For the 2023 Image Competition, the ACR sought images representing a diverse range of patients with idiopathic inflammatory myopathies (IIMs) or IIM mimics. The images selected as Best Overall by the judges this year come from North America.
Systemic Light Chain (AL) Amyloidosis Masquerading as Inflammatory Myositis
These images depict an 81-year-old man who presented with a two-year history of progressive dysphagia, which was associated with weight loss and generalized weakness. He had periorbital hyperpigmentation (A) and prominent submandibular glands (B). His creatine kinase was elevated, and electromyography showed an irritable myopathy. Muscle biopsy demonstrated severe neurogenic atrophy without obvious myositis. Small and large perimysial and epimysial vessels showed glassy, pink thickening approaching nodularity in areas. The material was strongly positive for Congo red (C) and exhibited the characteristic birefringence of amyloid on polarization (D). Mass spectroscopy analysis demonstrated the amyloid material was light chain in origin.
There are more than 30 amyloid precursor proteins, which, in healthy individuals, remain soluble. When proteins, such as the kappa or lambda light chain or transthyretin, become insoluble, they can cause amyloidosis. For more information on the condition, refer to articles in The Rheumatologist’s archives:
- Amyloidosis Is Often Underdiagnosed, Undertreated; and
- What Rheumatologists Need to Know about Identifying Amyloidosis.
Mary Purcell, MD, received her degree from Dalhousie University, Nova Scotia, Canada, where she also
completed her residency in rheumatology.
About the Contest
The Rheumatology Image Library is a highly accessed teaching resource and images from the ACR’s annual contest are added to it, keeping the library dynamic and current. In addition to the Best Overall Image in 2023, winning images were selected from four regions around the world. Look for the People’s Choice and regional winners in future issues and on our website.