TY - JOUR
T1 - Phases and Natural History of Sjögren's Disease
T2 - a new model for an old disease?
AU - Lee, Adrian Y.S.
AU - Wang, Jing Jing
AU - Gordon, Tom P.
AU - Reed, Joanne H.
PY - 2023/7
Y1 - 2023/7
N2 - Sjögren's disease (SjD) is an archetypal and heterogenous autoimmune disorder that is characterized by exocrine glandular dysfunction. A proportion of patients develop severe extraglandular manifestations, such as cryoglobulinemia, and have an increased risk of lymphoma, both of which can adversely affect quality of life and occasionally mortality. As with most autoimmune disorders, the pathogenesis is poorly understood and difficult to predict, and, frustratingly, there is a lack of targeted therapies to cure this disease. We review the disease manifestations of SjD and propose a staged model for understanding the evolution of pathology. In longitudinal studies, most patients remain relatively stable in terms of their laboratory and clinical parameters. However, in the setting of various risk factors, a proportion of patients develop severe symptoms and/or lymphoma. We discuss potential underlying mechanisms for disease progression and the strengths and limitations of using a staged model to correlate the pathogenesis and spectrum of manifestations in SjD. Ultimately, understanding how and why some patients remain relatively stable, whereas others progress and develop florid systemic disease and a fraction develop lymphoma, is key to developing preventative and therapeutic treatments.
AB - Sjögren's disease (SjD) is an archetypal and heterogenous autoimmune disorder that is characterized by exocrine glandular dysfunction. A proportion of patients develop severe extraglandular manifestations, such as cryoglobulinemia, and have an increased risk of lymphoma, both of which can adversely affect quality of life and occasionally mortality. As with most autoimmune disorders, the pathogenesis is poorly understood and difficult to predict, and, frustratingly, there is a lack of targeted therapies to cure this disease. We review the disease manifestations of SjD and propose a staged model for understanding the evolution of pathology. In longitudinal studies, most patients remain relatively stable in terms of their laboratory and clinical parameters. However, in the setting of various risk factors, a proportion of patients develop severe symptoms and/or lymphoma. We discuss potential underlying mechanisms for disease progression and the strengths and limitations of using a staged model to correlate the pathogenesis and spectrum of manifestations in SjD. Ultimately, understanding how and why some patients remain relatively stable, whereas others progress and develop florid systemic disease and a fraction develop lymphoma, is key to developing preventative and therapeutic treatments.
KW - Sjogren's syndrome
KW - exocrine glandular dysfunction
KW - disease manifestations
KW - systemic autoimmune diseases
KW - SjD
UR - http://www.scopus.com/inward/record.url?scp=85143239133&partnerID=8YFLogxK
UR - http://purl.org/au-research/grants/NHMRC/1142186
UR - http://purl.org/au-research/grants/NHMRC/1183619
U2 - 10.1002/acr.25011
DO - 10.1002/acr.25011
M3 - Review article
C2 - 36063396
AN - SCOPUS:85143239133
SN - 2151-464X
VL - 75
SP - 1580
EP - 1587
JO - Arthritis Care and Research
JF - Arthritis Care and Research
IS - 7
ER -