PMID- 37096149
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20230426
IS  - 2514-2119 (Electronic)
IS  - 2514-2119 (Linking)
VI  - 7
IP  - 4
DP  - 2023 Apr
TI  - Fulminant myocarditis in a young woman with mixed connective tissue disease: a 
      case report.
PG  - ytad174
LID - 10.1093/ehjcr/ytad174 [doi]
LID - ytad174
AB  - BACKGROUND: Although cardiac involvement is relatively common in mixed connective 
      tissue disease (MCTD), few reports on MCTD-associated fulminant myocarditis are 
      available. CASE SUMMARY: A 22-year-old woman diagnosed with MCTD was admitted to 
      our institution for cold-like symptoms and chest pain. Echocardiography revealed 
      that the left ventricular ejection fraction (LVEF) had rapidly decreased from 50 
      to 20%. Because endomyocardial biopsy revealed no significant lymphocytic 
      infiltration, immunosuppressant drugs were not started initially; however, 
      steroid pulse therapy (methylprednisolone, one1000 mg/day) was initiated due to 
      prolonged symptoms and unimproved haemodynamics. Despite strong immunosuppressant 
      therapy, the LVEF did not improve, and severe mitral regurgitation appeared. 
      Three days after steroid pulse therapy initiation, she experienced a sudden 
      cardiac arrest; thus, venoarterial extracorporeal membrane oxygenation (VA-ECMO) 
      and intra-aortic balloon pumping (IABP) were initiated. Subsequent 
      immunosuppressant therapy was continued with prednisolone (100 mg/day) and 
      intravenous cyclophosphamide (1000 mg). Six days after steroid therapy 
      initiation, the LVEF improved to 40% and then recovered to near-normal levels. 
      After successful weaning off of VA-ECMO and IABP, she was discharged. Thereafter, 
      a detailed histopathological examination revealed multi-focal signs of ischaemic 
      micro-circulatory injury and diffuse HLA-DR in the vascular endothelium, 
      suggesting an autoimmune inflammatory response. DISCUSSION: We report a rare case 
      of fulminant myocarditis in a patient with MCTD who recovered with 
      immunosuppressive treatment. Despite the absence of significant lymphocytic 
      infiltration findings on histopathological examination, patients with MCTD may 
      experience a dramatic clinical course. Although it is unclear whether myocarditis 
      is triggered by viral infections, certain autoimmune mechanisms may lead to its 
      development.
CI  - (c) The Author(s) 2023. Published by Oxford University Press on behalf of the 
      European Society of Cardiology.
FAU - Hamana, Tomoyo
AU  - Hamana T
AD  - Division of Cardiovascular Medicine, Department of Internal Medicine, Kobe 
      University Graduate School of Medicine, 7-5-1 Kusunoki-cho, Chuo-ku, Kobe, Hyogo 
      650-0017, Japan.
FAU - Kawamori, Hiroyuki
AU  - Kawamori H
AUID- ORCID: 0000-0001-6810-447X
AD  - Division of Cardiovascular Medicine, Department of Internal Medicine, Kobe 
      University Graduate School of Medicine, 7-5-1 Kusunoki-cho, Chuo-ku, Kobe, Hyogo 
      650-0017, Japan.
FAU - Satomi-Kobayashi, Seimi
AU  - Satomi-Kobayashi S
AD  - Division of Cardiovascular Medicine, Department of Internal Medicine, Kobe 
      University Graduate School of Medicine, 7-5-1 Kusunoki-cho, Chuo-ku, Kobe, Hyogo 
      650-0017, Japan.
FAU - Yamamoto, Yuzuru
AU  - Yamamoto Y
AD  - Division of Rheumatology and Clinical Immunology, Department of Internal 
      Medicine, Kobe University Graduate School of Medicine, Kobe, Japan.
FAU - Ikeda, Yoshihiko
AU  - Ikeda Y
AD  - Department of Pathology, National Cerebral and Cardiovascular Center, Suita, 
      Osaka, Japan.
FAU - Hirata, Ken-Ichi
AU  - Hirata KI
AUID- ORCID: 0000-0002-6782-3031
AD  - Division of Cardiovascular Medicine, Department of Internal Medicine, Kobe 
      University Graduate School of Medicine, 7-5-1 Kusunoki-cho, Chuo-ku, Kobe, Hyogo 
      650-0017, Japan.
LA  - eng
PT  - Case Reports
DEP - 20230410
PL  - England
TA  - Eur Heart J Case Rep
JT  - European heart journal. Case reports
JID - 101730741
PMC - PMC10122413
OTO - NOTNLM
OT  - Autoimmune diseases
OT  - Biopsy
OT  - Case reports
OT  - Mixed connective tissue disease
OT  - Myocarditis
COIS- Conflict of interest: None declared.
EDAT- 2023/04/25 06:42
MHDA- 2023/04/25 06:43
PMCR- 2023/04/10
CRDT- 2023/04/25 02:09
PHST- 2022/08/08 00:00 [received]
PHST- 2022/11/22 00:00 [revised]
PHST- 2023/04/03 00:00 [accepted]
PHST- 2023/04/25 06:43 [medline]
PHST- 2023/04/25 06:42 [pubmed]
PHST- 2023/04/25 02:09 [entrez]
PHST- 2023/04/10 00:00 [pmc-release]
AID - ytad174 [pii]
AID - 10.1093/ehjcr/ytad174 [doi]
PST - epublish
SO  - Eur Heart J Case Rep. 2023 Apr 10;7(4):ytad174. doi: 10.1093/ehjcr/ytad174. 
      eCollection 2023 Apr.