SALIVmY GLAND DISEASES 0030-6665/99 $8.00 + .OO

CHRONIC INFLAMMATORY DISORDERS OF

THE SALIVARY GLANDS Dale H. Rice, MD

CHRONIC SlALADENlTlS

The key causative event in chronic sialadenitis is believed to be a lowered secretion rate with subsequent salivary stasis. Chronic sialaden- itis, like acute sialadenitis, is more common in the parotid gland than in other locations. This condition can result from permanent damage to the gland from acute suppurative infection. This damage leads over time to sialectasis, ductal ectasia, and progressive acinar destruction combined with a lymphocytic infiltrate. Histologically, it is impossible to differen- tiate the subtypes of chronic salivary inflammation because the salivary glands have a similar tissue response in all these diseases (Fig. 1). The sialographic appearance parallels the degree of histologic damage.

In patients with chronic sialadenitis, there generally is a history of mildly painful recurrent parotid enlargement, usually aggravated by eat- ing. The physical examination confirms this enlargement, and massage of the gland may produce scanty saliva at the duct orifice. Up to 80% of these patients develop permanent xerostomia of the involved gland. Of impor- tance in the initial work-up, the clinician should look for a treatable pre- disposing factor, such as a calculus or a stricture. If no treatable cause is found, initial management should be conservative and includes the use

From the Department of Otolaryngology-Head and Neck Surgery, University of Southern California, Los Angeles, California

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OTOLARYNGOLOGIC CLINICS OF NORTH AMERICA

VOLUME 32 NUMBER 5 * OCTOBER 1999 813

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Figure 1. Chronic sialadenitis (hematoxylin-eosin, original magnification x 100).

of sialagogues, massage, and antibiotics for acute exacerbations. In gen- eral, conservative measures suffice. Other recommended treatment mea- sures, should conservative measures fail, include periodic ductal dilation, ligation of the duct, total gland irradiation, tympanic neurectomy, and excision of the gland. Only the last option works

Chronic recurrent parotitis may lead eventually to the development of the benign lymphoepithelial l e~ ion .~ This lesion belongs in the spectrum of diseases characterized by a lymphoreticular infiltrate combined with acinar atrophy and ductal metaplasia. The ductal metaplasia ends in the development of the so-called epimyoepithelial island. This lesion gener- ally is first noticed as an asymptomatic enlargement unless there is asso- ciated infection. There is a female predominance, and it is most common in the fifth and sixth decades. The parotid most commonly is involved. Clinically this is sometimes called Mikulicz’s disease. In the absence of complications, no active treatment is needed. If intermittent infections oc- cur, each should be treated as acute sialadenitis. If the lesion becomes cosmetically unacceptable, excision may be necessary.

Although a more conservative treatment is generally appropriate, lymphoepithelial lesions should be followed carefully. There are numer- ous well-documented cases of the evolution of this disease into more ag- gressive entities, including lymphoproliferative disorders, carcinoma, and pseudolymphoma. The lymphoproliferative disorders are usually non- Hodkin’s lymphomas involving extrasalivary sites. The carcinomas gen-

CHRONIC INFLAMMATORY DISORDERS OF THE SALIVARY GLANDS 815

erally have been salivary, with the most common type being undifferen- tiated carcinoma.

TUBERCULOSIS

Primary salivary tuberculosis is uncommon. The disease is usually unilateral, and the parotid gland is the most frequently involved salivary gland. It is believed to arise from spread of a focus of infection in the tonsils or the teeth. It occurs in two different forms-an acute inflam- matory lesion or a chronic tumorous lesion. The inflammatory lesion may be a difficult diagnostic problem because it may mimic the more common acute inflammatory diseases. Often the diagnosis is not made until an acid-fast salivary stain and a purified protein derivative (PPD) skin test are performed. The PPD test may be unreliable because infection caused by the atypical mycobacteria are increasing in relation to Mycobacterium tuberculosis hominis, and often produce a negative skin test.9 The treatment is as for any acute tuberculosis infection. The tumorous lesion is more subtle but may be diagnosed correctly by fine-needle aspiration cytology. If the mycobacterium involved is susceptible to the usual drugs, that is the appropriate treatment. If it is resistant, excision is curative. Secondary tuberculosis may involve also the salivary glands but tends to involve the submandibular glands more frequently than the parotid and is associated with active pulmonary tuberculosis.

SARCOIDOSIS

Sarcoidosis is a granulomatous disease of unknown cause, and is a diagnosis of exclusion. Histologically, there are noncaseating granulomas. Clinically, salivary gland involvement occurs in only 6% of cases, but in histologic studies involvement can be demonstrated in up to 33% of cases.5 Uveoparotid fever (Heerfordt’s disease) is a particular form of sarcoidosis characterized by uveitis, parotid enlargement, and facial paralysis. It usu- ally occurs in the 20s to 30s. Initial symptoms include a prodrome of fever, malaise, weakness, nausea, and night sweats lasting several days to sev- eral weeks. Uveoparotid fever may occur with or without the other sys- temic manifestations of sarcoidosis. The swelling lasts months to years without suppuration and with eventual resolution. The treatment is symptomatic, with steroids being most useful in the acute phase, particu- larly for facial paralysis. Without treatment, facial paralysis is usually transient. Uveitis should be followed closely because it can lead to glau- coma. In many patients with sarcoidosis, the serum angiotensin convert- ing enzyme level will be elevated.

