IgG4-related periaortitis/periarteritis: An under-recognized condition that is potentially life-threatening.

Published on Mar 8, 2019in Modern Rheumatology2.113
· DOI :10.1080/14397595.2018.1546367
Ichiro Mizushima13
Estimated H-index: 13
(Kanazawa University),
Satomi Kasashima22
Estimated H-index: 22
(Kanazawa University)
+ 2 AuthorsNobukazu Ishizaka37
Estimated H-index: 37
(Osaka Medical College)
Sources
Abstract
AbstractPeriaortitis and periarteritis are one of the clinical features of IgG4-related disease. Such vascular manifestations are reported to be present in 10–30% of overall IgG4-related disease, a...
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#1Nobukazu Ishizaka (Osaka Medical College)H-Index: 37
#2Yasunari Fujinaga (Shinshu University)H-Index: 20
Last. Koichi Sohmiya (Osaka Medical College)H-Index: 18
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: Immunoglobulin G4 (IgG4)-related disease, that is characterized by the elevation of circulating IgG4 level and the tissue-infiltration of IgG4-positive plasma cells, can target the cardiovascular tissue, although the diagnosis of IgG4-related cardiovascular lesion is not easy owing to the substantial risk for the tissue sampling. We herein examined the serum IgG4 levels among cardiac patients. In patients who were admitted to the cardiology department (n=477) and those who underwent computed to...
2 CitationsSource
#1Ryoto Hourai (Osaka Medical College)H-Index: 3
#2Satomi Kasashima (Kanazawa University)H-Index: 22
Last. Nobukazu Ishizaka (Osaka Medical College)H-Index: 37
view all 10 authors...
: A 74-year-old man was admitted for preoperative screening of aortic stenosis. Five months before this admission, he was found to have elevated serum immunoglobulin G4 (IgG4; 2,010 mg/dL). Computed tomography (CT) showed a soft tissue mass surrounding the abdominal aorta, suggestive of IgG4-related periaortitis. CT coronary angiography showed perivascular thickening of the right coronary artery, and subsequent coronary angiography showed a multi-vessel disease. The patient underwent aortic valv...
4 CitationsSource
#1Ichiro Mizushima (Kanazawa University)H-Index: 13
#2Satomi Kasashima (Kanazawa University)H-Index: 22
Last. Nobukazu Ishizaka (Osaka Medical College)H-Index: 37
view all 17 authors...
2 CitationsSource
#1Aureliano Ruggio (UCSC: Catholic University of the Sacred Heart)H-Index: 4
#2A. Iaconelli (UCSC: Catholic University of the Sacred Heart)H-Index: 8
Last. Filippo Crea (UCSC: Catholic University of the Sacred Heart)H-Index: 103
view all 10 authors...
Abstract We report the case of a 65-year-old man referred to our department because of a complex coronary aneurysmal disease presenting as subacute ST-segment elevation myocardial infarction due to intraluminal thromboembolism. The patient’s past medical history (recurrent episodes of lymphadenopathies, parotid swelling, prostatitis, and dacryoadenitis) raised the suspicion of a unifying systemic disorder with coronary involvement. An extensive infectious and autoimmune screening was performed, ...
5 CitationsSource
#1Mirei Shirakashi (Kyoto University)H-Index: 4
#2Hajime Yoshifuji (Kyoto University)H-Index: 23
Last. Tsuneyo Mimori (Kyoto University)H-Index: 77
view all 25 authors...
Glucocorticoids (GC) are effective for treating IgG4-related disease (IgG4-RD); however, relapse is often observed. We conducted a retrospective multicentre study to investigate risk factors in GC regimens associated with relapses of IgG4-RD. Data on 166 patients with definitive IgG4-RD diagnosis were collected from 12 institutions. Comprehensive surveillance of clinical backgrounds and GC regimens as well as multivariate analysis of factors associated with treatment responses and relapses was p...
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#1M. LuisH-Index: 2
#2Luisa BritesH-Index: 3
Last. Armando MalcataH-Index: 10
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Vascular involvement in IgG4-related disease (IgG4-RD), is a well-recognized feature and large vessel commitment, especially the aorta, can be the only manifestation of the disease. Being a newly recognized disease, its diagnosis and workup still represents a challenge in clinical practice. A 47-year-old-man with two aortic aneurysms ruptures, one at abdominal and the other at thoracic level, was referred to our rheumatology department. The initial analysis of the surgical specimen obtained 3 ye...
