Unexplained recurrent fever: when is autoinflammation the explanation?

Authors

  • T. Kallinich,

    Corresponding author
    1. Department of Pediatric Pneumology and Immunology, Charité University Medicine Berlin, Berlin, Germany
    • Autoinflammation Reference Center Charité (ARC²), Charité University Medicine Berlin, Berlin
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  • M. Gattorno,

    1. UO Pediatria II, G. Gaslini Institute, Genova, Italy
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  • C. E. Grattan,

    1. St John's Institute of Dermatology, St Thomas' Hospital, London, UK
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  • H. D. de Koning,

    1. Department of Dermatology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
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  • C. Traidl-Hoffmann,

    1. Department of Dermatology and Allergy Biederstein, Technische Universität, Munich
    2. ZAUM – Center for Allergy and Environment, Technische Universität Munich/Helmholtz Center, Munich
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  • E. Feist,

    1. Autoinflammation Reference Center Charité (ARC²), Charité University Medicine Berlin, Berlin
    2. Department of Rheumatology and Clinical Immunology, Charité University Medicine Berlin, Berlin, Berlin
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  • K. Krause,

    1. Autoinflammation Reference Center Charité (ARC²), Charité University Medicine Berlin, Berlin
    2. Department of Dermatology and Allergy, Charité University Medicine Berlin, Berlin, Germany
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  • D. Lipsker,

    1. Faculté de Médecine, Université de Strasbourg et Clinique Dermatologique, Hôpitaux universitaires de Strasbourg, Strasbourg, France
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  • A. A. Navarini,

    1. Department of Dermatology, University Hospital of Zurich, Zurich, Switzerland
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  • M. Maurer,

    1. Autoinflammation Reference Center Charité (ARC²), Charité University Medicine Berlin, Berlin
    2. Department of Dermatology and Allergy, Charité University Medicine Berlin, Berlin, Germany
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  • H. J. Lachmann,

    1. National Amyloidosis Centre, University College London Medical School, London, UK
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  • A. Simon

    1. Department of General Internal Medicine, Nijmegen Institute for Infection, Inflammation and Immunology (N4i), Centre for Immunodeficiency and Autoinflammation (NCIA), Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
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  • Edited by: Thomas Bieber

Correspondence

Dr. Tilmann Kallinich, Charité University Medicine Berlin, Pediatric Pneumology and Immunology, Augustenburger Platz 1, 13353 Berlin, Germany.

Tel.: +49-(0)30-450-566182

Fax: +49-(0)30-450-566931

E-mail: tilmann.kallinich@charite.de

Abstract

Recurrent fever can be the sole or leading manifestation of a variety of diseases including malignancies, autoimmune diseases and infections. Because the differential diagnoses are manifold, no formal guidelines for the approach of patients with recurrent fever exists. The newly recognized group of autoinflammatory diseases are often accompanied by repetitive fever attacks. As these episodes are frequently associated by a variety of divergent presentations, the differentiation of other causes for febrile illnesses can be difficult. In this article, we first review disease entities, which frequently present with the symptom of recurrent fever. In a next step, we summarize their characteristic pattern of disease presentation. Finally, we analyse key features of autoinflammatory diseases, which are helpful to distinguish this group of diseases from the other causes of recurrent fever. Recognizing these symptom patterns can provide the crucial clues and, thus, lead to the initiation of targeted specific diagnostic tests and therapies.

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