A Systematic Scoping Literature Review to Identify the Humanistic Burden Attributed to Living With Primary Sjogren’s Disease

OBJECTIVES: Primary Sjogren’s Disease (pSjD) is a chronic autoimmune condition affecting glands, frequently causing poorly tolerated eye and mouth dryness, pain, and fatigue. Up to 50% of patients experience extra-glandular involvement presenting articular, neurological, respiratory, and renal symptoms. We sought to define the humanistic outcomes of pSjD using a scoping systematic literature review (SLR).

METHODS: A systematic search conducted in Medline and EMBASE targeted English language studies reporting humanistic and patient-reported outcomes (PRO) since 2013. The PICOS considered adults with moderate-to-high disease severity. No restrictions were applied to interventions, comparators, or study design. Studies were included if reporting disease-specific or generic health-related quality of life (HRQoL) measures or any PRO.

RESULTS: Overall, 40 publications from 36 primary studies were included. The Short-form-36 (SF-36) was reported in 21 studies, the Hospital Anxiety and Depression (HADS) scale in 9 studies, several visual analogue scales were reported by 15 studies, and 5 studies used the EuroQol-5D (EQ-5D). Across publications, most participants were females (72-100%), aged 40-62 years, having prevalent pSjD for 2 to 54 years. Compared to general population values, SF-36 physical and mental health scores were commonly reduced across study designs, ranges 36-46 in both components (scored 0-100), as were mean EQ-5D total scores (range 59.2-64.9). Mapping of SF-36 to disease-specific instruments indicated that lower pSjD symptoms correlated with higher HRQoL. HADS-anxiety scores ranged from 6.1-8.5 and HADS-depression from 6.7-7.4 (0 lower, to 10 higher burden). FACIT-Fatigue scores were also substantially reduced in pSjD-affected individuals, 21.2 to 33.18 (range 0 higher fatigue to 52, lower). Overall, intervention studies did not show statistically significant improvement in clinical, HRQoL, or PRO.

CONCLUSIONS: Patients living with pSjD experience important physical and mental burden attributed to a range of symptoms. Understanding how pSjD impact HRQoL can be important for evaluating new therapies.