Work

Dysphagia as the Initial Manifestation of Anti‐HMGCR Antibody–Positive Immune‐Mediated Necrotizing Myopathy Complicated by Pulmonary Embolism

Year: 2026

Type: article

Abstract: Background: Anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (anti-HMGCR) antibody-positive immune-mediated necrotizing myopathy (IMNM) is a rare autoimmune myopathy characterized by progressive proximal muscle weakness and markedly elevated creatine kinase levels. Although commonly associated with prior statin exposure, statin-naïve cases and atypical presentations are well described. Patients with idiopathic inflammatory myopathies are also at increased risk for venous thromboembolism (VTE), though data specific to IMNM remain limited. Case Presentation: An 83-year-old man with brief, remote statin exposure presented with progressive proximal muscle weakness and a three-year history of oropharyngeal dysphagia preceding limb involvement. Laboratory evaluation revealed markedly elevated creatine kinase and transaminases. Anti-HMGCR antibodies were positive, and muscle biopsy confirmed necrotizing myopathy. During evaluation for occult malignancy, bilateral pulmonary emboli were incidentally identified. The patient required high-dose corticosteroids, mycophenolate mofetil, and intravenous immunoglobulin for refractory disease. With aggressive immunosuppressive therapy and rehabilitation, he demonstrated substantial clinical recovery at 6 months. Conclusion: This case highlights oropharyngeal dysphagia as an early and, for a prolonged period, isolated manifestation of anti-HMGCR antibody-positive IMNM; here, it preceded the onset of limb weakness by approximately 3 years. It underscores the importance of comprehensive diagnostic evaluation in patients with unexplained oropharyngeal dysfunction, in whom an inflammatory myopathy may otherwise be overlooked for years. Clinicians should maintain awareness of thrombotic risk in inflammatory myopathies, particularly during active disease. Early recognition and prompt immunosuppressive therapy remain critical to optimize outcomes and prevent irreversible muscle damage.

Source: Case Reports in Rheumatology

DOI: 10.1155/crrh/2235175