Clinical Utility of Diffusion-weighted Whole-body Magnetic Resonance Imaging with Background Body Signal Suppression for Assessing and Monitoring IgG4-related Disease
A 77-year-old man with symptoms of chest pain was diagnosed with immunoglobulin G4 (IgG4)-related disease. Fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) revealed an intense uptake in the submandibular gland, lymph nodes and abdominal aortic wall. Diffusion-weighted imaging with background body signal suppression (DWIBS) revealed signal enhancements at the same location as those of the FDG-PET/CT findings. The DWIBS signal intensity decreased after steroid treatment, so we decreased the steroid dosage. Relapse did not occur. DWIBS makes it possible to adjust the medicine dosage while confirming the therapeutic effects and will likely be a useful method for monitoring IgG4-related disease.
: A 26-year-old woman with Takayasu's arteritis (TAK) experienced back and neck pain during tocilizumab (TCZ) treatment. The levels of C-reactive protein were normal, and ultrasonography revealed no significant changes. Diffusion-weighted whole-body imaging with background body signal suppression (DWIBS) showed signal enhancement in the walls of several arteries. Contrast computed tomography showed arterial inflammation in the same lesion. After increasing the dose of prednisolone and TCZ, all s...
#1Gabriele Ironi(UniSR: Vita-Salute San Raffaele University)H-Index: 4
#2Enrico Tombetti(UniSR: Vita-Salute San Raffaele University)H-Index: 12
Last. Francesco De Cobelli(UniSR: Vita-Salute San Raffaele University)H-Index: 39
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Giant cell arteritis (GCA) is a granulomatous large-vessel vasculitis. The wall of inflamed arteries contains an abundant infiltrate of lymphocytes and macrophages and undergoes substantial remodeling, with hyperplasia of the intimal layer, luminal occlusion and potentially aneurysm formation and
#1Yoji Komiya(Tokyo Medical and Dental University)H-Index: 2
#2Makoto Soejima(Tokyo Medical and Dental University)H-Index: 3
Last. Hitoshi Kohsaka(Tokyo Medical and Dental University)H-Index: 32
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A 59-year-old man with swollen submandibular glands developed an aortic aneurysm requiring aortic prosthesis implantation. Echocardiography performed to evaluate the cardiac function before the surgery incidentally revealed masses around the coronary arteries. The serum IgG4 levels were increased. A post-operational pathological examination of the abdominal aneurysms revealed infiltration of plasma cells, with the ratio of IgG4/IgG-positive cells being >80%. The patient was diagnosed with IgG4-r...
Last. Issei Komuro(UTokyo: University of Tokyo)H-Index: 90
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Immunoglobulin G4 (IgG4)-related disease is a systemic inflammatory disorder that was first described in patients with autoimmune pancreatitis. Although IgG4-related disease is thought to involve the cardiovascular system, case reports describing coronary artery involvement are relatively rare. We describe a patient who was previously diagnosed with autoimmune pancreatitis and found to have coronary periarteritis and luminal narrowing. After the initiation of steroid treatment, the patient's cor...
Abstract Immunoglobulin 4-related disease (IgG4-related disease) is a systemic inflammatory disease that presents with increases of serum IgG4. It may affect various systems, including the cardiovascular (CV) system. Assessment of serum IgG4 levels and involved organ biopsy are necessary for diagnosis. IgG4-related disease is characterized by fibrosclerosis, lymphocytic infiltration and presence of IgG4-positive plasma cells. The disease usually responds to treatment with corticosteroids and/or ...
A 60-year-old man was admitted due to the onset of right coronary artery (RCA) aneurysms. Coronary angiography showed two RCA aneurysms and focal stenosis with limitations in the blood flow. Balloon angioplasty was performed. However, the follow-up coronary angiography showed restenosis, an enlarged proximal aneurysm and a newly formed aneurysm. The serum immunoglobulin G4 level was elevated to 1,350 mg/dL and fluorodeoxyglucose positron emission tomography showed increased uptake in the ascendi...
A 76-year-old woman with multiple coronary risk factors was admitted to our hospital because of episodes of new-onset chest pain that had begun 3 days previously. She underwent percutaneous coronary intervention (PCI) for severe stenoses in the two high lateral (HL) branches. Intravascular ultrasound (IVUS) revealed massive stenotic lesions in the HL branches and tumorous nonstenotic lesions in the left anterior descending coronary artery (LAD) and the left circumflex coronary artery (LCx). iMAP...
Immunoglobulin G4-related disease, a newly emerging systemic autoimmune disorder, can potentially involve the cardiovascular system. The standard treatment for immunoglobulin G4-related cardiovascular disease has not been established. We encountered a very rare case of an immunoglobulin G4-related inflammatory abdominal aortic aneurysm coexisting with a coronary artery aneurysm and periarteritis. The patient underwent surgical resection for the abdominal aortic aneurysm, followed by successful c...
Last. Ravi Lingam(Northwick Park Hospital)H-Index: 12
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Abstract Purpose Immunoglobulin G4-related disease (IgG4-RD) is a newly defined fibro-inflammatory multisystemic condition defined by a triad of diagnostic criteria based on clinical presentation, biochemical and histopathological findings. Whereas some subsites of this disease have been well described in the literature so far (e.g. pancreas, kidneys, retroperitoneum, salivary glands), more recently identified anatomical sites of involvement in the head and neck are less well understood (e.g. no...