Localized Breast Amyloidosis Associated with Sjörgren Syndrome

Case Reports in Pathology
Edgar G Fischer

Abstract

Sjörgren syndrome is a systemic autoimmune disease that is rarely associated with amyloid deposits, and in most reported cases, these deposits are localized to a single organ. Amyloidosis of the breast is a rare and unexpected finding, and only 5 case series with 63 patients have been published in the past 40 years. To date, only 6 cases have been reported in which Sjörgren syndrome is associated with amyloid deposits in the breast. A 61-year-old female diagnosed with Sjörgren syndrome underwent a breast needle core biopsy for calcifications. Microscopic examination revealed amyloid deposits in the periductular basement membranes, in the walls of arteries and veins, and in the surrounding connective tissue. No malignancy was found. Clinical workup revealed the amyloid deposits to be localized to the breast and did not reveal an underlying hematolymphoid neoplasm. The association between Sjörgren syndrome and breast amyloidosis is rare, but few reports have appeared in recent years, and it may be an emerging disease association. The finding of localized amyloid in the breast and other organs should lead to a clinical workup not only for hematopoietic neoplasms but also for autoimmune diseases such as Sjörgren syndrome.

References

May 22, 2002·Virchows Archiv : an International Journal of Pathology·Christoph RöckenRoland Bässler
Apr 20, 2011·Amyloid : the International Journal of Experimental and Clinical Investigation : the Official Journal of the International Society of Amyloidosis·Marjory CharlotVaishali Sanchorawala
Sep 29, 2012·Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc·Samar M SaidSejal S Shah
Oct 30, 2015·Pathobiology : Journal of Immunopathology, Molecular and Cellular Biology·Laura HerreroPedro Luis Fernández
Jul 8, 2016·Nature Reviews. Disease Primers·Pilar Brito-ZerónManuel Ramos-Casals
Jan 5, 2017·Current Rheumatology Reviews·Gabriela Hernandez-MolinaEdgardo Reyes
Feb 21, 2019·Surgical Case Reports·Makiko MoriHiroji Iwata
Nov 12, 2019·Pathology, Research and Practice·Andrew LytleUgur Ozerdem

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biopsy

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