A Case of Secondary Amyloidosis in a Patient with Well-Controlled Polymyalgia Rheumatica 1486

• Published in: The American journal of gastroenterology Vol. 113; no. Supplement; p. S853
• Main Authors: Owen, Samuel, Coss, Elizabeth, Klazynski, Brian
• Format: Journal Article
• Language: English
• Published: New York Wolters Kluwer Health Medical Research, Lippincott Williams & Wilkins 01.10.2018
• Subjects: Amyloidosis; Autoimmune diseases; Diarrhea; Disease; Gastroenterology; Steroids
• ISSN: 0002-9270; 1572-0241
• DOI: 10.14309/00000434-201810001-01486

Polymyalgia rheumatica (PMR) is an inflammatory disease that affects the shoulders, pelvic girdles and neck. It is usually found in individuals older than 50 years and is often concurrent with giant cell arteritis. Amyloidosis is a protein deposition disorder that can present in a myriad of ways. Although over twenty different proteins have been identified as causative agents of systemic amyloidosis, one of the best described etiologies is secondary to a persistent inflammatory state such as infection or an autoimmune disease (AA). Very few cases of AA amyloidosis secondary to PMR have been described in literature. We present a case of a patient with PMR on long term corticosteroids who subsequently developed AA amyloidosis of the GI tract presenting as diarrhea, weight loss and malabsorption. A 70 year old female presented to the hospital with four months of watery, non-bloody diarrhea and progressive weight loss. Her past medical was notable for an 18 year history of PMR well controlled on low dose corticosteroids. Laboratory studies were notable for an acute on chronic kidney injury along with a normocytic anemia. Imaging revealed a left sided pleural effusion and infectious work-up for causes of her diarrhea were unrevealing. Esophagogastroduodenoscopy and colonoscopy were significant for a diffuse coarse granularity of the gastric, duodenal and colonic mucosa with mild edema and friability. Perivascular amyloid deposition staining positive for Congo red was seen on biopsies from the stomach and rectum. AA amyloidosis was confirmed with amyloid fibril testing with identical findings seen on a renal biopsy performed during that same admission. Despite rheumatology evaluation no serologic or clinical evidence of an active autoimmune disease was discovered. Secondary AA amyloidosis involving the gastrointestinal tract is a rare disease, and although there have been great advances in treatment options for autoimmune diseases, the role of these new therapies in treating AA amyloidosis is not clearly established. The approach in these patients remains achieving adequate control of the underlying autoimmune disease. However what is notable in our patient is that her disease was quiescent on the current standard of care for PMR. AA amyloidosis developing in patients with PMR has only been described a few times in the literature and often progresses despite use of corticosteroids and clinical resolution of symptoms. Further research in this area is needed.