A 65-year-old woman comes to the physician to establish care. She reports a 10-year history of dry eyes and mouth that she has managed with over-the-counter artificial tear and saliva products. The patient has had intermittent painless swelling of her parotid glands for the past 3 years. She also complains of occasional joint pain relieved by ibuprofen. She does not use tobacco, alcohol, or illicit drugs.
Her temperature is 37.2° C (99° F), blood pressure is 142/92 mm Hg, and pulse is 78/min. Physical examination shows several mildly enlarged, nontender, mobile cervical and supraclavicular lymph nodes. Conjunctivae appear dry. Oral mucosa is dry with atrophic glossitis. Severe dental caries are noted as are several missing teeth. The left parotid gland is enlarged and hard to the touch but is nontender. There is no joint swelling or deformity. The remainder of the physical examination is within normal limits.
Laboratory results are as follows:

Which of the following is the most likely cause of the enlarged parotid gland?

Question

A 65-year-old woman comes to the physician to establish care.  She reports a 10-year history of dry eyes and mouth that she has managed with over-the-counter artificial tear and saliva products.  The patient has had intermittent painless swelling of her parotid glands for the past 3 years.  She also complains of occasional joint pain relieved by ibuprofen.  She does not use tobacco, alcohol, or illicit drugs.
Her temperature is 37.2° C (99° F), blood pressure is 142/92 mm Hg, and pulse is 78/min.  Physical examination shows several mildly enlarged, nontender, mobile cervical and supraclavicular lymph nodes.  Conjunctivae appear dry.  Oral mucosa is dry with atrophic glossitis.  Severe dental caries are noted as are several missing teeth.  The left parotid gland is enlarged and hard to the touch but is nontender.  There is no joint swelling or deformity.  The remainder of the physical examination is within normal limits.
Laboratory results are as follows:

Which of the following is the most likely cause of the enlarged parotid gland?

Quizplus · Accepted Answer

This patient's presentation with dry eyes (xerophthalmia), dry mouth (xerostomia), parotid gland enlargement, and dental caries suggests Sjögren's syndrome (SS). SS occurs most commonly in middle-aged women. Xerostomia can lead to difficulty chewing and swallowing, painful sensations in the mouth and tongue, and accelerated dental caries. Long-standing (> 6 years) SS has a nearly 5% lifetime risk of developing into non-Hodgkin lymphoma (NHL), which is the most likely diagnosis in this patient. Lymphoma is likely due to malignant transformation of lymphoproliferative glandular tissue in SS patients. Other risk factors include anti-Ro/SSA or anti-La/SSB antibody positivity, rheumatoid factor positivity, cryoglobulinemia, hypocomplementemia, and lymphocytopenia. The primary NHL associated with SS usually presents extranodally (commonly in the parotid gland) and is a low-grade, marginal zone B-cell lymphoma. Diagnosis is made similarly to other NHLs with excisional biopsy of an intact node. Chemotherapy is usually efficient in treating these lymphomas. (Choice B) Chronic hepatitis C virus infection may also be associated with sialadenitis and lymphomas. However, hepatitis C-associated glandular disease is often asymptomatic without the dry eyes and mouth seen in this patient. (Choice C) An impacted sialolith is more likely to be associated with pain, swelling, and erythema. Patients may also have systemic symptoms (fevers and chills) due to the infected salivary duct. (Choice D) Pleomorphic adenomas are the most common benign salivary gland tumors. However, this patient's history of long-standing SS makes B-cell lymphoma more likely. (Choice E) Positive rheumatoid factor is often seen in SS due to polyclonal immune activation. However, this patient has no signs and symptoms (eg, joint stiffness, rheumatoid nodules) suggesting rheumatoid arthritis. Educational objective: Non-Hodgkin lymphoma is a long-term (> 6 years) complication of Sjögren's syndrome. Risk factors include anti-Ro/SSA or anti-La/SSB antibody positivity, rheumatoid factor positivity, cryoglobulinemia, hypocomplementemia, and lymphocytopenia. __________ References: Lymphomas in patients with Sjögren's syndrome are marginal zone B-cell neoplasms, arise in diverse extranodal and nodal sites, and are not associated with viruses. (http://www.ncbi.nlm.nih.gov/pubmed/9226177) Predictors for the development of non-Hodgkin lymphoma in primary Sjögren's syndrome. (http://www.ncbi.nlm.nih.gov/pubmed/22867948)

A 65-Year-Old Woman Comes to the Physician to Establish Care