Sjögren syndrome (SS) is a systemic autoimmune disease characterized by immune-mediated injury of exocrine glands, as well as a diverse array of extraglandular manifestations. SS contains primary SS which usually presents alone while secondary SS occurs in connection with other autoimmune rheumatic diseases. Nowadays, primary SS is estimated to be the second most common systemic autoimmune disease alongside rheumatoid arthritis, which affects about 1.0% of the general population. With major advances having been made in understanding the pathogenesis of SS, B cell over-activation is regarded as a key feature of the disease, while the autoantibodies IgA and IgG level is higher in these patients, which indicates that IgA occupies a position in the pathogenesis of SS. Based on mature and established platforms in therapeutic antibody research, Creative Biolabs has accumulated experience with SS and we are glad to share it with our clients all over the world.
Clinical Presentation of Sjögren's Syndrome
SS has a wide spectrum of clinical features ranging from sicca symptoms to systemic manifestations, while local manifestations arising from salivary and lachrymal gland involvement are considered the typical features. However, SS may affect other organs beyond exocrine glands resulting from immunocomplexes deposition as a result of B cell hyperactivity, hence lymphoma and glomerulonephritis are common complications of SS.
Pathogenesis of Sjögren's Syndrome
SS is considered as a multifactorial process originating from the interaction between genetic factors and exogenous and endogenous agents able to trigger an abnormal autoimmune response mediated by T and B lymphocytes. Lymphocyte B hyperactivity and autoantibodies production lead to IgA deposition around lesions. While environmental triggers such as virus infections create an inflammatory microenvironment, autoantigens are released and exposed to dying cells. Immune complexes formed between autoantibodies and autoantigens augmented type I IFN production while type I IFNs drive autoantibody production through promoting differentiation and activation of the autoreactive B cells. Through this process, a self-perpetuating cycle of autoimmunity is created.
Fig.1 Schematic overview immunopathogenic mechanisms underlying Sjögren's syndrome. (Björk, 2020)
Treatment of Sjögren's Syndrome
Despite the dysimmune origin of the SS, immunosuppressive treatment remains disappointing. Up to now, treatment of SS is directed to provide symptomatic relief and to recognize and treat disease complications in a timely fashion. For the typical symptom of keratoconjunctivitis sicca, eye lubricants are the mainstay of treatment. However, besides genetic situation, factors in the environment also play a pivotal role in SS pathogenesis such as alcohol, smoking, stress, hormones, and so on. For chronic conditions such as SS whose effective therapy is still lacking, precaution is an important measurement to protect us.
As one of the most common systemic autoimmune diseases, SS attracts more attention from therapeutic molecule development groups including Creative Biolabs. For more details of SS or other research in the field of therapeutic protein research, please feel free to contact us.
Reference
- Björk, A.; et al. Environmental factors in the pathogenesis of primary Sjögren's syndrome. Journal of internal medicine. 2020, 287(5), 1069-1076.
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