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in children as part of the spectrum of juvenile rheumatoid arthritis. In concordance
with most of the infl ammatory arthritic conditions, SS has a strong female propensity
with a more than ten times female-to-male preponderance [ 3 ]. The prevalence of pSS
was reported around 0.4 % among Caucasian women from Birmingham, UK [ 4 ]. An
annual incidence of SS of approximately 4 per 100,000 people was found in a popu-
lation-based study from Olmsted County, Minnesota [ 5 ]. Of the incident cases, 70 %
were diagnosed as primary SS, and the remainder were associated with underlying
illnesses [ 5 ]. Among other rheumatic disorders, RA was depicted to be the highest in
its association with sSS [ 3 ].
Clinical Manifestations
SS characteristically manifests with dry eyes and dry mouth (sicca symptoms); how-
ever, systemic and extraglandular affection is not unco mmon [ 1 , 6 ]. Keratoconjunctivitis
sicca (KCS) is the term that is occasionally used to describe corneal and conjunctival
affection in dry eyes. The ocular symptoms include irritation, grittiness, and a foreign
body sensation. In advanced cases, infl ammation becomes progressive and visual dis-
turbances ensue [ 1 ]. Oropharyngeal manifestations due to salivary hyposecretion
include dry mouth, dysphagia, dental caries, and oral candidiasis, as well as salivary
glands enlargement, which may occur through the disease course. The parotid gland
enlargement that accompanies dry mouth is sometimes referred to as Mikulicz syn-
drome [ 1 , 2 ]. Both dry eyes and dry mouth were shown to have a signifi cant negative
impact on the patient’s quality of life (QOL) [ 7 ].
Patients with SS may additionally develop general systemic symptoms such as
fatigue, malaise, or fever. Fatigue is one of the most common and disabling com-
plaints in SS. The pathogenesis of fatigue is poorly understood; however, approxi-
mately 70 % of pSS patients suffer from a disabling fatigue [ 8 ]. Because of its high
prevalence, fatigue was identifi ed as one of the main causes of reduced health-
related quality of life (HRQOL) in SS patients [ 8 ].
Though sicca symptoms represent the main disease manifestation, it can be said
that almost every body organ or system is vulnerable. SS patients are prone to a
diverse array of extraglandular manifestations, including the joints (arthralgia or
arthritis), skin (e.g., vasculitis or skin rashes), muscles (myalgia or infl ammatory
myositis), lungs (e.g., interstitial lung disease), heart (e.g., pericarditis), liver (e.g.,
primary biliary cirrhosis), kidneys (interstitial nephritis), and nervous system [ 1 ].
The prevalence of nervous system involvement may exceed 20 % in SS patients [ 9 ].
There is a myriad of nervous system affection patterns including peripheral neu-
ropathy, focal neurologic defi cits due to central nervous system lesions, trigeminal
neuralgia, seizures, dementia, aseptic meningitis, optic neuritis, and transverse
myelitis [ 9 ].
M.O. Hegazi et al.