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Kawasaki Disease: Past, Present and Future

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Systemic Vasculitides: Current Status and Perspectives

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Abstract

Forty eight years ago Doctor Tomisaku Kawasaki firstly described a disease called “Acute febrile muco-cutaneous lymph node syndrome”, after labeled as Kawasaki disease (KD). It still remains a mysterious illness affecting coronaries in a quarter of untreated patients and is the most common cause of childhood-acquired heart disease in industrialized countries. Many gaps exist in our knowledge in the etiology and pathogenesis of KD. Numerous KD features still demand further efforts to achieve a better understanding of the disease. Some of these issues include coronaries’ injuries in children not fulfilling the classical diagnostic criteria, genetic predisposition, unpredictable ineffectiveness of current therapy in some cases, vascular dysfunction in patients not showing echocardiographic evidence of coronaries abnormalities in the acute phase and risk of potential premature atherosclerosis. The lack of specific laboratory tests for early identification of the atypical and incomplete cases, especially in infants, is one of the obstacles to treat early patients in order to reduce cardiovascular involvement. Transthoracic echocardiography remains the gold-standard for evaluation of coronaries in the acute phase and follow-up. In patients with severe vascular complications, more expensive and invasive investigations, such as coronary CT angiography and MRI, may be required. As children with KD grow-up, the acknowledgment and treatment of the potential sequelae become critical, requiring rheumatologists, cardiologists and infectious disease specialists cooperate to develop guidelines for a proper evaluation and management of patients. Deep education is recommended for physicians and other professionals about how to recognize the long-term impact of systemic problems related to KD.

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Author information

Authors and Affiliations

  1. Department of Clinical and Experimental Medicine, Section of Rheumatology, Transition Clinic, University of Florence, Florence, Italy

    Fernanda Falcini & Gemma Lepri

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  1. Fernanda Falcini
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  2. Gemma Lepri
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Correspondence to Fernanda Falcini .

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Editors and Affiliations

  1. University of Bari Medical School, Bari, Italy

    Franco Dammacco

  2. University of Bari Medical School, Bari, Italy

    Domenico Ribatti

  3. University of Bari Medical School, Bari, Italy

    Angelo Vacca

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Falcini, F., Lepri, G. (2016). Kawasaki Disease: Past, Present and Future. In: Dammacco, F., Ribatti, D., Vacca, A. (eds) Systemic Vasculitides: Current Status and Perspectives. Springer, Cham. https://doi.org/10.1007/978-3-319-40136-2_15

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