Antimitochondrial Antibody-associated Myopathy with Slowly Progressive Cardiac Dysfunction

Figures

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  Figure 1.. A transthoracic echocardiogram of the patient at the first admission (A, B) and the third admission (C, D). Echocardiography at the first admission revealed localized thinning of basal inferior of LV (arrows). This lesion had expanded by the third admission (arrows).

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  Figure 2.. Cardiac magnetic resonance imaging (MRI) [short inversion time inversion recovery (STIR) imaging and late gadolinium enhancement (LGE) imaging] performed at the first admission (A) and the third admission (B). STIR imaging at the first admission detected a high-intensity region in the basal inferoseptal to inferolateral wall (A, white arrowheads). LGE imaging revealed late gadolinium enhancement of the basal inferior to inferolateral wall (A, red arrowheads). Cardiac MRI at the third admission revealed negative STIR imaging and positive LGE of the mid-layer of the basal septum and inferior to the lateral wall, suggesting myocardial fibrosis (B, red arrowheads).

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  Figure 3.. Histopathological findings of the left biceps brachii muscle. Hematoxylin and Eosin staining showed variations in fiber size as well as necrotic and regenerating fibers (A), positive major histocompatibility complex (MHC) class I (B), and membrane attack complex (C) immunostaining.

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  Figure 4.. Clinical course of the patient. PSL: prednisolone, UDCA: ursodeoxycholic acid, CK: creatine kinase, ALT: alanine aminotransferase, ALP: alkaline phosphatase, AMA-M2: antimitochondrial-M2 antibody, BNP: brain natriuretic peptide, CTR: cardio-thoracic ratio

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  Figure 5.. Histopathological findings of the endomyocardial biopsy specimen. Hematoxylin and Eosin staining showed non-specific findings with mild hypertrophy of cardiomyocytes with some variation in size. No inflammatory cell infiltrate was present.