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

Authors :
Hom, Jason
Marwaha, Shruti
Postolova, Anna
Kittle, Jessie
Vasquez, Rosaline
Davidson, Jean
Kohler, Jennefer
Dries, Annika
Fernandez-Betancourt, Liliana
Majcherska, Marta
Dearlove, Joanna
Raghavan, Shyam
Vogel, Hannes
Bernstein, Jonathan A.
Fisher, Paul
Ashley, Euan
Sampson, Jacinda
Wheeler, Matthew
Undiagnosed Diseases Network
Source :
JGIM: Journal of General Internal Medicine; Jun2019, Vol. 34 Issue 6, p1058-1062, 5p, 3 Color Photographs
Publication Year :
2019

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. [ABSTRACT FROM AUTHOR]

Details

Language :
English
ISSN :
08848734
Volume :
34
Issue :
6
Database :
Complementary Index
Journal :
JGIM: Journal of General Internal Medicine
Publication Type :
Academic Journal
Accession number :
136746690
Full Text :
https://doi.org/10.1007/s11606-019-04931-w