A clinical and histopathologic comparison of cardiac sarcoidosis and idiopathic giant cell myocarditis

Yuji Okura, G. William Dec, Joshua Hare, Makoto Kodama, Gerald J. Berry, Henry D. Tazelaar, Kent R. Bailey, Leslie T. Cooper

Research output: Contribution to journalArticle

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Abstract

OBJECTIVES: The goal of this study was to determine the prognostic value of clinical data available at presentation and histology in cardiac sarcoidosis (CS) and idiopathic giant cell myocarditis (IGCM). BACKGROUND: The prognosis of patients with nonischemic cardiomyopathy is partly dependent on the histologic diagnosis. Survival in IGCM is poor. The prognosis of a histologically related entity, cardiac sarcoidosis (CS), is less well established, and the prognostic value of the distinction between CS and IGCM on endomyocardial biopsy (EMB) is unknown. METHODS: We identified 115 patients from the Multicenter IGCM Registry with CS (n = 42) and IGCM (n = 73). We compared the clinical data for these two groups using Cox proportional-hazards models to assess the association between histologic diagnosis and survival. In order to determine whether histologic features could reliably differentiate these two entities, two cardiac pathologists semiquantitatively graded the inflammatory infiltrate components and compared the results between groups. RESULTS: Black race was more frequent in the CS group (31% vs. 4%, p < 0.0001). Syncope and atrioventricular block were also more frequently observed in CS than IGCM (31% vs. 5%, p = 0.0002 and 50% vs. 15%, p < 0.0001, respectively). Left-sided heart failure was more common in IGCM (40% vs. 64%, p = 0.013). In CS patients diagnosed by EMB, the five-year transplant-free survival after diagnosis was 69.8% versus 21.9% for IGCM (p < 0.0001, log-rank test). In multivariate models, presentation with heart failure predicted IGCM, and presentation with heart block or more than nine weeks of symptoms predicted CS. Eosinophils, myocyte damage, and foci of lymphocytic myocarditis were more frequent in IGCM, while granulomas and fibrosis were more frequent in CS. CONCLUSIONS: Transplant-free survival is better for patients with CS than for IGCM diagnosed by EMB. Presentation with heart failure predicted IGCM, and presentation with heart block or more than nine weeks of symptoms predicted CS.

Original languageEnglish
Pages (from-to)322-328
Number of pages7
JournalJournal of the American College of Cardiology
Volume41
Issue number2
DOIs
StatePublished - Jan 15 2003
Externally publishedYes

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Myocarditis
Giant Cells
Sarcoidosis
Heart Block
Survival
Heart Failure
Biopsy
Transplants
Atrioventricular Block
Syncope
Granuloma
Cardiomyopathies
Proportional Hazards Models
Eosinophils
Muscle Cells
Registries
Histology
Fibrosis

ASJC Scopus subject areas

  • Nursing(all)

Cite this

A clinical and histopathologic comparison of cardiac sarcoidosis and idiopathic giant cell myocarditis. / Okura, Yuji; Dec, G. William; Hare, Joshua; Kodama, Makoto; Berry, Gerald J.; Tazelaar, Henry D.; Bailey, Kent R.; Cooper, Leslie T.

In: Journal of the American College of Cardiology, Vol. 41, No. 2, 15.01.2003, p. 322-328.

Research output: Contribution to journalArticle

Okura, Yuji ; Dec, G. William ; Hare, Joshua ; Kodama, Makoto ; Berry, Gerald J. ; Tazelaar, Henry D. ; Bailey, Kent R. ; Cooper, Leslie T. / A clinical and histopathologic comparison of cardiac sarcoidosis and idiopathic giant cell myocarditis. In: Journal of the American College of Cardiology. 2003 ; Vol. 41, No. 2. pp. 322-328.
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abstract = "OBJECTIVES: The goal of this study was to determine the prognostic value of clinical data available at presentation and histology in cardiac sarcoidosis (CS) and idiopathic giant cell myocarditis (IGCM). BACKGROUND: The prognosis of patients with nonischemic cardiomyopathy is partly dependent on the histologic diagnosis. Survival in IGCM is poor. The prognosis of a histologically related entity, cardiac sarcoidosis (CS), is less well established, and the prognostic value of the distinction between CS and IGCM on endomyocardial biopsy (EMB) is unknown. METHODS: We identified 115 patients from the Multicenter IGCM Registry with CS (n = 42) and IGCM (n = 73). We compared the clinical data for these two groups using Cox proportional-hazards models to assess the association between histologic diagnosis and survival. In order to determine whether histologic features could reliably differentiate these two entities, two cardiac pathologists semiquantitatively graded the inflammatory infiltrate components and compared the results between groups. RESULTS: Black race was more frequent in the CS group (31{\%} vs. 4{\%}, p < 0.0001). Syncope and atrioventricular block were also more frequently observed in CS than IGCM (31{\%} vs. 5{\%}, p = 0.0002 and 50{\%} vs. 15{\%}, p < 0.0001, respectively). Left-sided heart failure was more common in IGCM (40{\%} vs. 64{\%}, p = 0.013). In CS patients diagnosed by EMB, the five-year transplant-free survival after diagnosis was 69.8{\%} versus 21.9{\%} for IGCM (p < 0.0001, log-rank test). In multivariate models, presentation with heart failure predicted IGCM, and presentation with heart block or more than nine weeks of symptoms predicted CS. Eosinophils, myocyte damage, and foci of lymphocytic myocarditis were more frequent in IGCM, while granulomas and fibrosis were more frequent in CS. CONCLUSIONS: Transplant-free survival is better for patients with CS than for IGCM diagnosed by EMB. Presentation with heart failure predicted IGCM, and presentation with heart block or more than nine weeks of symptoms predicted CS.",
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AU - Okura, Yuji

