Abstract Details

Title Cardiac Involvements in Anti-Mitochondrial Antibody-Positive Myositis

Topic Muscle Disease/Neuromuscular Junction

Presentation(s) P07 - (-)

Poster/Presentation
Number 046

Objective

Background BACKGROUND: Our recent study has shown that inflammatory myopathies associated with serum AMA form a characteristic subgroup; chronic disease course, muscle atrophy, cardiac involvements, and histopathological features with chronic myopathic changes and granulomatous inflammation. Because cardiac involvements sometimes become fatal, their detailed analysis is necessary.

Design/Methods DESIGN/METHODS: Twenty-six myositis patients diagnosed as having myositis associated with AMA or primary biliary cirrhosis (PBC) from 1996 to 2011 were included. Fisher's exact test and Mann-Whitney test were used in comparing the features between 10 patients with cardiac involvements and 16 without them.

Results RESULTS: Among AMA-positive myositis patients, those with cardiac involvements had characteristic clinical and histopathological features; chronic disease course, associations with PBC and other collagen diseases, and granulomatous inflammation in muscle biopsy. Detailed analysis of cardiac involvements in nine patients (three male and six female; average age at disease onset, 58) whose clinical charts were available for more than 1 year revealed that seven patients had atrial tachycardia, four had ventricular tachycardia, and seven had conduction block including five with atrioventricular (AV) block (three with 3rd AV block) and one with sick sinus syndrome. Four patients showed left ventricular (LV) dysfunction, among which three had diffuse LV hypokinesis and an average ejection fraction rate of 45%. Seven patients underwent device therapy, including 3 with pacemaker implantation and 1 with cardioverter defibrillator implantation, or catheter ablations. Muscle symptoms preceded cardiac involvement in five patients. Four of them who were treated with low-dose corticosteroids or were without medication experienced some new cardiac events and had to undergo device implantation.

Conclusions CONCLUSIONS: AMA-positive myositis was sometimes associated with serious cardiac involvements. Immunopathological mechanism in the AMA-positive myositis patients with cardiac involvements may be different from that in those without them.

Authors/Disclosures
Meiko Maeda, MD, PhD (Tokyo university) PRESENTER	No disclosure on file
	No disclosure on file
Shoji Tsuji, MD, PhD (University of Tokyo)	The institution of Dr. Tsuji has received research support from Novelpharma Co. Ltd..
Jun Shimizu, MD, PhD (Tokyo University of Technology)	No disclosure on file
Edward M. Kaye, MD (Stroke Therapeutics)	No disclosure on file
Leigh E. Charvet, PhD (NYU Langone)	Dr. Charvet has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Johnson & Johnson. Dr. Charvet has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Springer Healthcare. Dr. Charvet has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for YBrain. Dr. Charvet has stock in Johnson&Johnson.

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