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Neuroradiology Case of the Month

November 2001

Jose A. Echeverri, M.D., Toshio Moritani, M.D., Ph.D., Ahmed Abdelhalim, M.D.,
and P.L. Westesson, M.D., Ph.D., D.D.S.

Clinical Presentation: A 64-year-old woman with a history of Sjogren’s syndrome and multiple sclerosis presents with enlargement of the parotid glands and was evaluated with MR imaging.

Imaging Findings: MRI demonstrates enlarged bilateral parotid glands with FSEIR hypointense, and T2 hyperintense globular cystic-like changes.

Diagnosis: Consistent with Sjogren’s syndrome involvement of the parotid glands.

Figure 1. Axial FSEIR: Enlarged bilateral parotid glands with hypointense cystic-like changes.	Figure 2. Axial T2W FSE: enlarged parotid glands containing T2 hyperintense globular collections of watery saliva.

Discussion: Sjogren’s syndrome is a systemic autoimmune disorder of the exocrine glands that occurs either alone (referred to as primary Sjogren’s syndrome) or with any of several connective tissue diseases (referred to as secondary Sjogren’s syndrome). Traditionally, the diagnosis is established when two or more of the following clinical hallmarks are present: keratoconjunctivitis sicca, xerostomia, and a connective tissue disease, which usually is rheumatoid arthritis. The incidence of sicca complex in rheumatoid arthritis is estimated to be 15-25%. Other connective tissue diseases associated with secondary Sjogren’s syndrome are SLE, progressive systemic sclerosis, polymyositis, poliarteritis nodosa, and mixed connective tissue disease [1].
Sjogren’s syndrome is characterized by failure of exocrine glands resulting in the so called sicca syndrome (dry eyes and mouth), parotid gland enlargement, dental caries, and recurrent tracheobronchitis [2].

In the earliest stages of Sjogren’s syndrome, CT and MRI of the involved glands are normal. As the disease progresses and the glands increase in size, on CT a honeycomb glandular appearance develops. This CT appearance, once thought to be diagnostic of Sjogren’s syndrome, is now known to be produced by other granulomatous diseases or chronic sialadenitis [1].

On MRI, globular collections of watery saliva can be seen in the parotid glands. This MR appearance is characteristic of Sjogren’s syndrome [1].

There are other immunologic disorders that are associated with salivary gland enlargement and which cause salivary dysfunction and a sicca syndrome . These include patients infected with HIV, human T-cell lymphotrophic virus type III (HTLV-3), sialosis, granulomatous-type diseases such as sarcoidosis, amyloidosis, primary biliary cirrhosis, and graft vs host disease [1].

References:

1. Som PM, Brandwein M, Salivary Glands. In: Som PM, Curtin HD eds. Head and Neck Imaging, 3rd edition. St. Louis, MO: Mosby, 1996; 823-914.
2. Kahl LE. In: Ewald GA, Mckenzie CR eds. The Washington Manual, 28th edition. St. Louis, MO: Little, Brown and Company, 1995; 505-528.