Acute Systemic Sclerosis-Associated Cardiomyopathy That Improved With Glucocorticoids and Cyclophosphamide

Andrew A. Gustafson; Katherine V. Trinh; Jon W. Lomasney; Sanjiv J. Shah; Monique E Hinchcliff

doi:10.1016/j.jaccas.2024.102948

Acute Systemic Sclerosis-Associated Cardiomyopathy That Improved With Glucocorticoids and Cyclophosphamide

Andrew A. Gustafson, Katherine V. Trinh, Jon W. Lomasney, Sanjiv J. Shah, Monique E Hinchcliff^*

^*Corresponding author for this work

Research output: Contribution to journal › Article › peer-review

Abstract

Background: Systemic sclerosis (SSc) cardiomyopathy has a prevalence of 7 to 39% and is associated with increased mortality. Despite this, little evidence informs SSc cardiomyopathy treatment. Case Summary: We present a patient with diffuse cutaneous SSc with acute heart failure. Extensive workup supported a diagnosis of SSc myopericarditis, although endomyocardial biopsies were unrevealing. She received intravenous cyclophosphamide and glucocorticoids and achieved significant and prolonged recovery. Discussion: Our patient presented with systolic dysfunction as opposed to diastolic dysfunction that is more typical in patients with SSc-cardiomyopathy. Endomyocardial biopsies lacked T-lymphocyte infiltration that may be due to sampling error because >17 samples are needed to diagnose myocarditis in >80% of cases.

Original language	English (US)
Article number	102948
Journal	JACC: Case Reports
Volume	30
Issue number	4
DOIs	https://doi.org/10.1016/j.jaccas.2024.102948
State	Published - Feb 19 2025

Keywords

cardiac MRI
cyclophosphamide
myocarditis
myopericarditis
scleroderma
systemic sclerosis

ASJC Scopus subject areas

Cardiology and Cardiovascular Medicine

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

Access to Document

10.1016/j.jaccas.2024.102948

Cite this

@article{52c91277047246a98cbad4ab9bff46a0,

title = "Acute Systemic Sclerosis-Associated Cardiomyopathy That Improved With Glucocorticoids and Cyclophosphamide",

abstract = "Background: Systemic sclerosis (SSc) cardiomyopathy has a prevalence of 7 to 39% and is associated with increased mortality. Despite this, little evidence informs SSc cardiomyopathy treatment. Case Summary: We present a patient with diffuse cutaneous SSc with acute heart failure. Extensive workup supported a diagnosis of SSc myopericarditis, although endomyocardial biopsies were unrevealing. She received intravenous cyclophosphamide and glucocorticoids and achieved significant and prolonged recovery. Discussion: Our patient presented with systolic dysfunction as opposed to diastolic dysfunction that is more typical in patients with SSc-cardiomyopathy. Endomyocardial biopsies lacked T-lymphocyte infiltration that may be due to sampling error because >17 samples are needed to diagnose myocarditis in >80% of cases.",

keywords = "cardiac MRI, cyclophosphamide, myocarditis, myopericarditis, scleroderma, systemic sclerosis",

author = "Gustafson, {Andrew A.} and Trinh, {Katherine V.} and Lomasney, {Jon W.} and Shah, {Sanjiv J.} and Hinchcliff, {Monique E}",

note = "Publisher Copyright: {\textcopyright} 2025 The Authors",

year = "2025",

month = feb,

day = "19",

doi = "10.1016/j.jaccas.2024.102948",

language = "English (US)",

volume = "30",

journal = "JACC: Case Reports",

issn = "2666-0849",

publisher = "Elsevier Inc.",

number = "4",

}

TY - JOUR

T1 - Acute Systemic Sclerosis-Associated Cardiomyopathy That Improved With Glucocorticoids and Cyclophosphamide

AU - Gustafson, Andrew A.

AU - Trinh, Katherine V.

AU - Lomasney, Jon W.

AU - Shah, Sanjiv J.

AU - Hinchcliff, Monique E

PY - 2025/2/19

Y1 - 2025/2/19

N2 - Background: Systemic sclerosis (SSc) cardiomyopathy has a prevalence of 7 to 39% and is associated with increased mortality. Despite this, little evidence informs SSc cardiomyopathy treatment. Case Summary: We present a patient with diffuse cutaneous SSc with acute heart failure. Extensive workup supported a diagnosis of SSc myopericarditis, although endomyocardial biopsies were unrevealing. She received intravenous cyclophosphamide and glucocorticoids and achieved significant and prolonged recovery. Discussion: Our patient presented with systolic dysfunction as opposed to diastolic dysfunction that is more typical in patients with SSc-cardiomyopathy. Endomyocardial biopsies lacked T-lymphocyte infiltration that may be due to sampling error because >17 samples are needed to diagnose myocarditis in >80% of cases.

AB - Background: Systemic sclerosis (SSc) cardiomyopathy has a prevalence of 7 to 39% and is associated with increased mortality. Despite this, little evidence informs SSc cardiomyopathy treatment. Case Summary: We present a patient with diffuse cutaneous SSc with acute heart failure. Extensive workup supported a diagnosis of SSc myopericarditis, although endomyocardial biopsies were unrevealing. She received intravenous cyclophosphamide and glucocorticoids and achieved significant and prolonged recovery. Discussion: Our patient presented with systolic dysfunction as opposed to diastolic dysfunction that is more typical in patients with SSc-cardiomyopathy. Endomyocardial biopsies lacked T-lymphocyte infiltration that may be due to sampling error because >17 samples are needed to diagnose myocarditis in >80% of cases.

KW - cardiac MRI

KW - cyclophosphamide

KW - myocarditis

KW - myopericarditis

KW - scleroderma

KW - systemic sclerosis

UR - http://www.scopus.com/inward/record.url?scp=85216694126&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85216694126&partnerID=8YFLogxK

U2 - 10.1016/j.jaccas.2024.102948

DO - 10.1016/j.jaccas.2024.102948

M3 - Article

C2 - 39972702

AN - SCOPUS:85216694126

SN - 2666-0849

VL - 30

JO - JACC: Case Reports

JF - JACC: Case Reports

IS - 4

M1 - 102948

ER -

Acute Systemic Sclerosis-Associated Cardiomyopathy That Improved With Glucocorticoids and Cyclophosphamide

Abstract

Keywords

ASJC Scopus subject areas

UN SDGs

Access to Document

Other files and links

Fingerprint

Cite this