AU - Dec, G. William

AU - Hare, Joshua

AU - Kodama, Makoto

AU - Berry, Gerald J.

AU - Tazelaar, Henry D.

AU - Bailey, Kent R.

AU - Cooper, Leslie T.

PY - 2003/1/15

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N2 - OBJECTIVES: The goal of this study was to determine the prognostic value of clinical data available at presentation and histology in cardiac sarcoidosis (CS) and idiopathic giant cell myocarditis (IGCM). BACKGROUND: The prognosis of patients with nonischemic cardiomyopathy is partly dependent on the histologic diagnosis. Survival in IGCM is poor. The prognosis of a histologically related entity, cardiac sarcoidosis (CS), is less well established, and the prognostic value of the distinction between CS and IGCM on endomyocardial biopsy (EMB) is unknown. METHODS: We identified 115 patients from the Multicenter IGCM Registry with CS (n = 42) and IGCM (n = 73). We compared the clinical data for these two groups using Cox proportional-hazards models to assess the association between histologic diagnosis and survival. In order to determine whether histologic features could reliably differentiate these two entities, two cardiac pathologists semiquantitatively graded the inflammatory infiltrate components and compared the results between groups. RESULTS: Black race was more frequent in the CS group (31% vs. 4%, p < 0.0001). Syncope and atrioventricular block were also more frequently observed in CS than IGCM (31% vs. 5%, p = 0.0002 and 50% vs. 15%, p < 0.0001, respectively). Left-sided heart failure was more common in IGCM (40% vs. 64%, p = 0.013). In CS patients diagnosed by EMB, the five-year transplant-free survival after diagnosis was 69.8% versus 21.9% for IGCM (p < 0.0001, log-rank test). In multivariate models, presentation with heart failure predicted IGCM, and presentation with heart block or more than nine weeks of symptoms predicted CS. Eosinophils, myocyte damage, and foci of lymphocytic myocarditis were more frequent in IGCM, while granulomas and fibrosis were more frequent in CS. CONCLUSIONS: Transplant-free survival is better for patients with CS than for IGCM diagnosed by EMB. Presentation with heart failure predicted IGCM, and presentation with heart block or more than nine weeks of symptoms predicted CS.

AB - OBJECTIVES: The goal of this study was to determine the prognostic value of clinical data available at presentation and histology in cardiac sarcoidosis (CS) and idiopathic giant cell myocarditis (IGCM). BACKGROUND: The prognosis of patients with nonischemic cardiomyopathy is partly dependent on the histologic diagnosis. Survival in IGCM is poor. The prognosis of a histologically related entity, cardiac sarcoidosis (CS), is less well established, and the prognostic value of the distinction between CS and IGCM on endomyocardial biopsy (EMB) is unknown. METHODS: We identified 115 patients from the Multicenter IGCM Registry with CS (n = 42) and IGCM (n = 73). We compared the clinical data for these two groups using Cox proportional-hazards models to assess the association between histologic diagnosis and survival. In order to determine whether histologic features could reliably differentiate these two entities, two cardiac pathologists semiquantitatively graded the inflammatory infiltrate components and compared the results between groups. RESULTS: Black race was more frequent in the CS group (31% vs. 4%, p < 0.0001). Syncope and atrioventricular block were also more frequently observed in CS than IGCM (31% vs. 5%, p = 0.0002 and 50% vs. 15%, p < 0.0001, respectively). Left-sided heart failure was more common in IGCM (40% vs. 64%, p = 0.013). In CS patients diagnosed by EMB, the five-year transplant-free survival after diagnosis was 69.8% versus 21.9% for IGCM (p < 0.0001, log-rank test). In multivariate models, presentation with heart failure predicted IGCM, and presentation with heart block or more than nine weeks of symptoms predicted CS. Eosinophils, myocyte damage, and foci of lymphocytic myocarditis were more frequent in IGCM, while granulomas and fibrosis were more frequent in CS. CONCLUSIONS: Transplant-free survival is better for patients with CS than for IGCM diagnosed by EMB. Presentation with heart failure predicted IGCM, and presentation with heart block or more than nine weeks of symptoms predicted CS.

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