**3. Classification criteria**

To standardize the diagnosis in patients taking part in clinical studies, to analyze results, and to facilitate the comparison of patients between centers, the classification criteria should be established.

**37**

[1, 2, 6, 22, 25].

*Sjögren's Syndrome as an Ocular Problem: Signs and Symptoms, Diagnosis, Treatment*

surface (≥4 van Bijsterveld or Ocular Staining Score OSS ≥ 5, [19–23]).

Because of their restrictive nature, the American College of Rheumatology approved the Sjögren's International Collaborative Clinical Alliance (SICCA) approach in 2012. To diagnose SS with ACR/SICCA criteria at least 2 of 3 are necessary: (1) a positive serum anti-Ro/-SSA or/and anti-La/SSB or [positive rheumatoid factor RF and antinuclear antibody (ANA) ≥ 1:320], (2) focal lymphocytic sialade-

Main differences between ACR/SICCA and AECG criteria are: (1) no subjective ocular and buccal symptoms and morphological or functional tests for salivary glands, (2) new OSS proposed by SICCA as the only test for ocular problems, (3) the association of RF positivity and ANA titer 1:320 as equivalent to anti-SSA/-SSB positivity [6, 21]. In 2016, ACR and EULAR published new pSS criteria containing again the Schirmer test and OSS in the dry eye assessment, as well as the test of unstimulated saliva production as a measuring tool for dryness in the mouth. The assessment of minor salivary gland biopsies and confirmation of the presence of the SS-A/Ro antibody were of particular importance. The remaining autoantibodies were not

The main ocular manifestation of SS is DED. It is mainly a result of impaired lacrimal gland function due to inflammatory tissue damage. Hyposecretion of tears leads to aqueous-deficient DED. Moreover, exocrine glands of conjunctiva are also affected in SS and in connection with goblet cells reduction reveal evaporative DED [1, 6, 9, 22]. Ocular surface inflammation with conjunctival hyperemia and corneal epithelial damage leads to functional vision impairment [1, 2, 6, 22, 24]. Squamous metaplasia of the ocular surface epithelium, follicular conjunctivitis, corneal epitheliopathy, filamentary keratitis, sterile infiltrates, scleritis, poor epithelial integrity, recurrent erosions, dysesthesia, corneal ulceration, vascularization, opacification, and sometimes perforation were also described in SS patients

Dry eye disease among other diseases is the symptom causing the greatest morbidity and impairment of quality of life. Therefore, the patient is actively seeking a medical help. DED is typically diagnosed by evaluation of both eye symptoms and results of eye examination that symptoms not always match signs. Some patients have no symptoms though they have changes of ocular surface and others have significant eye discomfort, but there are very little signs in eye examination [1, 2, 22, 26–28].

in labial salivary gland biopsy samples,

In 1993, the Preliminary European Classification criteria for SS were published, and for almost 10 years have been the base for clinical, observational, and interventional studies [6, 19]. In 2002, the American-European Consensus Group (AECG) published a revised version of the SS classification criteria [6, 20–22]. All documents include ocular disturbances. AECG criteria for DED consist of symptoms and signs. Positive response to one question: (1) Have you had daily, persistent, troublesome dry eyes for >3 months? (2) Do you have a recurrent sensation of sand or gravel in the eyes? (3) Do you use tear substitutes >3 times a day? or positive one of tests: Schirmer without anesthesia ≤ 5 mm/5 min or vital dye staining of the ocular

*DOI: http://dx.doi.org/10.5772/intechopen.83821*

nitis defined as focus score ≥ 1 focus/4 mm2

considered to be peculiar enough to diagnose pSS [21].

**4. Ocular manifestation of SS**

**5. Diagnostic procedures for DED**

(3) OSS ≥ 3 [6, 21, 23].

*Sjögren's Syndrome as an Ocular Problem: Signs and Symptoms, Diagnosis, Treatment DOI: http://dx.doi.org/10.5772/intechopen.83821*

In 1993, the Preliminary European Classification criteria for SS were published, and for almost 10 years have been the base for clinical, observational, and interventional studies [6, 19]. In 2002, the American-European Consensus Group (AECG) published a revised version of the SS classification criteria [6, 20–22]. All documents include ocular disturbances. AECG criteria for DED consist of symptoms and signs. Positive response to one question: (1) Have you had daily, persistent, troublesome dry eyes for >3 months? (2) Do you have a recurrent sensation of sand or gravel in the eyes? (3) Do you use tear substitutes >3 times a day? or positive one of tests: Schirmer without anesthesia ≤ 5 mm/5 min or vital dye staining of the ocular surface (≥4 van Bijsterveld or Ocular Staining Score OSS ≥ 5, [19–23]).

Because of their restrictive nature, the American College of Rheumatology approved the Sjögren's International Collaborative Clinical Alliance (SICCA) approach in 2012. To diagnose SS with ACR/SICCA criteria at least 2 of 3 are necessary: (1) a positive serum anti-Ro/-SSA or/and anti-La/SSB or [positive rheumatoid factor RF and antinuclear antibody (ANA) ≥ 1:320], (2) focal lymphocytic sialadenitis defined as focus score ≥ 1 focus/4 mm2 in labial salivary gland biopsy samples, (3) OSS ≥ 3 [6, 21, 23].

Main differences between ACR/SICCA and AECG criteria are: (1) no subjective ocular and buccal symptoms and morphological or functional tests for salivary glands, (2) new OSS proposed by SICCA as the only test for ocular problems, (3) the association of RF positivity and ANA titer 1:320 as equivalent to anti-SSA/-SSB positivity [6, 21].

In 2016, ACR and EULAR published new pSS criteria containing again the Schirmer test and OSS in the dry eye assessment, as well as the test of unstimulated saliva production as a measuring tool for dryness in the mouth. The assessment of minor salivary gland biopsies and confirmation of the presence of the SS-A/Ro antibody were of particular importance. The remaining autoantibodies were not considered to be peculiar enough to diagnose pSS [21].
