Clinical aspects of primary and secondary Sjögren’s syndrome

Summary Sjögren's syndrome (SS) is a rheumatic autoimmune disease of salivary and lacrimal gland. The disease has a heterogenous character, therefore multiple organ manifestations can occur next to dryness, fatigue and pain. The most common manifestations of the disease are dryness of the mouth and eyes, the sicca symptoms. In our study, we investigated the occurrence of sicca symptoms and secondary SS in patients with rheumatoid arthritis (RA). Patients with higher disease activity of RA had more often sicca complaints and secondary SS. We showed that the presence of and changes in organ involvement of SS can be studied with ESSDAI, a systemic disease activity score. Furthermore, joint involvement can be assessed in more detail by DAS-28 score. In this thesis, the diagnosis and treatment of SS in elderly patients was discussed. It is known that 5-10% of SS patients develop lymphomas. We found that serum levels of free light chains can be suitable as biomarker for systemic disease activity and MALT lymphoma in SS patients. Finally, an overview was given of different treatments of sicca complaints and extraglandular disease manifestations. Most patients experience fatigue as most important symptom. Treatment with rituximab resulted in significant improvement of physical fatigue, but the majority of patients still experienced a substantial level of fatigue. The findings from this thesis give an overview of clinical manifestations, biomarkers and effect of the treatment and can tribute to monitoring and treatment of SS patients in daily clinical practice

Sjogren's Syndrome in Older Patients Aetiology, Diagnosis and Management

Sjogren's syndrome (SS) is a systemic autoimmune disease, characterized by chronic inflammation of exocrine glands that results in development of xerostomia and keratoconjunctivitis sicca. The disease activity of SS is not restricted to exocrine glands, and many other organs and organ systems can be involved. Diagnosis of SS in the elderly population can be challenging because xerostomia, dry eyes, symptoms of fatigue, weight loss and muscle pain are also common features of old age. Delay between clinical onset and diagnosis of SS in the elderly may be due to the shared features of SS and old age. The 2002 revised American European Consensus Group (AECG) classification criteria for SS are the preferred tool used to confirm diagnosis of SS, but recently alternative criteria have been put forward by the American College of Rheumatology (ACR). The AECG criteria set combines subjective symptoms of dry eyes and dry mouth with objective signs of keratoconjunctivitis sicca, salivary gland dysfunction and histopathological (salivary gland biopsy) and serological (autoantibodies against SSA/Ro and SSB/La antigens) features. Treatment of SS in the elderly does not differ from that in younger patients. The aims of the treatment of SS are to control glandular and extraglandular manifestations, to prevent damage to organ systems and loss of function, and to decrease morbidity and mortality. Treatment of the elderly can be complicated by co-morbidities, an increased rate of adverse events related to therapeutic agents, and polypharmacy. Therefore, careful follow-up of the treatment is required

Physical fatigue characterises patient experience of primary Sjögren's syndrome

OBJECTIVES: Besides ocular and oral dryness, fatigue is a major symptom in patients with primary Sjögren's syndrome (pSS). Our aim was to investigate the importance of fatigue in relation to other symptoms experienced as well as to evaluate the effect of rituximab treatment on fatigue in pSS patients with active disease. METHODS: This analysis was based on data from our open-label rituximab study in 28 pSS patients. Symptoms of dryness, physical fatigue, pain, and mental fatigue were scored on 0-10 scales (according to ESSPRI). Systemic disease activity was assessed with ESSDAI. RESULTS: At baseline, 24 (86%) patients rated physical fatigue as the complaint most eligible for improvement (median importance of 10), followed by pain, dryness, and mental fatigue. After rituximab treatment, physical fatigue showed maximum improvement of 2.5 points and 31% in median values at group level, and 10 (36%) patients reached physical fatigue score<5 representing patient-acceptable symptom state (PASS). In comparison, systemic disease activity improved 5.5 points and 73% at group level, and 22 (79%) patients reached ESSDAI<5 representing low disease activity. GEE analysis over time revealed that physical fatigue was significantly associated with absolute number of B cells, dryness and mental fatigue, but not with ESSDAI, IgG levels and IgM-RF. CONCLUSIONS: Physical fatigue characterises patient experience of pSS. Rituximab treatment resulted in significant improvement of patient-reported symptoms. However, the large majority of patients still experienced physical fatigue at an unsatisfactory level, above the cut-off value for PASS. Therefore, attention for optimal management of this prominent symptom is warrante

Detailed Analysis of the Articular Domain in Patients with Primary Sjogren Syndrome

Objective. We used the 28-joint Disease Activity Score (DAS28) and the European League Against Rheumatism Sjoren's Syndrome Disease Activity Index (ESSDAI) articular domain to assess the effect of rituximab (RTX) and abatacept (ABA) on articular involvement in primary Sjoren syndrome (pSS). Methods. Patients with pSS treated with RTX (n = 18) or ABA ( n = 13) and having a DAS28 erythrocyte sedimentation rate (ESR)/C- reactive protein (CRP) level >= 3.2 at baseline were selected. Generalized estimating equations were used to analyze the DAS28 and ESSDAI articular domain over time. Results. In the RTX group, DAS28-ESR/CRP decreased significantly up to 48 weeks. In the ABA group, DAS28-ESR/CRP decreased significantly up to 24 weeks. DAS28 correlated significantly with ESSDAI articular domain. Conclusion. DAS28 is useful to evaluate the effect of biologicals on articular involvement in patients with pSS