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(anti-TNF)—infl iximab and etanercept—also did not show signifi cant improve-
ments. Of the biologic DMARDS, the anti-B lymphocyte rituximab was the only
remedy that showed promising results with improvements in both xerostomia/
xerophthalmia and extraglandular features as well [ 8 , 13 ].
Monitoring Response to Treatment
It is not enough to measure disease progression and response to treatment based on
symptoms, objective physical fi ndings, and laboratory criteria for each individual
patient without a standardized scale. Additionally, the patients’ self-reported
responses are increasingly gaining signifi cance and preference in the face of objec-
tive clinical and laboratory fi ndings. The need for a unifi ed SS disease activity index
has been growing over the last few decades. In the year 2010, the EULAR Sjögren’s
Syndrome Disease Activity Index (ESSDAI) was published [ 14 ] ( Appendix 1 ). It
has been developed as a reference index to assess disease activity and response to
therapy for both clinical practice and research purposes [ 14 ]. Giving more weight to
the patient responses, the EULAR Sjögren’s Syndrome Patient-Reported Index
(ESSPRI) was generated in the year 2011 [ 15 ] ( Appendix 2 ). ESSPRI is a very
simple index designed to measure patients’ symptoms in pSS [ 15 ]. It is validated for
use as an outcome measure in clinical trials [ 15 ]. More details about the aforemen-
tioned indices will follow in this chapter (see “Disease Activity”).
Prognosis and Impact on Quality of Life
Earlier studies revealed that dry eyes and dry mouth with or without the general
systemic symptoms, such as fatigue, have a signifi cant negative impact on p atients’
QOL [ 10 , 16 ]. The development of extraglandular organ or system involvement also
increases morbidity and further deteriorates the QOL. More details about QOL
measures in SS will follow in this chapter (see section “Health-Related Quality of
Life Measures in Sjögren’s Syndrome”). In addition to the burden imposed by sicca
symptoms and fatigue, SS patients could also be dreadfully at increased risk of both
mortality and malignancy [ 6 ]. Some of the systemic organ-specifi c complications
(e.g., glomerulonephritis or interstitial lung disease) of SS may approach a life-
threatening severity [ 13 ]. In a study carried out in Spain, patients with pSS, who
present at diagnosis with high systemic activity (ESSDAI ≥ 14) were shown to be at
higher risk of death [ 6 , 16 ]. Patients with SS and with severe involvement of the
exocrine glands, vasculitis, and cryoglobulinemia were found to be at increased risk
for developing non-Hodgkin’s lymphoma [ 6 ]. In a series of 380 Spanish patients
with pSS, 3 % had developed lymphoma during 9 years of observation [ 17 ]. It is
prudent to identify SS patients with predictors for the development of lymphoma,
who may need more vigilant screening and follow-up schedules.
M.O. Hegazi et al.