Initial Sjögren’s Manifestations Can Go Beyond Dry Eyes & Mouth

The Vocal Cords
Claus Lunau / Science Source

Primary Sjögren’s syndrome is a systemic autoimmune condition noted for findings of xerostomia, keratoconjunctivitis sicca and focal lymphocyte infiltrate in salivary glands.¹ In the initial publications regarding keratoconjunctivitis sicca, Henrik Sjögren, a Swedish ophthalmologist, described a group of 19 women with dry eyes, some of whom had other organ dryness and inflammatory infiltrates.^2,3 The syndrome that took his name can also involve extraglandular organs and include inflammatory arthritis, interstitial lung disease, pericarditis, myocarditis, primary biliary cirrhosis, vasculitis and blood dyscrasia. Laryngeal involvement is rare and is often missed as an initial manifestation of Sjögren’s syndrome.⁴

Our Case

A 41-year-old Caucasian female with no significant past medical history was referred for rheumatologic evaluation by her primary care physician after she experienced fatigue, xerophthalmia (i.e., abnormal dryness of the conjunctiva and cornea of the eye, with inflammation and ridge formation), xerostomia (i.e., oral dryness), triphasic color changes in her digits with exposure to cold and stress, and dyspareunia (i.e., difficult or painful sexual intercourse). These symptoms had been progressively worsening over the span of 18 months.

The patient reported a history of difficulty speaking (i.e., dysphonia) that started six to 12 months before the previously mentioned symptoms developed. However, she was not evaluated by an otolaryngologist until after her rheumatologic workup was initiated.

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