Sjögren syndrome (SS) is a systemic autoimmune disease primarily affecting the salivary and lacrimal glands resulting in dry eyes, dry mouth, and salivary gland swellings. SS may occur primarily or in the setting of autoimmune disease. Rheumatoid arthritis with SS is defined as secondary SS. The prevalence of SS is estimated to be 0.6–6/1000 with predominant female preponderance (10 : 1). The diagnosis (see Chapter 7.2.1 ) based on specific criteria from the American-European Consensus Group (2002) and American College of Rheumatology (2012) comprise of the presence of oral, ocular symptoms and objective measures such as presence of autoantibodies [anti-SSA (Ro) and anti-SSB (La)] and focal lymphocytic sialadenitis on minor salivary gland biopsy (focus score ≥1).
The patients can present predominantly sicca symptoms, or glandular swellings, or both. Sicca complex with dry eyes and dry mouth are serious symptoms that can affect quality of life, pose serious threat for infections, and loss of function. Generally, dry mouth is not treatable with sialendoscopy and consequently should not be considered a primary indication for the procedure. Some patients report benefit from interventional salivary endoscopy with or without infusion of steroids during the procedure; however, this symptomatic benefit tends to be brief and is not reproducible. The role of salivary endoscopy in patients with SS comes into play in patients who present with glandular swellings and pain.
Patients with SS may present with recurrent diffuse swelling of one or more major salivary glands that may or may not be associated with pain. Sialendoscopy can be valuable from a diagnostic and therapeutic perspective in these patients. Endoscopic findings with SS are blanched ducts, loss of vascular striations of the ductal walls, areas of focal stenosis, and debris within the ducts. These findings are also seen in non-stone conditions such as juvenile recurrent parotitis (JRP) and radioactive induced (RAI) sialadenitis that affect the major salivary glands. From a diagnostic perspective, the above endoscopic findings could prompt the sialendoscopist to pursue further work-up to prove an inflammatory or autoimmune etiology for idiopathic chronic recurrent sialadenitis and treat with standard endoscopic techniques that include gland washout with or without steroid infusion, dilation of stenosis, and stenting ( ). Vashishta and Gillespie found 10% of minor salivary gland biopsies in patients with idiopathic sialadenitis have findings suggestive of SS. These endoscopic signs are also often related to early onset disease and patients often report improvement in symptoms. This improvement could be resolution of glandular asymmetry or reduced pain and/or frequency of salivary gland swellings. In patients with longstanding or late-stage SS, endoscopic findings can include sialectasis seen as accordion-like narrowing and dilations of Stensen’s duct and multiple or diffuse stenosis of the ductal system. These patients also often present with thicker mucoid saliva and dry mouth as prominent symptoms ( Fig. 32.1 ). Clinical examination and radiology may demonstrate firm glandular swelling with punctate calcification and/or multicystic changes ( Fig. 32.2 ). Patients with late-stage disease may not benefit from salivary endoscopy. Intraglandular injection of botulinum toxin has been anecdotally helpful to control symptoms in patients who are resistant to medical management with immunosuppression, endoscopy, or anti-inflammatory medication. However, this treatment is not evidence-based. Gland resection is the final common pathway for end-stage disease.