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ANIMAL SCRATCH DISEASE

Animal scratch disease does not involve the salivary glands directly but may involve the periparotid and submandibular triangle lymph nodes and the salivary glands by contiguous spread. The causative agent of this disease has been identified but not classified. It is a gram-negative bacillus sensitive to gentamicin.2 The disease is self-limited, and treatment is symptomatic but probably should include gentamicin.

ACTINOMYCOSIS

Actinomycosis also may occur in the salivary glands. Treatment in- volves incision and drainage combined with long-term penicillin therapy, as with actinomycosis elsewhere in the head and neck. The spread of the bacteria is usually from the tonsils or teeth.

SJOGREN’S SYNDROME

Sjogren’s syndrome is characterized by a lymphocyte-mediated de- struction of the exocrine glands leading to xerostomia and keratoconjunc- tivitis sicca (Figs. 2 and 3). It is the second most common autoimmune disease after rheumatoid arthriti~.~ Ninety percent of cases occur in

Figure 2. Sjogren’s syndrome (hematoxylin-eosin, original magnification x 100).

CHRONIC INFLAMMATORY DISORDERS OF THE SALNARY GLANDS 817

Figure 3. Sialogram of Sjogren’s syndrome.

women, and the disease may occur in children. The average age of onset is 50 years. The classic monograph on the disease was published in 1933 by Sjogren: a Swedish ophthalmologist. Sjogren’s syndrome is now clas- sified into two forms: primary Sjogren‘s involves the exocrine glands only, whereas secondary Sjogren‘s is associated with a definable autoimmune disease, usually rheumatoid arthritis6

In 80% of primary and 30% to 40% of secondary cases, unilateral or bilateral salivary gland swelling occurs. The swelling may be intermittent or permanent. Arthritis is the second most common complaint in second- ary Sjogren’s syndrome. There are genetic differences between patients with primary and secondary Sjogren’s syndrome. Numerous other poten- tial symptoms may occur and include interstitial pneumonitis, dryness of the skin, Raynaud’s phenomenon, achlorhydria, hepatosplenomegaly genital dryness, hyposthenuria, myositis, and pancreatitis. Patients with primary Sjogren’s syndrome have a greater incidence of recurrent paro- titis, Raynaud’s phenomenon, purpura, lymphadenopathy, myositis, and renal involvement than those with secondary Sjogren’s syndrome. Sev- enty-five percent show evidence of respiratory involvement, usually dif- fuse interstitial disease or small airway disease.

A variety of laboratory findings suggest that one of the underlying defects in Sjogren’s syndrome is B-cell hyperactivity with or without ab- normalities of immunoregulation. This is manifest by a polyclonal hyper- gammaglobulinemia, numerous autoimmune antibodies, and circulating IgG immune complexes.’ Between 70% and 90% of the patients demon- strate rheumatoid factor, and 55% to 70% demonstrate antinuclear anti- bodies. There is a more detailed description in the article on Sjogren’s syndrome.

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References

1. Alspaugh MA, Tolal N, Ton E M Differentiation and characterization of autoantibodies

2. Bars JW, Vincent JM, Person DA: The expanding spectrum of Bartonella infections: I1 Cat-

3. Batsakis JG, Sylvest V: Pathology of the Salivary Glands. Chicago, American Society of

4. Casterline PF, Jaques DP: The surgical management of recurrent parotitis. Surg Gynecol

5. Hammer JE, Goefield HH: Cervical lymphadenopathy and parotid gland swelling in sar-

6. Moutsopoulos HM: Sjogren‘s syndrome (sicca syndrome): Current issues. Ann InternMed

7. Shearn MA: Sjogren’s syndrome. Med Clin North Am 61:271,1977 8. Sjogren H Zur Kerntnis der keratoconjunctivitis sicca (Keratitis filiforms bei hysfunktion

9. Wang ML, Jafek BM: Cervical mycobacterial disease. Transactions of the American Acad-

and their antigens in Sjogren’s syndrome. Arthritis Rheum 19216,1976

scratch disease. Pediatr Infect Dis J 16163,1997

Clinical Pathologists, 1977

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coidosis: A study of 31 cases. J Am Dent Assoc 74:1224,1967

92:212,1980

der tranendrusen). Acta Ophthalmolll:l, 1933

emy of Ophthalmology and Otolaryngology 78:75, 1974

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Department of Otolaryngology-Head and Neck Surgery University of Southern California

1200 North State Street, Box 795 Los Angeles, CA 90033