lactoferrin, beta-2-microglobulin, immunoglobulin A, lysozyme C, and cystatin C, and decreased salivary amylase and carbonic anhydrase [22, 23], but these findings are not specific enough for diagnostic purposes. Antibodies against exocrine gland cholinoreceptors correlate with dry mouth in persons with primary syndromes.

Heat shock proteins, mucins, carbonic anhydrases, enolase, vimentin, and cyclophilin B are among the proteins identified in minor salivary glands. The differences in the proteomes of minor salivary glands from primary SS patients and non-SS controls are mainly related to ribosomal proteins, immunity and stress. Alpha-defensin-1 and calmodulin are among six proteins exclusively identified in primary SS patients [24].

Fibronectin peptides in saliva from SS have recently been suggested as a method of monitoring repetition disease activity in primary SS [25].

6.7Hyposalivation: Clinical Features and Complications

6.7.1Clinical Features

Oral complaints (often the presenting feature) can include:

•Xerostomia: often the most frequent and obvious clinical component, although not all patients complain of dry mouth

•Soreness or burning sensation.

•Difficulty eating dry foods (the “cracker sign”).

•Difficulties in controlling dentures.

•Difficulties in speech: Difficulty speaking for long periods of time or the development of hoarseness there may be a clicking quality of the speech as the tongue tends to stick to the palate.

•Difficulties in swallowing.

•Complications such as unpleasant taste or loss of sense of taste; oral malodour; caries; candidiasis; sialadenitis.

•Putting a glass of water on the bedside table to drink at night (and sometimes, resulting nocturia).

•Salivary gland enlargement (see below).

92 C. Scully and E.A. Georgakopoulou

A positive response to the following questions is significantly associated with reduced salivary output [26]:

6.7.2Examination

The patient should be examined by

•Inspection

•For facial symmetry

•For evidence of enlarged glands

•Of the salivary ducts for pus

•Of the salivary pool under the tongue

•Of the mucosa for erythema, mucositis, angular cheilitis, dryness, or lingual depapillation

•Palpation of the salivary glands

•Parotids

•Submandibular glands

By bimanual palpation of the glands with fingers both inside and outside the mouth.

6.7.3Clinical Signs of Hyposalivation

Reduced salivation may result in a dry mucosa that becomes sticky. The lips may adhere to each other. There may be lack of salivary pooling in the floor of the mouth and saliva flows poorly, if at all, from the ducts of the major glands upon stimulation or palpation.

Physical findings may include a tendency of the mucosa to stick to a dental mirror or tongue spatula; food residues within the oral cavity; frothiness of saliva, particularly in the lower sulcular reflection; and the absence of frank salivation from major gland duct orifices (Fig. 6.1, 6.2). The tongue may develop a characteristic appearance: a lobulated, red surface with partial or complete depapillation. Salivary gland enlargement, particularly of the parotid glands, is also characteristic of SS (see below). However, not all patients with SS have salivary gland enlargement. Isolated enlargement of the submandibular glands is not typical of SS, but rather is more suggestive of IgG4-related systemic disease.

Fig. 6.1 Dry mouth; food residues

Fig. 6.2 Lobulated tongue

6.7.4Effect of Hyposalivation on Quality of Life

SS has a large impact on health-related quality of life (HR-QOL), employment, and disability of patients [27]. Furthermore, in SS patients, both the oral health-related and generic quality of life (QOL) are poor. The salivary flow rate correlates with both Disease Damage Index and Oral Health Impact Profile (OHIP-14) ratings. The number of autoimmune symptoms correlates with both oral and generic QOL; and oral health accounts for a significant percentage of variance in SF-36 domains of general health and social function [28].

Among patients with rheumatic disorders, xerostomia is common and the prevalence increases with age [29]. Women are more susceptible to rheumatic diseases than men and more likely to be affected by xerostomia and xerophthalmia. There is a significant correlation between xerostomia and xerophthalmia, but xerostomia