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A Patient with Sjogren's Syndrome and Subsequent Diagnosis of Inclusion Body Myositis and Light-Chain Amyloidosis

Authors :
Rosaline Vasquez
Jonathan A. Bernstein
Jacinda B. Sampson
Jason Hom
Jean M. Davidson
Jennefer N. Kohler
Annika M. Dries
Jessie Kittle
Hannes Vogel
Liliana Fernandez-Betancourt
Joanna Dearlove
Euan A. Ashley
Matthew T. Wheeler
Anna Postolova
Shyam S. Raghavan
Paul G. Fisher
Marta M. Majcherska
Shruti Marwaha
Source :
J Gen Intern Med
Publication Year :
2017

Abstract

We discuss a challenging case of a 58-year-old Vietnamese-American woman who presented to her new primary care provider with an 8-year history of slowly progressive dysphagia, hoarseness, muscle weakness with associated frequent falls, and weight loss. She eventually reported dry eyes and dry mouth, and she was diagnosed with Sjogren’s syndrome. Subsequently, she was additionally diagnosed with inclusion body myositis and gastric light-chain (AL) amyloidosis. Although inclusion body myositis has been previously associated with Sjogren’s syndrome, inclusion body myositis is rare in non-Caucasians, and the trio of Sjogren’s syndrome, inclusion body myositis, and AL amyloidosis has not been previously reported. Sjogren’s syndrome is a systemic autoimmune condition characterized by ocular and oral dryness. It is one of the most common rheumatologic disorders in the USA and worldwide. Early diagnosis of Sjogren’s is particularly important given the frequency and variety of associated autoimmune diseases and extraglandular manifestations. Furthermore, although inclusion body myositis has a low prevalence, it is the most common inflammatory myopathy in older adults and is unfortunately associated with long delays in diagnosis, so knowledge of this disorder is also crucial for practicing internists. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (10.1007/s11606-019-04931-w) contains supplementary material, which is available to authorized users.

Details

ISSN :
15251497
Volume :
34
Issue :
6
Database :
OpenAIRE
Journal :
Journal of general internal medicine
Accession number :
edsair.doi.dedup.....7328c244fcd619ee68e56c4fceb1fa9d