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

Yuji Okura, MD, PhD^*, G. William Dec, MD, FACC, Joshua M. Hare, MD, FACC^||, Makoto Kodama, MD^¶, Gerald J. Berry, MD^#, Henry D. Tazelaar, MD, Kent R. Bailey, PhD and Leslie T. Cooper, MD, FACC^*,*

^* Division of Cardiovascular Diseases, Mayo Clinic and Foundation, Rochester, Minnesota, USA
Division of Anatomic Pathology, Mayo Clinic and Foundation, Rochester, Minnesota, USA
Department of Biostatistics, Mayo Clinic and Foundation, Rochester, Minnesota, USA
Cardiovascular Division, Massachusetts General Hospital, Boston, Massachusetts, USA
^|| Cardiovascular Division, Johns Hopkins Hospital, Baltimore, Maryland, USA
^¶ First Department of Internal Medicine, Niigata University School of Medicine, Niigata, Japan
^# Department of Pathology, Stanford University Medical Center, Stanford, California, USA

View larger version (153K): [in a new window]   Figure 1 (A) Cardiac sarcoidosis characterized by coalescence of granulomatous elements along lymphatic pathways of the myocardial interstitium (H&E x 60). (B) High-power magnification showing well-formed nonnecrotizing granulomas composed of epithelioid histiocytes and giant cells embedded in callagenous stroma. Mononuclear inflammatory cells and fibroblasts surround the granulomas (H&E x 400). (C) Idiopathic giant cell myocarditis (IGCM) showing widespread necrosis of myocytes by a dense cellular infiltrate (H&E x 100). (D) High-power magnification of IGCM displaying giant cells, lymphocytes, histiocytes, eosinophils, and damaged myocytes (H&E x 400).

View larger version (23K): [in a new window]   Figure 2 Histologic scores for 75 cases of idiopathic giant cell myocarditis (IGCM) and cardiac sarcoidosis (CS). Scores were compared using the Wilcoxon rank-sum test. *p < 0.05; p < 0.01. Open bar = IGCM; solid bar = CS.

View larger version (18K): [in a new window]   Figure 3 Kaplan-Meier curves illustrating transplant-free survival in cardiac sarcoidosis (CS) and idiopathic giant cell myocarditis (IGCM) patients. (A) Time from symptom onset for 115 subjects from all centers. (B) Time from endomyocardial biopsy in 67 patients from all centers. (C) Time from endomyocardial biopsy in 41 patients at five core centers. All survival comparisons are by log-rank test.