ACE2 deficiency promotes the development of vascular diseases associated with Ang-II-mediated vascular inflammation and activation of the JNK signalling, leading to the notion that ACE2 potentially confers protection against vascular diseases.
Immunoglobulin G4 (IgG4)-related systemic disease was first recognized as a clinicopathological entity about 10 years ago, and since then, it has attracted growing attention. It is an autoimmune disease which affects multiple organs including the pancreas, bile duct, salivary glands and retroperitoneum. Further, it was recently reported that it can be manifested as periarteritis, often as inflammatory abdominal aortic aneurysm. We describe the case of a 75-year-old man with autoimmune pancreatitis and parotitis who presented with angina. The serum concentration of IgG4 was significantly increased at 2,510 mg/dl. Coronary angiography showed multiple stenotic lesions and pronounced dilatation of the right coronary artery. Cardiac computed tomography disclosed increased wall thickness of the coronary arteries and focal tumorous lesions surrounding the right coronary artery. Treatment with steroids proved only marginally effective and he underwent surgical resection of the aneurysm and coronary artery bypass grafting. The diagnosis of IgG4-related systemic disease was confirmed by histological examination of the resected mass, which showed a massive infiltration of IgG4-positive plasma cells. This case emphasizes the importance of considering the diagnosis in any patient with abnormally increased wall thickness or ectatic lesions in the coronary arteries.
We report the case of apical ballooning syndrome (ABS) in a female sibling. A 64-year-old woman was admitted to our hospital with sudden-onset chest pain. Cardiac enzymes were mildly elevated and an electrocardiogram showed broad ST-T changes. Emergency coronary angiography revealed no culprit lesion and left ventriculography demonstrated focal akinesis of the apical wall, which was consistent with ABS. Myocardial functional sympathetic innervations assessed using [(123)I]metaiodobenzylguanidine was severely impaired in the apical region. Her clinical symptoms and cardiac dysfunction recovered spontaneously. Just 1 year prior to our patient's cardiac event, her elder sister had the same symptoms and was also diagnosed with ABS. Both sisters were postmenopausal. The familial case of ABS is exceedingly rare, but these cases suggest a possible genetic etiology.
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