PMID- 30279795
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20201001
IS  - 1878-5409 (Print)
IS  - 1878-5409 (Electronic)
IS  - 1878-5409 (Linking)
VI  - 16
IP  - 2
DP  - 2017 Aug
TI  - Replacement myocardial fibrosis at the site of late gadolinium enhancement on 
      magnetic resonance imaging in a patient with diffuse cutaneous systemic 
      sclerosis: An autopsy report.
PG  - 48-51
LID - 10.1016/j.jccase.2017.04.005 [doi]
AB  - Late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR) is a 
      well-known finding indicative of cardiac involvement in systemic sclerosis (SSc 
      heart). However, few studies have reported the precise histopathology at the site 
      of LGE. We present an autopsy report of a 51-year-old man diagnosed with diffuse 
      cutaneous SSc according to a systematic diagnostic workup, including skin biopsy. 
      CMR indicated left ventricular (LV) dilatation and broadly distributed 
      subendocardial LGE in the LV walls. The patient was treated with 
      methylprednisolone pulse therapy because of multiple episodes of ventricular 
      tachycardia, whereas he subsequently died of left heart failure. An autopsy study 
      revealed broad subendocardial replacement fibrosis, concomitant with the 
      distribution of LGE on CMR, without inflammatory or edematous changes. Notably, 
      myocardial fibrosis was evident around the intramural coronary arteries, although 
      the arteries themselves were intact. These findings demonstrated that broad 
      subendocardial LGE on CMR reflected replacement myocardial fibrosis in a patient 
      with diffuse cutaneous SSc. These clinicopathological observations suggested that 
      spasms in the intramyocardial arteries or the cardiac Raynaud's phenomenon may 
      have provoked broad subendocardial fibrosis of the LV walls. <Learning objective: 
      The present autopsy report pathologically validated the presence of broad 
      myocardial fibrosis in the area of subendocardial late gadolinium enhancement 
      (LGE) on cardiac magnetic resonance in a patient with systemic sclerosis (SSc). 
      Lack of inflammatory changes along with intact coronary arteries suggested the 
      involvement of intramural coronary spasm in the development of cardiac 
      involvement in SSc. Such an LGE pattern may suggest end-stage cardiac involvement 
      and portend a poor prognosis.>.
FAU - Noguchi, Atsushi
AU  - Noguchi A
AD  - Division of Rheumatology, Endocrinology and Nephrology, Hokkaido University 
      Graduate School of Medicine, Hokkaido, Japan.
FAU - Tsujino, Ichizo
AU  - Tsujino I
AD  - Internal Medicine I, Hokkaido University Hospital, Hokkaido, Japan.
FAU - Oyama-Manabe, Noriko
AU  - Oyama-Manabe N
AD  - Department of Diagnostic and Interventional Radiology, Hokkaido University 
      Hospital, Hokkaido, Japan.
FAU - Tanino, Mishie
AU  - Tanino M
AD  - Laboratory of Cancer Research, Department of Pathology, Hokkaido University 
      Graduate School of Medicine, Hokkaido, Japan.
LA  - eng
PT  - Case Reports
DEP - 20170513
PL  - Japan
TA  - J Cardiol Cases
JT  - Journal of cardiology cases
JID - 101549579
PMC - PMC6149262
OTO - NOTNLM
OT  - Cardiac Raynaud's phenomenon
OT  - Late gadolinium enhancement
OT  - Myocardial fibrosis
OT  - Pathological evaluation
OT  - Systemic sclerosis
EDAT- 2017/05/13 00:00
MHDA- 2017/05/13 00:01
PMCR- 2017/05/13
CRDT- 2018/10/04 06:00
PHST- 2017/01/19 00:00 [received]
PHST- 2017/04/07 00:00 [revised]
PHST- 2017/04/11 00:00 [accepted]
PHST- 2018/10/04 06:00 [entrez]
PHST- 2017/05/13 00:00 [pubmed]
PHST- 2017/05/13 00:01 [medline]
PHST- 2017/05/13 00:00 [pmc-release]
AID - S1878-5409(17)30038-5 [pii]
AID - 10.1016/j.jccase.2017.04.005 [doi]
PST - epublish
SO  - J Cardiol Cases. 2017 May 13;16(2):48-51. doi: 10.1016/j.jccase.2017.04.005. 
      eCollection 2017 Aug.