Sjögren's syndrome is an autoimmune disorder that involves the immune-mediated destruction of the salivary and lacrimal glands, resulting in the loss of tear and saliva production. This report describes a patient with Sjögren's syndrome, whose unusual presentation included multiple parotid cysts.

A 54-year-old woman presented with a progressively enlarging, palpable mass in her right infra-auricular area. She had noticed this mass for about a year. On exam, two nodules, 1 cm and 0.5 cm in diameter, were palpated, which were without any signs of infection. During the interview and physcial exam, the patient also reported a 5-year history of dry eyes and a 2-year history of a dry mouth. She was not taking any medications, and did not have any contributing familial factors.

With a provisional diagnosis of Sjögren's syndrome, a computed tomography (CT) study revealed heterogeneous enhancement of both salivary glands with cystic changes, and atrophic changes in both submandibular glands (Fig. 1). The computed tomographic findings were consistent with a chronic connective tissue disorder such as Sjögren's syndrome. Other cystic conditions such as mucocele, branchial cyst, cystic Warthin's tumor, and human immunodeficiency virus (HIV)-related parotid cyst must be ruled out on clinical and histologic grounds [1]. Ultrasonographic findings revealed multiple homogenous, hypoechoic cystic lesions, most of which were <10 mm in size, in both parotid glands. Ultrasonography-assisted, fine-needle biopsy performed on the dominant cyst in the right gland was negative for malignancy, but polymorphous lymphoid cells were seen which indicate a benign lymphoepithelial disease consistent with Sjögren's syndrome (Fig. 2). A salivary scan showed that both the parotid and submandibular glands had decreased uptake and secretion, which also was suggestive of Sjögren's syndrome.

Serologic examinations revealed elevated rheumatoid factor levels (40.9 IU/mL), anti-SS-A/Ro antibodies (179 IU/mL), anti-SS-B/La antibodies (133 IU/mL), antinuclear antibodies of the speckled and nucleolar type, and 50% hemolytic unit of complement (CH50, 56.3 U/mL). Tests for HIV markers were negative. Schirmer tests were positive for both eyes.

The patient was prescribed methylprednisolone, pilocarpine, hydroxychloroquine, artificial tearing agents, and oral hydrating agents. At 3-months of follow-up, oral and ocular symptoms have improved, but parotid masses still remained. Patients with Sjogren's syndrome have greater than 40-fold higher risk of developing lymphoma. We planned regular follow-up for early detection of signs suggestive of lymphoma. Each follow-up visit should include a clinical examination of the neck to look for signs of lymphadenopathy and of the parotid gland to look for asymmetry and changes in size. If a malignancy is suspected on clinical grounds, the patient should undergo a repeat magnetic resonance imaging (MRI) or CT.

Depending on whether Sjögren's syndrome presents alone or coexists with other autoimmune disorders, it may be designated as "primary" or "secondary." Similar disorders include systemic lupus erythematosus, rheumatoid arthritis, polymyositis, and systemic sclerosis [2].

Diagnostic criteria have changed constantly over the years and controversy remains on which criteria are the most accurate. In 2002, the American-European Consensus Group put forth an international criteria: 1) Ocular signs, Shirmer I test positive; 2) Salivary gland biopsy, focus score of 1 or higher (50 or more lymphocytes per 4 square millimeters); 3) Ocular symptoms, subjective sense of dry eyes and/or use of 3 or more tear substitutes per day; 4) Oral symptoms, subjective sense of dry mouth, recurrent of persistent swollen salivary glands, and/or liquid substitute to assist food swallowing; 5) Autoantibodies, anti-SSA (Ro) or anti-SSB (La), or both; 6) Oral signs, unstimulated whole salivary flow, abnormal parotid sialography, abnormal salivary scintigraphy. To qualify for a diagnosis, patients must present with at least 4 of the criteria with either positive serologic studies (positive anti-SSA and/or anti SSB antigen) or positive salivary gland biopsy [3].

Although the most typical symptoms of Sjögren's syndrome are dry eyes and dry mouth, the clinical presentations vary widely. Approximately one-third of patients with primary Sjögren's syndrome also have diffuse parotid gland enlargement, and 80% of patients experience parotid gland enlargement during their lifetime. However, the presence of multiple, bilateral parotid cysts upon presentation is rare in Sjögren's syndrome; only few such cases have been reported (Table 1) [1,2,4,5,6]. The main differential diagnoses for bilateral parotid cysts, observed by imaging, are cystic Warthin tumors, HIV-related benign lymphoepithelial lesions, and branchial cysts [1].

Various imaging modalities such as CT, MRI, magnetic resonance sialography, and ultrasonography have been applied to evaluate the pathological changes in the salivary glands. Parotid MRI images usually reveal multiple, mixed, hypointense, and hyperintense foci, described as a "salt and pepper appearance", which are considered strongly suggestive of Sjögren's syndrome [2,4,6]. Sun et al. [7], in their evaluation of the diagnostic accuracy of parotid CT, concluded that heterogeneity, abnormal diffuse fat tissue deposition, and diffuse punctate calcification were specific for Sjögren's syndrome. A small proportion (5.8%) of patients had presented with well-circumscribed cystic lesions, which were be confirmed as lymphoepithelial cysts. The pathogenesis of abnormal adipose deposition is still unclear and may be related to the destruction and shrinkage of the excretory system. Lymphocytic infiltration, destruction of the acini, and fibrosis characterized the pathological changes, when the major salivary glands were affected in Sjögren's syndrome.

The imaging findings of the patients previously reported with an initial presentation of multiple, bilateral parotid cysts are shown in Table 1. Som et al. [1] described such findings, but did not publish CT and MRI images of multiple cysts. The first report CT image of such a case was by Plaza et al. [5] in 2003. However, these multifocal cysts had not been detected on CT and were found later using MRI. Gadodia et al. [2] also described CT images of enlarged bilateral glands with various cysts, but did not show the images. The multiple, small, bead-like cysts in our case are clearly evident in the CT sections-a visible reminder that this could be the initial presentation in a patient with Sjögren's syndrome.

Sjögren's syndrome is not an uncommon disease, with an estimated prevalence of 1% to 3% in the general population. However, surgeons often conduct the initial interview the patients presenting with a swelling or mass-like lesion of the cheek or neck. A Sjögren's syndrome diagnosis requires accurate history taking and requires high clinical suspicion for detection. Although multicystic parotid involvement is rare, this presentation should also trigger suspicion.