Sjögren’s syndrome is a chronic, autoimmune disease identified by lymphocytic infiltration of exocrine glands and epithelia, which leads to sicca symptoms of dry eyes and dry mouth, and objective evidence of keratoconjunctivitis sicca and xerostomia. Sjogren’s syndrome can be present as primary Sjogren’s syndrome, as itself, or as secondary Sogren’s syndrome, in association with underlying immune conditions. Clinical presentation can vary considerably from relatively mild sicca symptoms, fatigue and arthralgia to severe systemic symptoms such as glomerulonephritis, vasculitis and a host of neurological manifestations.
Primary Sjögren’s syndrome is the most common syndrome that varies within the same geographical area and has a strong female propensity. Studies show that the female to male ratio in primary Sjögren’s syndrome varies from as high as 20:1 to 9:1. Caucasian are more prone to the primary Sjögren’s syndrome with mean age of around 40 years. The annual incidence of primary Sjogren’s syndrome was 3.9 per 100,000 persons in year 2002 with higher prevalence in women as compared to men.
As studied by Fox and colleagues, Sjögren’s syndrome mostly occurs due to environmental factors, either viral or non-viral. Sjögren’s syndrome can also occur due to production of cytokine that regulates chemokines with subsequent homing of autoimmune lymphocytes and dendritic cells into the glands via high endothelial venules that express increased levels of cell adhesive molecules. The upregulation of major histocompatibility antigens and adhesive molecules on epithelial cells in the lacrimal and salivary glands leads to the activation of autoantibodies. The secretion of injured lacrimal and salivary gland decrease and resistance of T-cells within glands undergo apoptosis. The infiltrated T cells, B cells and dendritic cells cause destruction of glandular elements by cell mediated mechanisms.
Treatment algorithms are often complicated, with the present treatments for Sjögren’s syndrome including, but not limited to, anti- B-cell-activating factor (BAF), B lymphocyte stimulation (BLyS) inhibitors, bruton’s tyrosine kinase (BTK) inhibitors, endogenous B7 molecules inhibitor and cathepsin S antagonist.
SJÖGREN'S SYNDROME PIPELINE SPLIT BY ROUTE OF ADMINISTRATION (2017)
As of March 2017, the Sjögren’s syndrome pipeline comprises of approximately 42 drug candidates in different stages of development.
Some of the key players developing drugs for Sjögren’s syndrome include Kissei Pharmaceutical Co., Ltd., UCB Group, Bristol-Myers Squibb Company and others.
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