Primary Sjogren syndrome diagnosed simultaneously with localized amyloidosis of the lacrimal gland

Abstract

Introduction:Amyloidosis accompanied by Sjögren's syndrome (SS) has been reported to occur primarily in the skin, lungs, tongue, and mammary gland. However, SS in association with secondary amyloidosis is rarely reported, and knowledge of its relevance is inadequate. Here we report a case of primary SS diagnosed simultaneously with localized amyloidosis of the lacrimal gland.Case presentation:A 45-year-old woman complaining of a left eyelid mass was referred to the hospital and was diagnosed with localized amyloidosis after excisional biopsy. She was then referred to the rheumatology department for additional evaluation for amyloidosis. Subsequently, her diagnosis was primary SS based on the presented symptoms and results of the Schirmer test, serologic testing, and minor salivary gland biopsy. Pilocarpine (10 mg/d) and hydroxychloroquine (200 mg/d) were initiated for the treatment of SS. Six months after the initial diagnosis, the dry eyes and mouth did not worsen and no masses suggestive of localized amyloidosis were reported.Conclusion:This is a rare case of amyloidosis, localized to the lacrimal gland, with SS. Therefore, despite its rarity, physicians should be aware of the potential coexistence of secondary amyloidosis, even in the localized form, in patients with SS.

A Rare Case of Renal AA Amyloidosis Secondary to Sjogren’s Syndrome

“…In cases where amyloidosis accompanies SS, amyloidosis is diagnosed 1–25 years after the development of inflammation, during the course of SS, although in some cases these conditions have been diagnosed simultaneously. This may be the result of delayed diagnosis of SS in patients who previously had only mild or no symptoms associated with the disease [8] , which seems to be the case in our patient.…”

“…Amyloidosis in patients with primary SS is rare [1, 8] . The risk of developing AA amyloidosis is related to continuous, subclinical inflammation and, consequently to the magnitude and persistence of elevated AA amyloid levels [1] .…”

“…Additionally, chronic B-cell activation is responsible for the more severe complication of primary SS (2–9%) as a result of B-cell non-Hodgkin’s lymphoma, which may rarely involve the kidney [4] . Although AA amyloidosis is associated with aetiologies such as rheumatoid arthritis and ankylosing spondylitis [5–9] , the association between SS and amyloidosis is not well established [8] .…”

AA Amyloidosis Secondary to Primary Sjögren Syndrome: Can It Be Developed without Chronic Inflammation?

“…When associated to PSS, amyloidosis is diagnosed 1 to 25 years after the diagnosis of the disease, although in some cases they were diagnosed simultaneously. This may be the consequence of a delayed diagnosis of PSS in patients who previously had only mild symptoms related to the disease [10], which seems to be the case among these patients. Despite being a common complication of chronic inflammatory disease, few studies have evaluated the natural history, prognostic markers and treatment of AA amyloidosis [11].…”

Lacrimal Gland Amyloidosis in an Elderly Patient

“…The patient described in this report was being treated for sarcoidosis uveitis. Since there have been reports of lacrimal amyloidosis complicated by systemic diseases such as Sjögren's syndrome, sarcoidosis, and multiple myeloma [9,10], the possibility of systemic amyloidosis was also considered. Several studies have reported that amyloidosis can be complicated by sarcoidosis [11][12][13][14][15][16].…”