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Parotid Sarcoidosis

last modified on: Tue, 04/16/2024 - 09:00

​see also: Salivary Swelling; Sarcoidosis

Note: last updated before 2013

Sarcoidosis is a multi-system disease of unknown etiology, characteristically causing bilateral hilar lymphadenopathy and granulomatous lesions of the lungs, eyes, or skin (Surattanont et al. 2002, Iannuzzi et al. 2007). Parotid sarcoidosis is not well-known and often goes undiagnosed (Funk et al. 2009). The parotid gland is rarely affected, and sarcoidosis of the parotid gland is rarely the primary manifestation (Sinha et al. 2004).

Parotid sarcoidosis is presented as a persistent asymptomatic or painful, diffuse, non-nodular parotid swelling (Iko et al. 1986, Sinha et al. 2004). Sarcoidosis of the parotid gland is more often bilateral, with onset slightly more common in women in their third or fourth decades (Greenberg et al. 1964, James et al. 2000, Sinha et al. 2004). It also demonstrates widespread involvement of other tissue systems, particularly the lungs, lymph nodes, spleen, uveal tract, and lacrimal glands (Greenberg et al. 1964, James et al. 2000). A pathognomonic pattern of sarcoidosis involving the parotid gland is also seen in Heerfordt’s syndrome, a triad of symptoms including bilateral parotid swelling, uveitis, and cranial nerve involvement that is usually manifested as a transient facial nerve paralysis (Surattanont et al. 2002, James et al. 2000, Sinha et al. 2004).

There are significant variations in patient presentation. Parotid enlargement subside within 6 months in some patients, while others report varying periods of swelling and enlargement lasting up to 3 years (Greenberg et al. 1964).

Definitive diagnosis of parotid sarcoidosis is cumulatively based on the patient’s medical history, clinical examination, and the presence of non-caseating granulomas (Surattanont et al. 2002). An accurate radiological study is essential for further assessment. Because the parotid glands are superficially located structures, high-frequency ultrasound evaluation is a first-line diagnostic modality (Teymoortash et al. 2009). Other imaging modalities such as sialography or computed tomography are useful options.

The majority of patients diagnosed with sarcoidosis are not disabled by the illness, and sarcoidosis of the parotid gland has shown to be self-limited in many (Iannuzzi et al. 2007). The decision to provide treatment should thus be weighted for the risks and benefits that may follow (Iannuzzi et al. 2007).

The conventional therapy of choice for sarcoidosis is either no treatment at all, due to its spontaneous remission, or corticosteroids. The use of corticosteroids is the most common medical therapy when organ symptomatology is severe (Mandel et al. 2005). To avoid steroid toxicity, other steroid-sparing or steroid-substituting agents may be therapeutic: methotrexate, azathioprine, cyclosporine, or hydroxychloroquine (Mandel et al. 2005). However, corticosteroid therapy demonstrated no clear-cut benefit for patients of persistent, chronic sarcoidosis of the parotid gland (Iko et al. 1986).

Patients that do not respond to steroid therapy may be responsive to infliximab, a monoclonal antibody against tumor necrosis factor-alpha (TNF-α) (Mandel et al. 2005). The level of circulating TNF-α is increased in patients of sarcoidosis and is also found in the sarcoid nodules (Mandel et al. 2005). Infiximab acts to block the interaction between TFN-α and its receptor binding sites.

Parotid gland swelling is not an uncommon chief complaint, but the consideration of sarcoidosis in its differential diagnosis is not common. The involvement of sarcoidosis in parotid glands is extremely rare. Moreover, it is even rarer for parotid glands to be the primary manifestation site of sarcoidosis. Knowledge of the clinical symptomatology of sarcoidosis and the presence of bilateral parotid gland swelling could be a significant diagnostic clue (Mandel et al. 2005).

References

Surattanont F, Mandel L, Wolinsky B. Bilateral parotid swelling caused by sarcoidosis. J Am Dent Assoc. 2002;133:738-741.

Iannuzzi MC, Rybicki BA, Teirstein AS. Sarcoidosis. New Engl J Med. 2007;357:2153-2165.

Iko BO, Chinwuba CE, Myers EM, Teal JS. Sarcoidosis of the parotid gland. Br J Radiol. 1986;59:547-552.

Greenberg G, Anderson R, Sharpstone P, James DG. Enlargement of parotid gland due to sarcoidosis. Brit Med J. 1964;2:861-862.

James DG, Sharma OP. Parotid gland sarcoidosis. Sarcoidosis Vasc Dif. 2000;17(1):27-32.

Funk M, Santana O. An unusual presentation of an uncommon disease. Int J Gen Med. 2009;2:259-261.

Sinha R, Gaur SN. Sarcoidosis presenting as acute bilateral parotid swelling. Asian Pac J Allergy. 2004;22:171-174.

Teymoortash A, Werner JA. Parotid gland involvement in sarcoidosis: sonographic features. J Clin Ultrasound. 2009;37(9):507-510.

Mandel L, Wolinsky B, Chalom EC. Treatment of refractory sarcoidal parotid gland swelling in a previously reported unresponsive case. J Am Dent Assoc. 2005;136:1282-1285.