Dysphagia as the Initial Manifestation of Anti‐HMGCR Antibody–Positive Immune‐Mediated Necrotizing Myopathy Complicated by Pulmonary Embolism
6 affiliations
- 1Department of Family Medicine Mayo Clinic Health System Eau Claire Wisconsin USA mayoclinichealthsystem.org
- 2Department of Rheumatology Mayo Clinic Health System Eau Claire Wisconsin USA mayoclinichealthsystem.org
- 3Department of Hospital Internal Medicine Mayo Clinic Health System Eau Claire Wisconsin USA mayoclinichealthsystem.org
- 4Department of Pulmonary and Critical Care Medicine Mayo Clinic Health System Eau Claire Wisconsin USA mayoclinichealthsystem.org
- 5Department of Neurology Mayo Clinic Rochester Minnesota USA mayo.edu
- 6Department of Radiology Mayo Clinic Health System Eau Claire Wisconsin USA mayoclinichealthsystem.org
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.
Related
- Necrotizing Myositis as a Rare Presenting Manifestation of Childhood-Onset Granulomatosis With Polyangiitis: A Case Report.
- Diabetic Ketoacidosis Triggered by Retropharyngeal Abscess Complicated by Descending Necrotizing Mediastinitis
- Case Report: Pediatric group A streptococcal toxic shock syndrome complicated by acute necrotizing tracheobronchitis requiring veno-venous extracorporeal membrane oxygenation
- A case of recurrent membranous nephropathy with multiple necrotizing pulmonary nodules
- Isolated necrotizing scleritis revealing silent pulmonary granulomatosis with polyangiitis : A case report of rare diagnostic clue
BibTeX
@article{kalluri2026dysphagia,
title = {{Dysphagia as the Initial Manifestation of Anti‐HMGCR Antibody–Positive Immune‐Mediated Necrotizing Myopathy Complicated by Pulmonary Embolism}},
author = {Rohini Kalluri and Elena Ciofoaia and Omobolanle Adetimehin and Aryan Shiari and Igor Dumic and Margherita Milone and Juan Vera Gomez and Erik Sviggum and Celestine Odenigbo and Cristian Madrid and Charles W. Nordstrom},
journal = {Case Reports in Rheumatology},
year = {2026},
month = nov,
doi = {10.1155/crrh/2235175},
url = {https://doi.org/10.1155/crrh/2235175},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - Dysphagia as the Initial Manifestation of Anti‐HMGCR Antibody–Positive Immune‐Mediated Necrotizing Myopathy Complicated by Pulmonary Embolism
AU - Rohini Kalluri
AU - Elena Ciofoaia
AU - Omobolanle Adetimehin
AU - Aryan Shiari
AU - Igor Dumic
AU - Margherita Milone
AU - Juan Vera Gomez
AU - Erik Sviggum
AU - Celestine Odenigbo
AU - Cristian Madrid
AU - Charles W. Nordstrom
PY - 2026
DA - 2026/11/03
JO - Case Reports in Rheumatology
DO - 10.1155/crrh/2235175
UR - https://doi.org/10.1155/crrh/2235175
AB - 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.
SN - 2090-6889
SN - 2090-6897
ER -