P4859 - A Case of Hepatocellular Injury Caused by Anti-3-Hydroxy-3-Methylglutaryl Coenzyme A Reductase (HMGCR) Myopathy

Gopala Koneru, MBBS, Yasameen Muzahim, MD, Tomohiro Tanaka, MD, MPH, Matthew Gosse, MD
University of Iowa Hospitals & Clinics, Iowa City, IA

Introduction: Anti-HMGCR myopathy, rare at 0.9 to 1.4 cases per 100,000, can manifest even in statin-exposed patients not currently using the medication. We detail a case of a 61-year-old male with progressively abnormal liver enzymes, eventually diagnosed with Anti-HMGCR myopathy due to statin exposure 3 years prior.

Case Description/Methods: Previously healthy patient developed abdominal pain in January 2022, accompanied by abnormal liver enzymes and portal vein thrombosis (PVT) on imaging. Investigations for common chronic liver conditions as well as hypercoagulable workup was negative, and anticoagulation was initiated. Soon after, he presented with fevers, abdominal pain, and continued liver enzyme abnormalities. Magnetic Resonance Imaging with contrast revealed known unchanged PVT and new multiple liver abscesses. The abscesses were managed with antibiotics and Computed Tomography-guided drainage of largest lesion, leading to resolution. Initial labs in 2022: Alkaline Phosphatase (ALP) 169, Total Bilirubin 1.2, Alanine Transaminase (ALT) 141, Aspartate Transferase (AST) 65, attributed to liver abscesses. Liver enzymes persisted and worsened a year later (ALT 358, AST 214, total bilirubin 0.5, ALP 89), leading to referral to tertiary Hepatology clinic. Extensive investigations ruled out common liver pathologies such as viral and autoimmune diseases. Trans jugular liver biopsy showed nonspecific minimal portal chronic inflammation (figure 1). Patient later developed myalgias, rash, and weakness, with creatine kinase (CK) level at 11,301. Despite no statin exposure in the last 3 years, MRI of bilateral thighs during hospitalization revealed bilateral thigh muscle edema, suggestive of inflammatory myopathy. High-dose steroids were initiated. Neurology consultation confirmed proximal myositis with Electromyography (EMG) and highly positive Anti-HMG Co reductase antibody, diagnosing anti-HMG Co A reductase antibody myopathy. Treatment by Rheumatology included high-dose steroids, Intravenous Immunoglobulin, and mycophenolate.




Discussion: This case highlights the significance of considering extrahepatic causes of abnormal liver enzymes and early recognition of anti-HMGCR myopathy. Despite its rarity, this condition, stemming from common drug exposure, carries substantial morbidity and often necessitates prolonged immunosuppressive therapy, as it frequently relapses and does not resolve with statin discontinuation.



Disclosures:


Gopala Koneru, MBBS, Yasameen Muzahim, MD, Tomohiro Tanaka, MD, MPH, Matthew Gosse, MD. P4859 - A Case of Hepatocellular Injury Caused by Anti-3-Hydroxy-3-Methylglutaryl Coenzyme A Reductase (HMGCR) Myopathy, ACG 2024 Annual Scientific Meeting Abstracts. Philadelphia, PA: American College of Gastroenterology.