Dataset Information

IgG4-related aortitis/periaortitis and periarteritis: a distinct spectrum of IgG4-related disease.

ABSTRACT: BACKGROUND:Large vessels could be involved in immunoglobulin (Ig)-G4-related disease (IgG4-RD). This study aimed to clarify the clinical features and evaluate the treatment efficacy for IgG4-RD with aortitis/periaortitis and periarteritis (PAO/PA). METHODS:This study prospectively enrolled 587 patients with IgG4-RD with a follow-up time of more than 6?months. The distribution of IgG4-related PAO/PA was classified into four types: type 1, thoracic aorta; type 2a, abdominal aorta; type 2b, abdominal aorta and iliac artery; type 2c, iliac artery; type 3, thoracic and abdominal aorta; and type 4, other arteries. Patient's demographic data, clinical characteristics, laboratory parameters, and treatment efficacy were analyzed. RESULTS:Of 587 IgG4-RD patients, 89 (15.2%) had PAO/PA. The average age was 58.3?±?11.1?years, with male predominance (85.4%). Vessels affected were as follows: abdominal aorta (83.1%), iliac artery (70.8%), thoracic aorta (13.5%), and other vessels (13.5%). The most prevalent distribution type of IgG4-related PAO/PA was type 2b, with 74 (83.1%) patients, followed by type 2a, type 2c, type 3, and type 1. Fifty-five (61.8%) PAO/PA patients had hydronephrosis, with renal insufficiency occurring in 43 (48.3%), and 31 (34.8%) PAO/PA patients had D-J stent drainage due to severe ureteral obstruction. After treatment with a glucocorticoid and immunosuppressants, 82% patients achieved remission with shrinking of the perivascular mass by more than 30%. CONCLUSIONS:IgG4-RD with PAO/PA was distinct from non-PAO/PA in demographic features, organ involvement distribution, inflammatory markers, and serum IgG4 and IgE. The most common affected vessel was the abdominal aorta, and most patients responded well with treatment.

SUBMITTER: Peng L

PROVIDER: S-EPMC7197178 | biostudies-literature | 2020 May

REPOSITORIES: biostudies-literature

ACCESS DATA

Publications

IgG4-related aortitis/periaortitis and periarteritis: a distinct spectrum of IgG4-related disease.

Peng Linyi L Zhang Panpan P Li Jieqiong J Liu Zheng Z Lu Hui H Zhu Liang L Wang Xiaorong X Teng Fei F Li Xuemei X Guo Huifang H Fei Yunyun Y Zhang Wen W Zhao Yan Y Zeng Xiaofeng X Zhang Fengchun F

Arthritis research & therapy 20200504 1

<h4>Background</h4>Large vessels could be involved in immunoglobulin (Ig)-G4-related disease (IgG4-RD). This study aimed to clarify the clinical features and evaluate the treatment efficacy for IgG4-RD with aortitis/periaortitis and periarteritis (PAO/PA).<h4>Methods</h4>This study prospectively enrolled 587 patients with IgG4-RD with a follow-up time of more than 6 months. The distribution of IgG4-related PAO/PA was classified into four types: type 1, thoracic aorta; type 2a, abdominal aorta; t ...[more]

PMID: 32366271

Similar Datasets

Project description:BackgroundChest pain is a common reason for admission to the Emergency Department and aortic disease is a relatively frequent cause amongst the total number of admissions due to chest pain. IgG4-related disease (IgG4-RD) is a multi-organ immune-mediated condition that mimics several malignant, infectious, and inflammatory disorders.Case summaryWe report a rare case of IgG4-related aortitis complicated with severe aortic regurgitation and multivessel coronary artery disease in a 64-year-old man with a history of atypical chest pain. The diagnosis was made performing transthoracic echocardiography, transoesophageal echocardiography, and left heart catheterization; the aortitis was an incidental finding discovered by computed tomography angiography. Unusually, the positron emission tomography-computed tomography (PET-CT) scans did not reveal metabolic activity in the aortic wall. This last finding prompted us to exclude more aggressives arteritis (such as Horton's disease or Takayasu arteritis); syphilis infection and other infective or autoimmune diseases were excluded with laboratory tests. The patient underwent cardiac surgery with replacement of both the aortic valve and the ascending aorta, also performing a coronary aortic bypass graft (CABG). Despite the PET-CT scans were negative in the aortic wall, the histological specimens showed diffuse lymphoid infiltration, fibro-atheromatosis lesions, and medium-interstitial hyperplasia compatible with aortic atherosclerosis and IgG4-RD. The post-surgery course was free of complications and the patient was discharged in good clinical condition. He was referred to the Rheumatologic Department and a corticosteroid therapy has been started.DiscussionThe growing recognition of IgG4-related systemic disease as a clinical entity underscores the importance of considering this diagnosis in patients with any type of idiopathic aortitis and aortic valve disease. Noteworthy, the PET-CT scans could be negative in this disease, so histological exams are mandatory to make the diagnosis; in our case an unusual post-surgery histopathological finding prompted us to diagnose a rare, unrecognized disease and allowed us to treat the patient properly.

Project description:The main IgG4 antibody-mediated neurological disorders (IgG4-ND) include MuSK myasthenia; CIDP with nodal/paranodal antibodies to Neurofascin-155, contactin-1/caspr-1, or pan-neurofascins; anti-LGI1 and CASPR2-associated limbic encephalitis, Morvan syndrome, or neuromyotonia; and several cases of the anti-IgLON5 and anti-DPPX-spectrum CNS diseases. The paper is centered on the clinical spectrum of IgG4-ND and their immunopathogenesis highlighting the unique functional effects of the IgG4 subclass compared to IgG1-3 antibody subclasses. The IgG4 antibodies exert pathogenic effects on their targeted antigens by blocking enzymatic activity or disrupting protein-protein interactions affecting signal transduction pathways, but not by activating complement, binding to inhibitory FcγRIIb receptor or engaging in cross-linking of the targeted antigen with immune complex formation as the IgG1-IgG3 antibody subclasses do. IgG4 can even inhibit the classical complement pathway by affecting the affinity of IgG1-2 subclasses to C1q binding. Because the IgG4 antibodies do not trigger inflammatory processes or complement-mediated immune responses, the conventional anti-inflammatory therapies, especially with IVIg, immunosuppressants, and plasmapheresis, are ineffective or not sufficiently effective in inducing long-term remissions. In contrast, aiming at the activated plasmablasts connected with IgG4 antibody production is a meaningful therapeutic target in IgG4-ND. Indeed, data from large series of patients with MuSK myasthenia, CIDP with nodal/paranodal antibodies, and anti-LGI1 and CASPR2-associated syndromes indicate that B cell depletion therapy with rituximab exerts long-lasting clinical remissions by targeting memory B cells and IgG4-producing CD20-positive short-lived plasma cells. Because IgG4 antibody titers seem reduced in remissions and increased in exacerbation, they may serve as potential biomarkers of treatment response supporting further the pathogenic role of self-reacting B cells. