Case Report

Unusual Presentation of Sjögren Syndrome

Authors: K Kulkarni, MD


This case report describes a patient with Sjögren syndrome (SS) whose sole presenting feature was bilateral and painful submandibular gland enlargement. Extensive workup for alternate etiologies was negative and while serologies specific for SS were unremarkable, the diagnosis was eventually suggested by excisional biopsy. This case highlights the difficulty in making an early diagnosis of SS and demonstrates the important role of excisional biopsy in that diagnosis.

Key Points

* Sjögren syndrome is an autoimmune condition characterized by exocrine gland dysfunction that presents with a variety of symptoms, including keratoconjunctivitis sicca, xerostomia, and salivary gland swelling typically involving the parotid gland.

* Most diagnostic criteria rely on the presence of autoantibodies and the objective demonstration of salivary gland involvement through biopsy or imaging studies.

* Since autoantibodies may be normal early in the disease, biopsy is indicated for younger patients in whom the diagnosis is being entertained.

* Early diagnosis is essential because long-term surveillance for the development of malignant lymphoma is necessary.

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