A 48-year-old woman comes to the office due to difficulty swallowing dry foods such as crackers. She has no pain during swallowing or heartburn. The patient is a college professor and has to drink water frequently during lectures to keep her mouth moist. She also reports a gritty sensation in her eyes, which is often worse in the evening, and occasional brief episodes of joint pain. Medical history is significant for hypothyroidism, for which she takes levothyroxine. Physical examination shows mild conjunctival erythema and cracking of the lips. There is diffuse, nontender thyroid enlargement, which is unchanged from prior examination. The remainder of the examination is normal. Biopsy of the lip mucosa is most likely to reveal which of the following findings?

Question

A 48-year-old woman comes to the office due to difficulty swallowing dry foods such as crackers.  She has no pain during swallowing or heartburn.  The patient is a college professor and has to drink water frequently during lectures to keep her mouth moist.  She also reports a gritty sensation in her eyes, which is often worse in the evening, and occasional brief episodes of joint pain.  Medical history is significant for hypothyroidism, for which she takes levothyroxine.  Physical examination shows mild conjunctival erythema and cracking of the lips.  There is diffuse, nontender thyroid enlargement, which is unchanged from prior examination.  The remainder of the examination is normal.  Biopsy of the lip mucosa is most likely to reveal which of the following findings?

Quizplus · Accepted Answer

11ec160b_3a6a_77ae_9249_5bbf434dd5cf_MD0001_00 Sjögren syndrome is an autoimmune disorder characterized by inflammation of exocrine (eg, salivary, lacrimal) glands. It can occur as an isolated disorder or as a manifestation of another autoimmune syndrome (eg, rheumatoid arthritis). Patients typically have severe dry mouth (xerostomia) and dry eyes (keratoconjunctivitis sicca); vaginal gland involvement is also common. Characteristic serologic markers of Sjögren syndrome include anti-Ro (SSA) and anti-La (SSB). Antinuclear antibodies and rheumatoid factor are often positive but less specific. Biopsy of the labial salivary glands demonstrates intense periductal lymphocytic infiltrates (focal lymphocytic sialadenitis), often with germinal centers; the glandular tissue is typically atrophic and fibrotic. (Choice A) Amyloidosis is a group of disorders characterized by deposition of abnormal proteins in various tissues (eg, kidneys, heart). Light microscopy shows amorphous eosinophilic protein deposits; polarized microscopy with Congo red staining may elicit bright green birefringence. (Choice B) Oral leukoplakia is a premalignant lesion characterized by hyperplasia of the squamous mucosa. The hyperkeratinized patches appear white against the normal red or pink mucosa. In the absence of malignant transformation, these lesions are typically asymptomatic; this patient's dry mouth and dry eye symptoms are more consistent with Sjögren syndrome. (Choice C) Pemphigus vulgaris is an autoimmune disease characterized by painful, flaccid bullae and erosions of the skin and mucosa. Biopsy of an active lesion will show intraepithelial cleavage with detached keratinocytes (acantholysis), retained keratinocytes along the basement membrane, and an eosinophilic inflammatory infiltrate. (Choice E) Sarcoidosis is an inflammatory disease characterized by noncaseating granuloma formation in multiple tissues. Pulmonary and constitutional symptoms are common. Ocular involvement may cause uveitis, but conjunctival erythema and dry eye symptoms are more consistent with Sjögren syndrome. Educational objective: Sjögren syndrome is an autoimmune disorder characterized by inflammation of exocrine glands. Biopsy of the labial salivary glands shows periductal lymphocytic infiltrates (focal lymphocytic sialadenitis), often with germinal centers; the glandular tissue is typically atrophic and fibrotic.

A 48-Year-Old Woman Comes to the Office Due to Difficulty