Anti-Ku Myositis Presenting with Progressive Inflammatory myocarditis: Diagnostic Pitfalls in a Fatal Case: Case Report

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ID: 327169
2026
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Abstract
Abstract Background Anti-Ku antibodies are rare disease of overlap myositis and often signal multisystem involvement. Overt heart failure, however, is seldom reported. Case summary A 60-year-old man presented with progressive exertional dyspnoea and chest tightness. Initial echocardiography showed mildly reduced left-ventricular ejection fraction (LVEF 49%), and coronary angiography was normal, leading to a diagnosis of MINOCA. One month later he re-presented with worsening breathlessness, dysphagia, and proximal muscle weakness. Laboratory tests revealed serum creatine kinase 2,680 IU/L, troponin I 928 ng/L, and pro-BNP 1,049 ng/L. Repeat echocardiography demonstrated dilated ventricles and LVEF 35%. During admission he developed High grade AV dissociation requiring dual-chamber pacing and abrupt thrombocytopenia. Cardiac MRI identified a diffuse infiltrative pattern with LVEF 18%. Myositis-specific immunoblot detected high-titre anti-Ku antibodies, and deltoid biopsy confirmed inflammatory myopathy. Despite high-dose corticosteroids, mycophenolate, cyclophosphamide, and intravenous immunoglobulin, cardiac function deteriorated to LVEF 12%. Recurrent aspiration pneumonia necessitated gastrostomy. Eight months after initial presentation the patient died following transition to palliative care. Conclusion This case illustrates (i) the propensity of anti-Ku myositis for aggressive cardiac involvement, (ii) diagnostic delay when heart disease precedes overt muscle symptoms, and (iii) limited efficacy of conventional immunosuppression once advanced cardiomyopathy is established. Early autoimmune testing—including myositis panels—in patients with unexplained cardiomyopathy plus systemic features may avert missed therapeutic window.
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Authors Ahmed ELBekiey, Mohamed Wahib Zhlawi, Jie Tong, Debashis Roy, Hossam ElGendi
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Journal European Heart Journal - Case Reports
Year 2026
DOI
10.1093/ehjcr/ytag625
URL
https://academic.oup.com/ehjcr/advance-article-pdf/doi/10.1093/ehjcr/ytag625/70877489/ytag625.pdf
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