作者
Eli Muchtar, Daniele Derudas, Michelle Mauermann, Teerin Liewluck, Angela Dispenzieri, Shaji K Kumar, David Dingli, Martha Q Lacy, Francis K Buadi, Suzanne R Hayman, Prashant Kapoor, Nelson Leung, Rajshekhar Chakraborty, Wilson Gonsalves, Stephen Russell, John A Lust, Yi Lin, Ronald S Go, Steven Zeldenrust, Robert A Kyle, S Vincent Rajkumar, Morie A Gertz
发表日期
2016/10/1
期刊
Mayo Clinic Proceedings
卷号
91
期号
10
页码范围
1354-1361
出版商
Elsevier
简介
Objective
To characterize the natural history of immunoglobulin light chain amyloidosis–associated myopathy and to provide guidelines for recognition.
Patients and Methods
Fifty-one patients with systemic immunoglobulin light chain amyloidosis and biopsy-confirmed muscle amyloid deposition diagnosed between January 1, 1995, and December 31, 2015, were included in this study.
Results
Common presenting symptoms were muscle weakness in 49 patients (96%), dysphagia in 23 (45%), myalgia in 17 (33%), macroglossia in 17 (33%), jaw claudication in 13 (25%), and hoarseness in 9 (18%). The median time from the onset of symptoms to diagnosis was almost 2 years. Less than two-thirds of the patients with an outside muscle biopsy (16 of 27) had an established pathologic confirmation of amyloidosis due to failure to routinely incorporate Congo red staining. Moreover, 12 patients were incorrectly treated …
学术搜索中的文章
E Muchtar, D Derudas, M Mauermann, T Liewluck… - Mayo Clinic Proceedings, 2016