[Current treatment, foreseeable newcomer and new treatment approaches for Sjögren's disease]

Although Sjögren's disease (SjD) is the most frequent connective tissue disease, there are still no approved medications for systemic treatment. Many disease-modifying antirheumatic drugs (DMARD) and biologicals have not shown sufficient efficacy in studies. The reasons for this are the complex immunological mechanisms and a high degree of clinical and immunological heterogeneity of the patients; however, significant progress has been made in recent years: for the first time 2 phase 3 studies (ianalumab and telitacicept) reported positive results. The current treatment recommendations are based on the European Alliance of Associations for Rheumatism (EULAR) guidelines (2020) and the recommendations of the British Society for Rheumatology (2024). The treatment is currently primarily based on the leading symptoms and includes consistent treatment of dry eye and dry mouth symptoms (eye and mouth care, pilocarpine if necessary). Extraglandular manifestations continue to be treated predominantly off-label with conventional DMARDs; in severe courses rituximab is used and less frequently cyclophosphamide or intravenous immunoglobulins (IVIG). New treatment approaches show consistent effects on reduction of disease activity (EULAR Sjogren's syndrome disease activity index, ESSDAI) for the first time: In particular, B‑cell-targeted therapies (ianalumab, telitacicept), FcRn inhibitors (nipocalimab, efgartigimod), CD40/CD40L blockade, kinase inhibitors and other immunomodulatory strategies have achieved significant improvements in ESSDAI and, in some cases EULAR Sjögren's syndrome patient reported index (ESSPRI) with an acceptable safety profile. Although basic measures remain indispensable, new forms of targeted treatment mark a turning point in the treatment of SjD. In particular, B‑cell and FcRn-targeted approaches open up for the first time the prospect of evidence-based systemic treatment that goes beyond mere symptom control.