic Inflammatory Myopathies (IIM) and Sjögren's Disease (SjD), a co-occurrence that complicates diagnosis and treatment? This retrospective study analyzed 23 patients with biopsy-confirmed IIM and coexisting SjD (IIM-SjD), comparing them to IIM controls without SjD, suggesting that IIM-SjD represents a distinct clinical entity characterized by significantly more asymmetric muscle weakness and higher extramuscular involvement. The most critical finding is that while enhanced immunosuppression, including high-efficacy agents like rituximab, led to significantly better outcomes (higher total improvement scores) for patients with associated polymyositis or dermatomyositis, the presence of Inclusion Body Myositis (IBM) was identified as a strong predictor of persistently high systemic disease activity (ESSDAI scores) at follow-up. This paper is crucial for rheumatologists and neurologists, emphasizing the need for systematic SjD screening in IIM patients to guide tailored therapeutic strategies. However, due to the limited sample size and retrospective nature, the findings carry a risk of confounding by indication, meaning treatment escalation may have been influenced by the clinical diagnosis of SjD.

Citation: Konen FF, Güzeloglu YE, Seeliger T, Jendretzky KF, Nay S, Grote-Levi L, Schwenkenbecher P, Gründges C, Ernst D, Witte T and Skripuletz T. Idiopathic inflammatory myopathy associated with Sjögren’s disease: features of a distinct clinical entity. Frontiers in Immunology. 2025. DOI: 10.3389/fimmu.2025.1654576

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