Clinical Manifestations Of Sjogrens Syndrome

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Most patients with Sjogren's syndrome develop symptoms related to decreased salivary gland and lacrimal gland function. Primary Sjogren's syndrome patients generally complain of dry eyes, often described as a sandy or gritty feeling under the eyelids. Other symptoms such as itching of the eyes, eye fatigue, and increased sensitivity to light can accompany the primary symptoms. Many of these symptoms are due to the destruction of corneal and bulbar conjunctival epithelium and come under the diagnosis of keratoconjunctivitis sicca. This disorder is assessed by tear flow and composition. Tear flow is measured using the Schirmer test, while tear composition can be determined by tear break-up time or tear lysozyme content. The Schirmer test is considered positive when filter paper wetting of less than 5 mm occurs in 5 minutes, and suggests clinically significant keratoconjunctivitis sicca (Moutsopoulos 1993). There are, nonetheless, numerous false positive and negative results, such that the predictive value is limited. The integrity of the corneal and bulbar conjunctiva may be assessed using the Rose Bengal staining procedure and slip lamp examination. Punctate corneal ulcerations and attached filaments of corneal epithelium indicative of corneal and bulbar conjunctival epithelial destruction are noted on slip lamp examination in Sjogren's patients.

Xerostomia is the second principal symptom of Sjogren's syndrome. Xerostomia can be documented by salivary flow measurements, parotid sialography, and salivary scintigraphy. Salivary flow measurements must be adjusted for age, time of day, gender, and concomitant medications. Patients with dry mouths complain of a burning oral discomfort and difficulty in chewing and swallowing dry foods. Xerostomia is commonly associated with changes in taste and the inability to speak continuously for longer than several minutes.

Salivary gland enlargement occurs in as many as 30% of patients with Sjogren's syndrome during the course of their illness, with the parotid gland being most often enlarged (Figure 6.1) (Kulkarni 2005). Bilateral painful submandibular glands have been described as a presenting symptom of this syndrome (Kulkarni 2005). While the parotid glands are most commonly enlarged, they may be the last glands to be affected in patients with Sjogren's syndrome from the standpoint of decreased saliva production (Pijpe, Kalk, and Bootsma et al. 2007). The parotid glands have a longer-lasting secretory capacity in patients with Sjogren's syndrome, and therefore are the last glands to manifest hyposaliva-tion during the disease. In the more advanced stages of the disease, both unstimulated and stimulated submandibular, sublingual, and parotid func-

Figure 6.1. A 36-year-old woman with a known history of Sjogren's syndrome associated with rheumatoid arthritis. She described a recent history of painful swelling of the right parotid gland such that an incisional parotid biopsy was recommended.

Sjogren Swollen Salivary Glands

Lymphoma

Lymphoma in Sjogren's syndrome

Figure 6.1. A 36-year-old woman with a known history of Sjogren's syndrome associated with rheumatoid arthritis. She described a recent history of painful swelling of the right parotid gland such that an incisional parotid biopsy was recommended.

Lymphoma in Sjogren's syndrome

Sjogren Syndrome Salivary Gland Biopsy

Lymphoma in benign lymphoepithelial lesion

Benign lymphoepithelial lesion in Sjogren's syndrome

Sjogren's syndrome

Lymphoma in benign lymphoepithelial lesion

Benign lymphoepithelial lesion in Sjogren's syndrome

Benign lymphoepithelial lesion

Figure 6.2. The association between the lymphoepithelial lesion, Sjogren's syndrome, and lymphoma.

tions fall to a low level. The accelerated development of dental caries is also noted. Enlargement of the lacrimal glands is uncommon. Even when the salivary glands are not enlarged, they always exhibit lymphohistiocyte-mediated acinar destruction (Marx 1995). When enlarged, however, they show features of the benign lymphoepithelial lesion (BLL) in almost all cases. These lesions may occur in patients who do not have Sjogren's syndrome. Furthermore, they may undergo malignant transformation to lymphomas in patients with or without Sjogren's syndrome (Figure 6.2). This concept, as well as the entity Mikulicz's disease, is clearly worthy of additional discussion.

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