PMID- 33204972 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20231112 IS - 2514-2119 (Electronic) IS - 2514-2119 (Linking) VI - 4 IP - 5 DP - 2020 Oct TI - Adolescent Kawasaki disease shock syndrome with inflammatory cell infiltration into the myocardium: a case report. PG - 1-7 LID - 10.1093/ehjcr/ytaa304 [doi] AB - BACKGROUND: Kawasaki disease (KD) is a self-limiting form of systemic vasculitis. KD usually occurs in infants and young children and is rarely seen in adolescents. On rare occasions, KD is accompanied with reduced organ perfusion due to systolic hypotension, a condition known as Kawasaki disease shock syndrome (KDSS). The multifactorial causes of KDSS may include intensive vasculitis with capillary leak, myocardial dysfunction, and release of proinflammatory cytokines. However, the mechanisms underlying the pathophysiology of KDSS have not been fully elucidated. CASE SUMMARY: A febrile 17-year-old male with cervical lymphadenopathy developed extreme shock with rapid cardiac dysfunction and reduced organ perfusion. Electrocardiogram revealed ST elevation in the precordial leads and increased serum levels of cardiac enzyme levels. Endomyocardial biopsy at the acute phase revealed CD3(+), CD4(+) or CD8(+), and CD20(-) lymphocytes and CD68(+) macrophages within infiltrates in the myocardium with mild interstitial fibrosis. He was treated with intravenous immunoglobulin (IVIG) and followed by glucocorticoids with mechanical circulatory support. His cardiac function recovered rapidly with no apparent adverse effects. DISCUSSION: Our results suggest that KDSS may be a form of myocarditis, a condition in which inflammatory cells infiltrate the myocardium. Early immunosuppressive therapy, including IVIG and glucocorticoid therapy, may limit the severity of disease and improve the prognosis. As shown by this case, an accurate diagnosis of KD and KDSS will lead to early intervention and improved prognosis even among those in an older cohort. CI - (c) The Author(s) 2020. Published by Oxford University Press on behalf of the European Society of Cardiology. FAU - Sugiura, Yuki AU - Sugiura Y AD - Department of Cardiology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, Aichi 466-8560, Japan. FAU - Okumura, Takahiro AU - Okumura T AUID- ORCID: 0000-0001-5076-2052 AD - Department of Cardiology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, Aichi 466-8560, Japan. FAU - Kondo, Toru AU - Kondo T AUID- ORCID: 0000-0001-6853-7574 AD - Department of Cardiology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, Aichi 466-8560, Japan. FAU - Murohara, Toyoaki AU - Murohara T AD - Department of Cardiology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, Aichi 466-8560, Japan. LA - eng PT - Case Reports DEP - 20200909 PL - England TA - Eur Heart J Case Rep JT - European heart journal. Case reports JID - 101730741 PMC - PMC7649480 OTO - NOTNLM OT - Case report OT - Endomyocardial biopsy OT - Immunosuppressive therapy OT - Inflammatory cell OT - Kawasaki disease OT - Shock EDAT- 2020/11/19 06:00 MHDA- 2020/11/19 06:01 PMCR- 2020/09/09 CRDT- 2020/11/18 06:01 PHST- 2020/03/30 00:00 [received] PHST- 2020/05/18 00:00 [revised] PHST- 2020/08/07 00:00 [accepted] PHST- 2020/11/18 06:01 [entrez] PHST- 2020/11/19 06:00 [pubmed] PHST- 2020/11/19 06:01 [medline] PHST- 2020/09/09 00:00 [pmc-release] AID - ytaa304 [pii] AID - 10.1093/ehjcr/ytaa304 [doi] PST - epublish SO - Eur Heart J Case Rep. 2020 Sep 9;4(5):1-7. doi: 10.1093/ehjcr/ytaa304. eCollection 2020 Oct.