Why carry out this study?

Primary Sjögren’s is a multi-system autoimmune disease that affects the salivary and lachrymal glands.

The global burden of primary Sjögren’s is poorly understood, which is partly due to a lack of patient and healthcare professional awareness of the disease and limited available epidemiological evidence.

The aim of this research was to conduct a systematic literature review (SLR) to identify and synthesize the most recent evidence on global incidence, prevalence, age of onset, and age of diagnosis of primary Sjögren’s.

What was learned from this study?

Sixty-eight relevant publications, corresponding to 62 unique studies, were identified. The SLR found that incidence and prevalence of primary Sjögren’s was highest in females and in older age groups (≥65 years), while the average age at onset and diagnosis of primary Sjögren’s ranged between 34–57 years and 40–67 years, respectively.

The SLR revealed that there were a limited number of studies reporting on the incidence and prevalence of primary Sjögren’s and estimates varied widely between studies, highlighting a key weakness in the epidemiological evidence base.

To address the weaknesses in the epidemiological evidence base, the defined classification criteria for primary Sjögren’s should be adopted consistently and studies should adhere to available reporting guidelines for incidence and prevalence data. This would improve the comparability of epidemiological data between studies, settings, and countries and lead to a better understanding of the true burden of primary Sjögren’s.