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September 2, 2026European Heart Journal - Case ReportsOpen Access

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

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Key result

Anti-Ku myositis linked to fatal inflammatory myocarditis with LVEF declining to 12% despite aggressive immunosuppression.

  • n=1

Population

A 60-year-old man with anti-Ku myositis presenting with progressive inflammatory myocarditis

Design

Case_report

Follow-up

8 months

Authors

AEAhmed ELBekieyMZMohamed Wahib ZhlawiJTJie Tong

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Overview

Highlights potential fatality in anti-Ku myocarditis despite immunosuppression; leaves open role of early antibody screening.

Key Points

  • To describe a fatal presentation of anti-Ku overlap myositis masquerading as isolated cardiac disease and identify clinical pitfalls in diagnosis and management.
  • Clinical, diagnostic, and therapeutic evaluation of a 60-year-old male presenting with progressive heart failure and subsequent proximal myopathy.
  • Diagnostic procedures included serial echocardiography, coronary angiography, cardiac MRI, deltoid muscle biopsy, and myositis-specific antibody immunoblotting.
  • Left-ventricular ejection fraction progressively declined from an initial 49% to 12%, complicated by high-grade atrioventricular block requiring dual-chamber pacing.
  • Immunoblot confirmed high-titre anti-Ku antibodies and deltoid biopsy showed inflammatory myopathy, alongside serum creatine kinase of 2,680 IU/L and troponin I of 928 ng/L.
  • Despite combination immunosuppression with corticosteroids, mycophenolate, cyclophosphamide, and intravenous immunoglobulin, cardiac failure proved refractory, resulting in death at eight months.

Study Design

Type

Case Report (n=1)

Structured PICO

P
Population
A 60-year-old man with anti-Ku myositis presenting with progressive inflammatory myocarditis and heart failure, followed for 8 months until death.
E
Exposure
High-dose corticosteroids, mycophenolate, cyclophosphamide, and intravenous immunoglobulin
O
Outcome
Clinical course and survival

Anti-Ku myositis can present with aggressive cardiac involvement and may be refractory to conventional immunosuppression once advanced cardiomyopathy is established.

Cite This Study

ELBekiey et al. (2026) conducted a case report in Anti-Ku myositis with progressive inflammatory myocarditis (n=1). Anti-Ku myositis was evaluated on Cardiac function and survival. Anti-Ku myositis in a 60-year-old man led to fatal progressive inflammatory myocarditis, with left ventricular ejection fraction declining from 49% to 12% despite aggressive immunosuppression.

synapsesocial.com/papers/6a97e2eac562ede874ec7441https://doi.org/10.1093/ehjcr/ytag625
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