Anti-signal Recognition Particle Antibody-positive Necrotizing Myopathy with Secondary Cardiomyopathy: The First Myocardial Biopsy- and Multimodal Imaging-proven Case

• Published in: Internal Medicine Vol. 58; no. 21; pp. 3189 - 3194
• Main Authors: Takeguchi-Kikuchi, Shiori, Hayasaka, Taiki, Katayama, Takayuki, Kano, Kohei, Takahashi, Kae, Saito, Tsukasa, Sawada, Jun, Minoshima, Akiho, Sakamoto, Naka, Akasaka, Kazumi, Miyokawa, Naoyuki, Nishino, Ichizo, Ishibashi-Ueda, Hatsue, Hasebe, Naoyuki
• Format: Journal Article
• Language: English
• Published: Japan The Japanese Society of Internal Medicine 01.11.2019; Japan Science and Technology Agency
• Subjects: Aged; anti-signal recognition particle (SRP) antibody; Autoantibodies - blood; Biopsy; Cardiomyopathies - diagnostic imaging; Cardiomyopathies - etiology; Cardiomyopathy; Case Report; Contrast Media; Creatine; Creatine kinase; Dyslipidemia; Dysphagia; Echocardiography; Female; Gadolinium; Humans; Immunoglobulins; Immunoglobulins, Intravenous - therapeutic use; Immunosuppressive Agents - therapeutic use; Inflammation; Internal medicine; Intravenous administration; Magnetic Resonance Imaging; Multimodal Imaging; Muscle Weakness - pathology; Muscle, Skeletal - pathology; Muscles; Muscular Diseases - complications; Muscular Diseases - diagnostic imaging; Muscular Diseases - pathology; myocardial biopsy; Myopathy; necrotizing myopathy; Positron emission tomography; Positron Emission Tomography Computed Tomography; positron-emission tomography (PET); Signal recognition particle; Signal Recognition Particle - immunology; Steroids; Tacrolimus; Tacrolimus - therapeutic use; Ventricle; positron-emission tomography (PET); myocardial biopsy; cardiomyopathy; necrotizing myopathy; anti-signal recognition particle (SRP) antibody
• ISSN: 0918-2918; 1349-7235
• DOI: 10.2169/internalmedicine.2564-18

A 69-year-old Japanese woman was admitted to our hospital with progressive muscle weakness and dysphagia. She was taking pitavastatin for dyslipidemia. Her serum creatine kinase was 6,300 U/L. Pitavastatin was stopped, but her symptoms deteriorated, and cardiac congestion appeared. A muscle biopsy showed necrotizing myopathy (NM), and anti-signal recognition particle (SRP) antibody was positive. 18F-fluorodeoxyglucose-positron emission tomography showed an abnormal uptake, and magnetic resonance imaging showed abnormal gadolinium enhancement in the left ventricular wall. An endomyocardial biopsy revealed inflammatory cardiomyopathy. Steroid, tacrolimus, and intravenous immunoglobulins were effective against the symptoms. This is the first case of biopsy-proven secondary cardiomyopathy due to anti-SRP-positive NM.