16.1Introduction

SjšgrenÕs syndrome (SS) is an insidious progressive autoimmune disease. The disorder is characterized by exocrinopathy and is present in two forms, namely, primary SjšgrenÕs syndrome (pSS) and secondary SjšgrenÕs syndrome (sSS). SjšgrenÕs syndrome is more common in women and affects up to 3% of the American population

P.C. Belafsky ( )

The Center for Voice and Swallowing, University of California at Davis, Davis, CA, USA

e-mail: pbelafsky@sbcglobal.net

[1Ð3]. It is the second most common autoimmune disease after rheumatoid arthritis. The disorder has devastating effects and is responsible for dramatic reductions in quality of life.

Although symptoms may affect many organ systems, head and neck manifestations predominate and are frequently the initial presenting complaints [1, 4]. A high index of suspicion should be maintained to permit early diagnosis and minimize long-term complications of the disease. The purpose of this chapter is to review the otolaryngologic manifestations of SjšgrenÕs syndrome.

16.2Diagnosis

There is no single test for SS. There have been at least seven different sets of criteria proposed over the last quarter century [5]. The myriad of criteria has clouded the diagnostic waters and confused both patients and care givers alike. Delay between the onset of patient symptoms and the actual diagnosis may average greater than 6 years [1, 5]. The protracted time until diagnosis often exacerbates patient frustration. Clinicians should remain empathetic and work diligently to restore patientÐphysician conÞdence.

In recent years, trans-Atlantic discussion has resulted in the AmericanÐEuropean Consensus Group criteria published in 2002 [5Ð9]. The criteria exhibit 95% sensitivity and speciÞcity for diagnosis. These criteria include subjective and objective measures related to the sicca syndrome-type ÒglandularÓ manifestationsÑthat

is xerostomia and xerophthalmia caused by exocrine gland dysfunction (salivary and lacrimal glands, respectively) [1, 6]. Up to one-third of patients will also suffer extra-glandular manifestations that may affect diverse organ systems and be mistaken for other disease entities [2, 3]. Pulmonary, renal, cutaneous, and hematologic involvement have all been well documented [1Ð4, 9Ð11].

Workup in patients suspected of SS should include a comprehensive history and physical examination as listed in Table 16.1. Salivary gland enlargement is one of the most frequent physical examination Þndings encountered in the head and neck and is often one of the initial manifestations of the disease. Patients with diffuse parotid or submandibular gland enlargement and dry mucous membranes should be considered to have SS until proven otherwise. When examining the oral cavity and pharynx with a

wooden disposable tongue blade, the moisture in the mouth should not allow the blade to stick to the inner mucosa of the cheek. If the tongue blade does stick to the mucosa, oral moisture is poor and screening for SS is indicated. We refer to this as the Oral Allen Test.

Investigations in addition to normal serological markers include salivary gland ultrasound, computed tomography (CT), and sialography. Biopsies of affected minor or major salivary glands can be diagnostic and there is new evidence that suggests Þne needle aspiration of the parotid gland can be diagnostic even when labial biopsies fail to demonstrate SS [12]. Pijpe and colleagues have compared parotid biopsy to labial biopsy and suggest it is equally speciÞc and sensitive in the diagnosis of SS [13]. They site ease of harvest, low morbidity, and incidental diagnosis of lymphoma as advantages of parotid biopsy [13]. Distinct lesions palpable in glands must be biopsied promptly and may require ultrasound guidance. CT scanning allows assessment of both glands for lesions such as WarthinÕs tumors that may be seen bilaterally or for discreet lesions such as lymphoma. CT imaging also helps deÞne the medial extent of a parotid mass into the parapharyngeal space (seen with deep lobe pleiomorphic adenomas) and helps identify cervical lymphadenopathy.

Recent publications have focused on new diagnostic techniques with the aim of early identiÞcation of SjšgrenÕs development and knowledge that 15Ð26% of SS patients may not display typical xerostomia [1, 14]. Mignogna and colleagues suggest that sialochemistry, sialorrhea, early dental loss, and salivary gland swelling may occur well before development of hyposalivation and resultant xerostomia [14]. Hocevar et al. have suggested the use of an ultrasound scoring system (USS) to assist in the diagnosis given the non-invasive nature of the examination [15]. A recent publication further supports USS as a cheap, readily available, and non-invasive method of examining salivary glands in SS. Parenchymal inhomogeneity is said to be most speciÞc for involvement of glands with SS [16, 17]. USS was suggested as a further diagnostic adjunct to the

current AmericanÐEuropean Consensus Group criteria [16]. Sialography involves Þlling the salivary gland ductal system with radio-opaque contrast media and obtaining plain X-ray Þlms. A typical pattern of ductal ectasia with a ÒsnowstormÓ punctuate appearance of isolated acini can be seen in established SjšgrenÕs syndrome. This is a technically demanding study requiring cannulation of StensonÕs or WhartonÕs duct and is frequently painful [18, 19]. Salivary scintigraphy has been in use for more than 30 years and is non-invasive. It requires, however, the use of radioactive tracers and lacks quantitative parameters necessary to consistently establish a diagnosis [18]. The most reliable Þndings are a reduced parotid:submandibular gland uptake ratio (P:S ratio) and excretion fraction ratio less than 50% in the parotid (suggesting abnormal function). More than 25% of the gland mass must be lost to see a detectable difference. This is highly relevant in light of recent evidence that functional acini remain present in dysfunctional glands, suggesting neurohumoral mechanisms of reduced salivary ßow [14]. It is used less commonly now, being replaced by CT scanning, USS, and magnetic resonance scans.

Magnetic resonance imaging and MR sialography (MRS) are fast becoming diagnostic modalities. They are highly sensitive (comparable to USS and better than scintigraphy), noninvasive, quickly performed, and not particularly operator-dependent. Performed with surface coils rather than head coils, the resolution is improved, particularly when the studies are combined. Excellent deÞnition of the ductal system can be achieved and parenchymal changes noted [20, 21]. Furthermore Morimoto et al. have described dynamic MRS combining both morphological assessment and functional assessment (contrasting scans before and after citric acid stimulation) [22]. It is likely that MR studies will become more widespread in the diagnosis of salivary glandular abnormalities and SS [20Ð22].

The astute clinician employs a combination of serologic tests, imaging, and/or labial/parotid gland biopsy at an early stage to conÞrm diagnostic suspicion.