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CASE REPORT article
Front. Immunol.
Sec. Autoimmune and Autoinflammatory Disorders : Autoimmune Disorders
Volume 16 - 2025 | doi: 10.3389/fimmu.2025.1590913
This article is part of the Research TopicDiseases with Immune Dysregulation in AfricaView all articles
Anti-HMGCR Myopathy: A First Case Report from North Africa and Literature Insights
Provisionally accepted
Houssem Abida1
Imen Zamali1,2,3
Imène Rachdi1,4
Zakaria Saied1,5
Ahlem Ben Hmid1,2,3
SAMAR SAMOUD2,3Yousr Galai2Fatma Daoud1,4Fatma Boussema1,4Samia Ben Sassi1,5Zohra Aydi1,4Mélika Ben Ahmed1,2,3*- 1Faculty of Medicine, Tunis El Manar University, Tunis, Tunis, Tunisia
- 2Department of Clinical Immunology, Pasteur Institute of Tunis, Tunisia, Tunis, Tunisia
- 3Laboratory of Transmission Control and Immunobiology of Infections, Pasteur Institute of Tunis, Tunis, Tunisia
- 4Internal Medicine, Habib Hospital Thameur, Tunis, Tunisia
- 5Neurology, National Institute of Neurology, Tunis, Tunisia
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Anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (anti-HMGCR) myopathy is a rare idiopathic inflammatory myopathy characterized by severe muscle damage and minimal extramuscular involvement. This report presents the first documented case of severe, treatment-resistant HMGCR-myopathy in a Tunisian and North African patient. A 43-year-old man with no significant medical history experienced progressive muscle weakness over one year, leading to difficulty walking. Examination revealed pronounced proximal muscle weakness, particularly in the lower limbs, with significant quadriceps atrophy. Laboratory results indicated elevated Creatine Kinase (CK) levels at 10000 UI/l and Lactate dehydrogenase (LDH) at 400 UI/l. Electromyography confirmed myogenic damage, and muscle biopsy revealed extensive muscle necrosis and regeneration with moderate inflammatory infiltrates. Screening for anti-HMGCR antibodies was positive. Initial treatment with high-dose prednisone showed a good response but led to flares upon tapering. Subsequent treatment with methotrexate, azathioprine, and rituximab resulted in partial clinical and biological improvement.
Keywords: case report, Autoimmunity, Anti-HMGCR, Inflammatory myopathy, Therapeutics
Received: 11 Mar 2025; Accepted: 14 Apr 2025.
Copyright: © 2025 Abida, Zamali, Rachdi, Saied, Ben Hmid, SAMOUD, Galai, Daoud, Boussema, Ben Sassi, Aydi and Ahmed. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Mélika Ben Ahmed, Faculty of Medicine, Tunis El Manar University, Tunis, 1007, Tunis, Tunisia
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