TITLE:
AA Amyloidosis Secondary to Primary Sjögren Syndrome: Can It Be Developed without Chronic Inflammation?
AUTHORS:
Soukaina Zaher, Kawtar Nassar, Ibtissam Razzouki, Meriem Regragui, Mehdi Karkouri, Saadia Janani
KEYWORDS:
AA Amyloidosis, Amyloid, Primary Sjögren Syndrome
JOURNAL NAME:
Open Journal of Rheumatology and Autoimmune Diseases,
Vol.11 No.2,
April
22,
2021
ABSTRACT: Background: The association of primary Sjögren syndrome (PSS) and AA amyloidosis is a rare occurrence. Objective: To describe the phenotype of patients with this association through our two cases and a literature review. Materials and methods: A report of two cases of AA amyloidosis complicating primary Sjögren syndrome with a literature review. Results: Eight patients of Primary Sjögren’s Syndrome complicated by AA amyloidosis were studies. Six cases were reported in the literature by consulting several databases. 50% of patients had a positive immunological assessment, three cases with kidney damage, and three cases lung damage. Conclusion: The immunological activity in the Primary Sjogren’s Syndrome requires the search not only a lymphoma but also AA amyloidosis apart from any clinical or biological chronic inflammation.