A case of limited cutaneous systemic sclerosis developing anti-mitochondria antibody positive primary biliary cirrhosis after acute myocardial infarction

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Clin Rheumatol DOI 10.1007/s10067-006-0465-1

CASE REPORT

A case of limited cutaneous systemic sclerosis developing anti-mitochondria antibody positive primary biliary cirrhosis after acute myocardial infarction Kiyomitsu Miyachi & Raleigh Hankins & Minoru Ihara & Akira Miyamoto & Tetsuroh Okano & Miwako Iwai & Katsuhiko Mikoshiba & Marvin J. Fritzler

Received: 18 September 2006 / Revised: 4 October 2006 / Accepted: 5 October 2006 # Clinical Rheumatology 2006

Abstract In this report, we present a 63-year-old woman who had limited cutaneous systemic sclerosis and subsequently developed typical primary biliary cirrhosis after an acute myocardial infarction. The patient initially developed Raynaud’s phenomenon, and 4 years later visited the clinic in 1994 complaining of abdominal distress, xerostomia, and xerophthalmia. A diagnosis of limited cutaneous systemic sclerosis was based on Raynaud’s phenomenon, sclerodactyly and anti-centromere antibodies. She was also found to have anti-inositol 1,4,5-trisphosphate receptor 3 (IP3R3) antibodies, but anti-mitochondrial antibodies were only weakly positive. Seven years later, she developed vertigo

and nausea, and was hospitalized due to complaints of an oppressive sensation of the anterior chest. Electrocardiogram results showed a reduction of R waves and ST segment elevation in II, III, and aVf leads. Coronary angiography showed 99% obstruction of the left anterior descending artery and 50% of stenosis of the right coronary artery. Three years later, the patient was noted to have antimitochondrial antibodies. Retrospective analysis of the patient’s sera showed that IP3R3 antibodies were decreasing. Since myocardium is particularly rich in mitochondria, it is thought that myocardial infarction may have been the triggering event that initiated antigen-presenting cells to selectively induce an anti-mitochondrial antibody response.

K. Miyachi (*) Rheumatology and Clinical Immunology, Keigu Clinic, 2-2 Ichibanishinaka-cho, Tsurumi-ku, Yokohama, Kanagawa 230-0023, Japan e-mail: [email protected]

Keywords Anti-centromere antibody (ACA) . Antimitochondrial antibody (AMA) . High mobility group box1 (HMGB1) . Inositol 1,4,5-trisphosphate receptor (IP3R) . Primary biliary cirrhosis (PBC) . Receptor for advanced glycation end-products (RAGE)

K. Miyachi : R. Hankins First Diagnostic Division, Health Sciences Research Institute, Yokohama, Japan M. Ihara : A. Miyamoto Division of Cardiology, Kawasaki Saiwai Hospital, Kawasaki, Japan T. Okano Allied Health Science Immunology, Kitazato University, Sagamihara, Japan M. Iwai : K. Mikoshiba Division of Molecular Neurobiology, University of Tokyo, Tokyo, Japan M. J. Fritzler Faculty of Medicine, University of Calgary, Calgary, Canada

Introduction Patients with anti-centromere antibodies present with various clinical manifestations such as Raynaud’s phenomenon, sclerodactyly, xerostomia, xerophthalmia, and liver damage and are commonly diagnosed as having either systemic sclerosis (SSc), Sjögren’s syndrome, primary biliary cirrhosis (PBC), or overlap syndrome [1, 2]. In this case report, a SSc woman having high titers of anticentromere antibodies developed a myocardial infarction (MI). A few years later, anti-mitochondrial antibodies (AMA) were detected by indirect immunofluorescence

Clin Rheumatol

(IIF) and the presence of M2 (pyruvate dehydrogenase) antibodies was confirmed by enzyme-linked immunosorbent assay (ELISA) [3]. We discuss the significance of antiIP3R3 antibodies and the possibility that the appearance of AMA was induced by MI.

Materials and methods Immunoblotting for anti-IP3Rs antibodies Microsome fractions were prepared from Sf9 cells overexpressing mouse IP3Rs as described previously [4]. Proteins were separated by 5% sodium dodecyl sulfate-polyacrylamide gel electrophoresis and transferred to polyvinylidene difluoride membrane. The membrane was blocked with 3% skim milk in phosphate-buffered saline plus 0.05% Tween 20 (PBST) and incubated overnight at 4°C with test serum diluted 1:300. After three subsequent washes with PBST, the membrane was incubated with anti-human IgG (heavy and light chains) conjugated to horseradish peroxidase (BETHYL laboratories; 1:2,000) for 1 h at room temperature. Bands were visualized using ECL Western blotting detection reagents (GE Healthcare). The signal intensities were calculated using Scion Image software (Scion), and the values were recorded based on the average of at least three measurements. The signal intensities themselves were semiquantitative, and signals were judged positive when yielding intensities exceeding 80 (twice or more the average of intensity obtained from normal healthy subjects).

elevated M2 of 18.8 were noted, but the AMA was
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