| Literature DB >> 30887439 |
Jason Hom1,2, Shruti Marwaha3,4, Anna Postolova5, Jessie Kittle6, Rosaline Vasquez7, Jean Davidson3, Jennefer Kohler3, Annika Dries3, Liliana Fernandez-Betancourt3, Marta Majcherska3, Joanna Dearlove8, Shyam Raghavan9, Hannes Vogel9, Jonathan A Bernstein3,10, Paul Fisher3,11, Euan Ashley3,4, Jacinda Sampson3,8, Matthew Wheeler3,4.
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.Entities:
Keywords: Sjogren’s syndrome; amyloidosis; immunophenotyping; inclusion body myositis
Year: 2019 PMID: 30887439 PMCID: PMC6544676 DOI: 10.1007/s11606-019-04931-w
Source DB: PubMed Journal: J Gen Intern Med ISSN: 0884-8734 Impact factor: 5.128