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#1Deng Hui (Peking Union Medical College)H-Index: 1
#2Sheng ZhaoH-Index: 24
Last. Xinying XueH-Index: 9
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IgG4-related disease (IgG4-RD) is recognized as an immune-mediated condition with pathology features of lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis, accompanied with or without elevated serum IgG4 concentrations. However, few of pulmonary artery IgG4-RD causing pulmonary hypertension (PH) was reported. The medical records of 3 patients with pulmonary artery IgG4-RD inducing PH were analyzed retrospectively. Imaging findings demonstrated that the lesions of 3 pati...
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#1Mohammad Almeqdadi (Tufts University)H-Index: 6
#2Mohammed Al-Dulaimi (Tufts University)H-Index: 1
Last. Bertrand L. Jaber (Tufts University)H-Index: 64
view all 5 authors...
: Retroperitoneal fibrosis (RPF) is a progressive fibroinflammatory disease that can be complicated by urinary obstruction. RPF can be the only manifestation of IgG4-related disease (IgG4-RD). Treatment of IgG4-related RPF is challenging and mostly consists of long-term glucocorticoids leading to significant side effects and treatment intolerance. Recent exploration of the role of rituximab as a B-cell depleting therapy in the treatment of IgG4-RD provides therapeutic potential as a well-tolerat...
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#1Kazunori Yamada (Kanazawa University)H-Index: 18
#2Motohisa Yamamoto (Sapporo Medical University)H-Index: 41
Last. Mitsuhiro Kawano (Kanazawa University)H-Index: 21
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Background The aim was to further characterize immunoglobulin G4-related disease (IgG4-RD) by a large-scale multicenter study of its clinical and laboratory features conducted by multidisciplinary physicians of IgG4-RD in Japan.
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#1Catalina Sanchez-Alvarez (Mayo Clinic)H-Index: 5
#2Andrew W. Bowman (Mayo Clinic)H-Index: 6
Last. Benjamin Wang (Mayo Clinic)H-Index: 1
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Cited By14
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#1Ichiro Mizushima (Kanazawa University)H-Index: 13
#2Takahiro Yamano (Kanazawa University)
Last. Mitsuhiro Kawano (Kanazawa University)H-Index: 34
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OBJECTIVES The 2019 ACR/EULAR classification criteria for IgG4-related disease (IgG4-RD) have exclusion criteria including positive disease-specific autoantibodies, and these have been documented to have a high specificity. This study aimed to further validate these criteria as well as identify characteristics of patients showing false-negative results. METHODS We retrospectively analysed 162 IgG4-RD patients and 130 mimickers. The sensitivity, specificity and fulfilment rates for each criterion...
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#1Amrita Valluri (Marshall University)H-Index: 1
#2Juliana Sitta (UMMC: University of Mississippi Medical Center)H-Index: 1
Last. Candace M. Howard (UMMC: University of Mississippi Medical Center)H-Index: 17
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Abstract null null Immunoglobulin G4-related aortitis (IgG4-RA) is histologically characterized by the infiltration of IgG4 positive plasma cells and fibrosis in systemic organs and the elevation of serum IgG4 levels. The cardiovascular system is commonly involved with various possible presentations such as aortitis, arteritis, periaortitis, periarteritis, and inflammatory aneurysm. We present a case of a 48-year-old male without known previous medical history, admitted for further workup of lon...
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#1G. Saitakis (MEE: Massachusetts Eye and Ear Infirmary)
#2Bart K Chwalisz (Harvard University)H-Index: 6
Abstract Purpose of review IgG4-related disease (IgG4-RD) is emerging as a fibro-inflammatory entity affecting multiple organs, including manifold neurologic manifestations. This review discusses general characteristics of IgG4-RD neurologic disease including epidemiology, histology, clinical picture and treatment approaches. Recent findings IgG4-RD is increasingly recognized as an important underlying pathophysiology in multiple disorders of neurologic interest, including orbital inflammation, ...
