Rare Case of Amyloidosis as Secondary Feature of Sjogren’s Syndrome Described in Report

Although rare, localized amyloidosis can sometimes appear as a consequence of Sjogren’s syndrome, according to a case report from South Korea.

The study, “Primary Sjogren syndrome diagnosed simultaneously with localized amyloidosis of the lacrimal gland: A case report,” was published in the journal Medicine.

A 45-year-old woman arrived at Pusan National University Hospital in South Korea complaining of a mass in her left eyelid. The mass was later removed and analyzed.

The results confirmed the accumulation of amyloid protein and the patient was diagnosed with amyloidosis, a disease characterized by the buildup of amyloid in organs and tissues throughout the body.

In this case, it was restricted to the lacrimal gland, as no signs of amyloid accumulation were found in other tissues or organs.

The patient was referred to the rheumatology department for additional testing to assess the origin of the amyloid buildup. A salivary gland biopsy showed chronic inflammation accompanied by aggregation of plasma cells, confirming a diagnosis of Sjogren’s syndrome.

She also tested positive in the Schirmer test, which determines whether the tear glands produce enough tears to keep the eyes adequately moist.

The disease was also confirmed according to the American-European consensus criteria for Sjogren’s syndrome, and she tested positive for anti-nuclear autoantibodies.

“We confirmed the diagnosis of the patient, that is, simultaneous localized amyloidosis of the lacrimal gland and primary [Sjogren’s syndrome],” researchers wrote.

In previous studies reporting localized amyloidosis in Sjogren’s syndrome, the accumulation of amyloid was mostly found in the skin and lungs.

“To our knowledge, this is the first case in which amyloidosis with [Sjogren’s syndrome] occurred in the lacrimal gland, but not in other more common sites such as skin and lung,” they added.

The patient was given pilocarpine (10 mg per day), an orally available medicine used to treat symptoms of dry mouth, and hydroxychloroquine (200 mg per day), a treatment for joint pain or stiffness, to treat Sjogren’s syndrome. No therapy was administered for her amyloidosis.

Six months after the initial diagnosis, clinicians didn’t see any worsening in her dry eyes and mouth, and no masses suggestive of localized amyloidosis.

This case report suggests that “despite its rarity, physicians should be aware of the potential coexistence of secondary amyloidosis, even in the localized form, in patients with [Sjogren’s syndrome],” the authors said.