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The heart of anti-Ku related disease: extensive review and meta-analysis. Response to: ‘Anti-Ku syndrome with elevated CK: association with myocardial involvement in systemic sclerosis’ by Campochiaro et al
  1. Lionel Spielmann1,
  2. François Séverac2,3,
  3. Alain Meyer4,5
  1. 1Service de Rhumatologie, Hôpitaux civils de Colmar, Colmar, France
  2. 2Service de Santé Publique, GMRC, CHU de Strasbourg, Strasbourg, France
  3. 3ICube, UMR 7357, équipe IMAGeS, Université de Strasbourg, Strasbourg, France
  4. 4Exploration Fonctionnelle Musculaire, Service de physiologie, Hôpitaux Universitaires de Strasbourg, Strasbourg, France
  5. 5Centre National de Référence des Maladies Auto-Immunes Systémiques Rares de l'Est et du Sud-Ouest, Service de rhumatologie, Hôpitaux Universitaires de Strasbourg, Strasbourg, France
  1. Correspondence to Dr Lionel Spielmann, Service de Rhumatologie, Hospices civils de Colmar, Colmar 3072, France; lionel.spielmann{at}

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We thank Campochiaro et al for their interesting comment1 on our work in which we used hierarchical clustering on principal components to define clinically meaningful subgroups of patients with anti-Ku antibodies.2

Among a bi-centric cohort of patients with systemic sclerosis (SSc), Campochiaro et al identified four patients with anti-Ku and retrospectively reviewed these cases.

All patients had increased creatine kinase (CK), three (75%) of whom had interstitial lung disease (ILD). These findings support our observations according to which anti-Ku patients with elevated CK are at risk of ILD.

Of particular interest, Campochiaro et al proposed that myocarditis could further represent a specific feature of anti-Ku patients with elevated CK given that all of their four anti-Ku SSc patients had cardiac magnetic resonance (CMR) imaging established myocarditis according to Lake Louise criteria. Two (50%) had heart failure while the remaining two had subclinical presentation. By contrast, in our cohort, …

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