Salivary manifestations of systemic disease

Question 1

What is the pathophysiologic mechanism of

Sjögren syndrome?

Answer 1

Autoimmune destruction of exocrine glands

Question 2

What are the two broad classifications of Sjögren

syndrome?

Answer 2

The two classifications are primary and secondary Sjögren
syndrome. Primary cases involve only exocrine glands.
Secondary cases involve exocrine glands and any additional
connective tissue disease.

Question 3

What are the common initial signs and symptoms

of Sjögren syndrome?

Answer 3

Xerostomia, keratoconjunctivitis sicca, and parotid gland

enlargement

Question 4

Patients with Sjögren syndrome are at risk for developing what neoplasm?

Answer 4

Patients with Sjögren syndrome have a 44 times greater risk

of lymphoma than do matched controls; 5% of patients with Sjögren syndrome develop lymphoma.

Question 5

What autoimmune disease is associated with primary mucosa-associated lymphoid tissue, or MALT, lymphoma of the salivary glands?

Answer 5

Sjögren n syndrome

Question 6

What are the risk factors for lymphoma development in Sjögren syndrome?

Answer 6

Risk factors include persistent enlargement of parotid
glands, splenomegaly, lymphadenopathy, palpable purpura,
leg ulcers, or a mixed monoclonal cryoglobulinemia.

Question 7

Other than clinical history and laboratory workup, what in-office procedure can be performed to help
establish the diagnosis of Sjögren syndrome?

Answer 7

Lower-lip minor salivary gland biopsy

Question 8

What laboratory tests are most commonly ordered for suspected Sjögren syndrome?

Answer 8

A rheumatologic workup for Sjögren syndrome includes SS-
A and SS-B, the most specific tests for Sjögren syndrome.
Additional tests including antinuclear antibodies, rheumatoid factor, and sedimentation rates are also frequently obtained.

Question 9

True or false. Benign lymphoepithelial lesion of the salivary glands is another name for benign lymphoepithelial cysts of the salivary glands.

Answer 9

False. Although often used incorrectly in the literature, the term benign lymphoepithelial lesions of the salivary glands are clinically and histologically distinct from benign lymphoepithelial cysts. Benign lymphoepithelial lesions are thought to result from an autoimmune condition related to other disease processes such as Sjögren syndrome, Hashimoto disease, chronic sclerosing sialadenitis, sarcoidosis, and HIV.

Question 10

True or false. Patients with benign lymphoepithelial lesions of the salivary glands are at increased risk for malignancy.

Answer 10

True, unlike benign lymphoepithelial cysts, benign lymphoepithelial lesions are at increased risk for lymphoma transformation.

Question 11

A patient with multiple parotid cysts should be tested for what disease?

Answer 11

HIV

Question 12

What are the initial symptoms of Kimura disease?

Answer 12

Diffuse lymphadenopathy, salivary gland swelling (parotid or submandibular), red cutaneous nodules on the head and
neck, as well as pigmented, coarse, pruritic skin overlying
glandular swelling

Question 13

What is the population most commonly affected by Kimura disease?

Answer 13

Young males in their 20s and 30s from Southeast Asia

Question 14

What inflammatory cell is most commonly elevated in Kimura disease?

Answer 14

Eosinophils (both peripheral eosinophilia and intralesional

eosinophilia).

Question 15

Aside from a CBC, what other blood tests should be ordered in a patient with Kimura disease?

Answer 15

Blood urea nitrogen (BUN), creatinine, and urinary protein

to rule out nephrotic syndrome

Question 16

What are the treatment options for Kimura disease?

Answer 16

Surgical resection has been considered the treatment of
choice. Observation can be offered if the patient is
asymptomatic. Medical treatment includes steroids and
immunosuppressants.

Question 17

What is the most common initial manifestation of

necrotizing sialometaplasia?

Answer 17

Ulcer on the posterior hard palate is the most common
symptom. The lesions of necrotizing sialometaplasia heal
spontaneously in 5 to 9 weeks; thus, the only treatment
required is supportive care.

Question 18

In a patient with hard palate ulceration, what

conditions should be considered in the differential diagnosis?

Answer 18

Aphthous ulcer, trauma, malignancy such as mucoepidermoid carcinoma or squamous cell carcinoma, necrotizing sialometaplasia

Question 19

What are the initial symptoms of a patient with adenomatoid hyperplasia of a salivary gland?

Answer 19

Painless swelling of the oral mucosa, suggestive of neoplasia, is usually seen; pathology reveals benign salivary tissue.

Question 20

Heerfordt syndrome is an extrapulmonary form of what systemic disease?

Answer 20

Sarcoidosis

Question 21

What are the characteristics of Heerfordt syndrome?

Answer 21

Heerfordt’s syndrome, also know as uveoparotid fever,
presents with uveitis, non-suppurative parotitis, and in 50%
of the patients, facial palsy.

Question 22

How is uveoparotid fever treated?

Answer 22

Heerfordt syndrome, also known as uveoparotid fever,
initially manifests with uveitis, nonsuppurative parotitis, and
facial palsy. Treatment for Heerfordt syndrome includes
systemic steroids and eye care.

Question 23

What are the treatment options for benign

lymphoepithelial cysts associated with HIV?

Answer 23

Cysts in such cases can be treated with observation,
repeated aspiration, and antiretroviral medication such as
zidovudine, sclerosing agents, radiation, or surgery.

Question 24

True or false. Benign lymphoepithelial cysts

associated with HIV have malignant potential if not removed.

Answer 24

False. HIV-positive patients are at increased risk of

malignancy, but no reports are documented that show malignant potential of benign lymphoepithelial cysts.

Question 25

Rapid growth of a salivary gland in a patient with HIV is most concerning for what pathology?

Answer 25

Malignancy such as Kaposi sarcoma or lymphoma

Question 26

When is surgery indicated for management of

salivary gland enlargement associated with HIV?

Answer 26

When a gland undergoes rapid growth or other clinical indicators of malignancy, surgery may also be offered if
conservative management fails and a patient is worried
about cosmesis.

Question 27

Apart from Heerfordt syndrome, how often does sarcoidosis affect the salivary glands?

Answer 27

About 5 to 7% of sarcoidosis patients have salivary gland

enlargement.

Question 28

How should extrapulmonary sarcoidosis with

persistent parotid swelling be treated?

Answer 28

Oral steroids

Question 29

What is the presentation of salivary gland involvement with Wegener granulomatosis?

Answer 29

It is a rare association, but patients with Wegener granulomatosis and parotid involvement most often have painful parotid swelling. Biopsy shows necrotizing granulomatous changes and microabscesses.