has been identiÞed in patients with autoimmune pathologies such as SjšgrenÕs syndrome, rheumatoid arthritis, and systemic lupus erythematosus (SLE) [2, 47]. Murano describes two patients in whom this lesion was identiÞed prior to the diagnosis of autoimmunity. Subsequent testing revealed one patient to have pSS and the other sSS associated with SLE. Microscopically the ÒnodeÓ seems to consist of eosinophilic material surrounded by a granulomatous reaction and Þbrosis [47]. Murano suggests that identiÞcation of the bamboo node can be the Þrst indication of the underlying systemic pathology in these cases and that voice quality is affected by the node due to stiffness and loss of the mucosal wave in the vocal fold at the site [47].

Vocal fold immobility in patients exhibiting SS Þndings may be due to either cricoarytenoid joint ankylosis or autoimmune neuropathy. In cases of secondary SSs that are associated with rheumatoid arthritis there may be cricoarytenoid joint ankylosis and rheumatoid bamboo nodes.

Treatment of laryngeal symptoms includes humidiÞcation, usually best achieved with personal steamers or nebulizer devices that aerosolize sterile water or saline, control of reßux (see below), and avoidance of other irritants to the larynx such as tobacco smoke and environmental pollutants. Drying medications including anti-cholinergic and anti-histamine preparations should be used with caution as they may aggravate the autoimmune xerotrachea and xerostomia already present. Anti-histamine medications may also thicken mucus, which can make it harder to clear. Dysphonia secondary to vocal fold immobility can be addressed by joint mobilization, vocal fold augmentation or, rarely, arytenoid adduction.

16.3.6 Esophageal

Dysphagia is a common symptom among SS patients. Reports suggest that anywhere from one-third to 80% of patients complain of difÞculty swallowing [43, 48Ð52]. Multiple mechanisms contribute to swallowing difÞculties in these patients (Table 16.4). Lack of saliva

Table 16.4 Mechanisms contributing to dysphagia in SjšgrenÕs syndrome

HyposalivationÑpoor lubrication, poor bolus transit, loss of salivary buffering, poor esophageal residue clearance Esophageal dysmotilityÑprimary or secondary to prolonged acid clearance,

Gastroesophageal reßux (GER)

Esophageal seromucinous gland failure

Autonomic dysfunction

Webs and strictures

Esophagitis

contributes to dysphagia both directly, by failing to adequately lubricate a bolus and indirectly by the inability to ßush the esophagus and neutralize regurgitated acid content. Gastroesophageal and laryngopharyngeal reßuxes occur, as in the normal population, but patients with SS lack the innate defenses to cope with acid and pepsincontaining reßuxate.

Usually a three-tiered defense system protects the esophagus and larynx from gastric content. The combined valves of the upper and lower esophageal sphincters prevent gross volume reßux. Despite tonic closure in most situations the lower esophageal sphincter (LES) is known to experience transient relaxations (TLESRÕs) many times per day [53Ð56]. It is likely a venting mechanism to release gas and reduce pressure [43]. Investigators have demonstrated a hypotonic upper esophageal sphincter (UES) and LES (a risk factor for reßux) in up to 62% of patients with SS [46, 48]. Up to 50 acidiÞcation episodes (pH < 4) per 24 h is considered physiological reßux. Normal subjects respond to these episodes by secondary esophageal peristalsis to clear the majority of reßuxate and by combined secretion from esophageal submucosal glands and saliva, which dilutes and neutralizes the acid component, ßushing the remaining reßuxate back into the stomach [43, 57]. The patient with SS lacks the buffering capacity and dilutional effects of saliva. This may result in esophagitis or other complications of reßux (stricture, esophageal spasm) in these patients (Fig. 16.6).

In addition, one-third of SS patients may experience esophageal dysmotility, exacerbating poor

esophageal clearance [43, 48, 49, 52, 58, 59]. Increased esophageal intraluminal acid time may have deleterious effects on the esophagus, causing or worsening dysmotility and poor contractility, thus creating a vicious cycle [43, 48]. Motility disorders identiÞed in the SS population cover a wide range of pathologies from achalasia to non-speciÞc body dysmotility [48, 49, 58, 59]. Volter suggests that dysmotility is the result of GER and prolonged esophageal acid exposure rather than being a primary abnormality in these patients [48]. There is a general lack of consistency in Þndings surrounding dysmotility, however, with studies demonstrating contradictory results [48, 50Ð52, 59, 60]. Various investigators have reported increased and decreased LES pressures [49, 50, 59, 61]. The most consistent Þnding is of decreased esophageal body velocities [59, 61]. There may be difÞculty comparing study Þndings as many older studies use different diagnostic criteria for deÞning SS (prior to the AmericanÐEuropean Consensus Group criteria).

An esophageal web has been described in up to 10% of patients with SS [50]. This prevalence is higher than age-matched controls. Webs frequently cause solid food dysphagia and are very amenable to dilation. Autonomic neuropathy has also been detected in SS, particularly impaired parasympathetic function. This was signiÞcantly related to dysphagia symptoms [51]. Autonomic failure may impact submucosal gland secretory function, particularly if antibodies to muscarinic

receptors are present [51]. Neuropathy, both central and peripheral, can be detected in some SS patients [1, 62]. It is possible that neural dysfunction contributes to myenteric plexus failure, secretory failure, and esophageal dysmotility. Myenteric plexus failure contributes to achalasia and this has been described in association with pSS [13, 62].

One of the most common medications prescribed for reßuxÑomeprazoleÑmay decrease salivary ßow and thus be less beneÞcial in SS patients [43].

Patients complaining of heartburn or dysphagia should undergo screening with esophagoscopy and videoßuoroscopy. If the endoscopy and ßuoroscopy do not identify the etiology of dysphagia, manometry is indicated. The advent of unsedated transnasal esophagoscopy (TNE) has greatly increased our ability to screen the esophagus in the ofÞce setting. The majority of complications of esophagoscopy are respiratory and cardiac events related to the intravenous sedation. Unsedated transnasal ofÞce esophagoscopy has greatly improved the safety and accessibility of endoscopic esophageal screening. Ambulatory pH testing may be useful if there are equivocal signs of reßux. Esophagitis, strictures, hiatal hernia, and BarrettÕs metaplasia (BM) can be identiÞed and treated appropriately. All patients with BM must be biopsied and routinely followed to ensure there is no progression to adenocarcinoma.