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#1George E Fragoulis (UoA: National and Kapodistrian University of Athens)H-Index: 16
#2Gerasimos Evangelatos (UoA: National and Kapodistrian University of Athens)H-Index: 4
Last. Maria G Tektonidou (UoA: National and Kapodistrian University of Athens)H-Index: 45
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IgG4-related disease (IgG4-RD) can affect almost every tissue/organ. Vascular involvement in the setting of IgG4-RD is increasingly being recognized with most of the cases pertaining patients with aortitis and/or periaortitis with or without aneurysm formation. However, it is now evident that other vessels like iliac arteries, coronary arteries, carotids, and others can also be affected being often underdiagnosed. Vascular involvement is expressed as vessel wall thickening, while aneurysm format...
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#1Yoshinori Takahashi (Kanazawa University)H-Index: 3
#2Ichiro Mizushima (Kanazawa University)H-Index: 13
Last. Mitsuhiro Kawano (Kanazawa University)H-Index: 34
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Objectives In IgG4-related dacryoadenitis and/or sialadenitis (IgG4-DS), involvement of two or more sets of lacrimal glands (LGs) and/or major salivary glands (MSGs) is regarded as a specific finding with diagnostic significance. This study aimed to clarify the influence of this factor on the overall clinical picture of IgG4-DS. Methods We retrospectively reviewed the medical records of 130 patients with IgG4-related disease, 97 of whom were diagnosed with IgG4-DS. We determined their clinical f...
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#1Aamina T Shakir (University of Oklahoma Health Sciences Center)
Last. Guha Krishnaswamy (Wake Forest University)H-Index: 5
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Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory disease characterised by multiorgan lymphoplasmacytic infiltration, obliterative phlebitis and storiform fibrosis. It can be associated with cardiovascular pathology. The objective of this narrative review is to summarise the published literature on cardiovascular manifestations of IgG4-RD and to provide a basis for diagnosis and management of the condition by the practising cardiologist. We propose the following categor...
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#1Annarosa Floreani (UNIPD: University of Padua)H-Index: 59
#2Kazuichi Okazaki (Kansai Medical University)H-Index: 85
Last. M. Eric Gershwin (UC Davis: University of California, Davis)H-Index: 139
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IgG4-related disease (IgG4-RD) represents an immune-mediated fibroinflammatory condition with peculiar histopathologic changes that can affect various organs. In 2012 its unified nomenclature was published, which allows to abandon other synonymous names. Up to now, only little is known about its epidemiology around the world. However, although it is generally considered a rare condition, the number of patients with IgG4-RD is increasing enormously. Likewise, the annual number of publications on ...
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#1Shokoufeh Hajsadeghi (IUMS: Iran University of Medical Sciences)H-Index: 6
#2Marziyeh Pakbaz (IUMS: Iran University of Medical Sciences)H-Index: 1
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The IgG4-related disease is a distinct, steroid-responsive fibro-inflammatory disorder of unknown etiology. This multiorgan disease is characterized by tumefactive lesions that contain rich infiltrations of IgG4-positive plasma cells, with the pancreas, and the salivary and lacrimal glands being the main involved. The more common cardiovascular involvements include inflammatory peri-aortitis, coronary arteritis, and pericarditis. Intra-cardiac tumefactive lesions are rarely reported. Herein, we ...
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#1T. Lenfant (University of Paris)
#2J. Moroch (University of Paris)
Last. Patricia Senet (University of Paris)H-Index: 14
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Resume Introduction Les phenomenes de Raynaud secondaires sont exceptionnellement rapportes au cours de la maladie associee aux IgG4. Nous en rapportons une observation et detaillons les nouveaux criteres diagnostiques de la maladie associee aux IgG4. Observation Un homme de 47 ans sans antecedent presentait un phenomene de Raynaud des mains, bilateral, d'apparition recente, sans trouble trophique. Les examens vasculaires revelaient des thromboses arterielles multiples et distales sans anomalie ...
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#1Sanjiv Sharma (AIIMS: All India Institute of Medical Sciences)H-Index: 18
#2Niraj Nirmal Pandey (AIIMS: All India Institute of Medical Sciences)H-Index: 5
Last. Sheragaru Hanumanthappa Chandrashekhara (AIIMS: All India Institute of Medical Sciences)H-Index: 8
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Aortitis includes conditions with infectious or non-infectious etiology, characterized by inflammatory changes in one or more layers in aortic wall. Age at onset, geographic predilections, distribution and pattern of involvement in aorta, its branches and pulmonary arteries, and systemic associations provide a clue to etiology. Clinical presentations are often non-specific. An integrated approach including clinical, laboratory and imaging assessment is essential to confirm diagnosis and plan tre...
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