© Springer International Publishing Switzerland 2016 309
Y. El Miedany (ed.), Patient Reported Outcome Measures in Rheumatic Diseases,
DOI 10.1007/978-3-319-32851-5_12
Chapter 12
PROMs for Sjögren’s Syndrome
Mohamed Osama Hegazi , Sally Youssef , and Yasser El Miedany
Introduction
Sjögren’s (pronounced Show-grin’s) syndrome (SS) is a chronic infl ammatory
condition characterized by affection of the exocrine lacrimal and salivary glands
leading to dry eyes and dry mouth [ 1 ]. It is categorized as one of the systemic autoim-
mune disorders for a number of reasons. These include, but are not limited to, the
presence of autoantibodies, the shared clinical manifestations (e.g., arthritis) with other
autoimmune rheumatic diseases, and the association of SS with disorders such as sys-
temic lupus erythematosus (SLE) and rheumatoid arthritis (RA) [ 2 , 3 ]. SS is mainly
classifi ed as primary SS (pSS) if it occurs without another underlying or associated
autoimmune disease, and as secondary SS (sSS) whenever it is associated with an
autoimmune disorder such as RA, SLE, spondyloarthritis, and systemic sclerosis [ 1 ].
SS is a disease of adults with the mean age of onset usually in the fourth to fi fth
decade [ 3 ]. There are two peaks of the disease age of onset. The fi rst peak occurs dur-
ing the childbearing period in the mid 30s and a second peak in postmenopausal years
during the mid 50s, although the condition can occur at virtually any age, including
M. O. Hegazi , M.R.C.P. (U.K.) (*)
Medical Department , Al Adan Hospital , P.O. Box 262 , Hadiya 52853 , Kuwait
e-mail: [email protected]
S. Youssef , M.D.
Rheumatology and Rehabilitation Department , Ain Shams University , Cairo , Egypt
e-mail: [email protected]
Y. El Miedany , M.D., F.R.C.P.
Honorary Senior Clinical Lecturer, King’s College London , London , UK
Professor of Rheumatology and Rehabilitation, Ain Shams University , Cairo , Egypt
Department of Rheumatology , Darent Valley Hospital , Gravesend , Kent , UK
e-mail: [email protected]