Salivary manifestations of systemic disease Flashcards
What is the pathophysiologic mechanism of
Sjögren syndrome?
Autoimmune destruction of exocrine glands
What are the two broad classifications of Sjögren
syndrome?
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.
What are the common initial signs and symptoms
of Sjögren syndrome?
Xerostomia, keratoconjunctivitis sicca, and parotid gland
enlargement
Patients with Sjögren syndrome are at risk for developing what neoplasm?
Patients with Sjögren syndrome have a 44 times greater risk
of lymphoma than do matched controls; 5% of patients with Sjögren syndrome develop lymphoma.
What autoimmune disease is associated with primary mucosa-associated lymphoid tissue, or MALT, lymphoma of the salivary glands?
Sjögren n syndrome
What are the risk factors for lymphoma development in Sjögren syndrome?
Risk factors include persistent enlargement of parotid
glands, splenomegaly, lymphadenopathy, palpable purpura,
leg ulcers, or a mixed monoclonal cryoglobulinemia.
Other than clinical history and laboratory workup, what in-office procedure can be performed to help
establish the diagnosis of Sjögren syndrome?
Lower-lip minor salivary gland biopsy
What laboratory tests are most commonly ordered for suspected Sjögren syndrome?
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.
True or false. Benign lymphoepithelial lesion of the salivary glands is another name for benign lymphoepithelial cysts of the salivary glands.
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.
True or false. Patients with benign lymphoepithelial lesions of the salivary glands are at increased risk for malignancy.
True, unlike benign lymphoepithelial cysts, benign lymphoepithelial lesions are at increased risk for lymphoma transformation.
A patient with multiple parotid cysts should be tested for what disease?
HIV
What are the initial symptoms of Kimura disease?
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
What is the population most commonly affected by Kimura disease?
Young males in their 20s and 30s from Southeast Asia
What inflammatory cell is most commonly elevated in Kimura disease?
Eosinophils (both peripheral eosinophilia and intralesional
eosinophilia).
Aside from a CBC, what other blood tests should be ordered in a patient with Kimura disease?
Blood urea nitrogen (BUN), creatinine, and urinary protein
to rule out nephrotic syndrome
What are the treatment options for Kimura disease?
Surgical resection has been considered the treatment of
choice. Observation can be offered if the patient is
asymptomatic. Medical treatment includes steroids and
immunosuppressants.
What is the most common initial manifestation of
necrotizing sialometaplasia?
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.
In a patient with hard palate ulceration, what
conditions should be considered in the differential diagnosis?
Aphthous ulcer, trauma, malignancy such as mucoepidermoid carcinoma or squamous cell carcinoma, necrotizing sialometaplasia
What are the initial symptoms of a patient with adenomatoid hyperplasia of a salivary gland?
Painless swelling of the oral mucosa, suggestive of neoplasia, is usually seen; pathology reveals benign salivary tissue.
Heerfordt syndrome is an extrapulmonary form of what systemic disease?
Sarcoidosis
What are the characteristics of Heerfordt syndrome?
Heerfordt’s syndrome, also know as uveoparotid fever,
presents with uveitis, non-suppurative parotitis, and in 50%
of the patients, facial palsy.
How is uveoparotid fever treated?
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.
What are the treatment options for benign
lymphoepithelial cysts associated with HIV?
Cysts in such cases can be treated with observation,
repeated aspiration, and antiretroviral medication such as
zidovudine, sclerosing agents, radiation, or surgery.
True or false. Benign lymphoepithelial cysts
associated with HIV have malignant potential if not removed.
False. HIV-positive patients are at increased risk of
malignancy, but no reports are documented that show malignant potential of benign lymphoepithelial cysts.
Rapid growth of a salivary gland in a patient with HIV is most concerning for what pathology?
Malignancy such as Kaposi sarcoma or lymphoma
When is surgery indicated for management of
salivary gland enlargement associated with HIV?
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.
Apart from Heerfordt syndrome, how often does sarcoidosis affect the salivary glands?
About 5 to 7% of sarcoidosis patients have salivary gland
enlargement.
How should extrapulmonary sarcoidosis with
persistent parotid swelling be treated?
Oral steroids
What is the presentation of salivary gland involvement with Wegener granulomatosis?
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.
