Sicca syndrome due to primary amyloidosis

Abstract

Sicca syndrome consists of two major clinical findings: keratoconjunctivitis sicca and xerostomia due to destruction of the lacrimal and salivary gland parenchyma. Although it is most often due to Sjögren's syndrome, a variety of other diseases causes sicca syndrome. We report the rare case of a patient with gland infiltration in primary amyloidosis. Sonographic, computed tomographic and magnetic resonance findings are presented.

Primary Localized Nodular Tongue Amyloidosis Associated with Sjögren’s Syndrome

“…Several cases with primary amyloidosis presenting sicca syndrome have been reported previously [1][2][3][4][5][6][7][8][9][10][11], In these cases, the cause of sicca syndrome was destruction of sali vary glands by amyloid deposition, and chronic inflamma tory cell infiltration was not prominent histologically. The present case is apparently different from those.…”

Lacrimal Gland Amyloidosis: A Clinicopathological Correlation of a Rare Disorder and Review of Literature

“…[1][2][3][4][5][6][7][8][9][10][11][12][13][14][15] Localized amyloidosis is an infrequently encountered disease but has an affinity to mucous membranes like the conjunctiva. 8,10,13 Ocular amyloidosis is most commonly seen involving the conjunctiva and the lid.…”

“…[1][2][3][4][5][6][7][8] This deposition may be secondary to a systemic disease or occur as primary amyloidosis in absence of a systemic disease. Amyloid deposition can be localized to a single site or may occur as generalized deposits in multiple tissues.…”

“…Primary, localized orbital amyloidosis is uncommon and accounts for around 4% of localized amyloidosis of the head and neck region. [1][2][3][4][5][6][7][8][9][10] Lids and conjunctiva are usual sites of involvement commonly associated with a systemic disease. Lacrimal gland involvement is rare and, to our knowledge, there are only 18 previously reported cases of lacrimal gland amyloidosis.…”

Amyloid and the Gut

“…The tongue may be very vascular, leading to hem orrhage, and the troublesome effects of mac roglossia may be exacerbated by xerostomia [24], Keratoconjunctivitis sicca may also be present [25], with or without enlargement of the salivary and lacrimal glands. …”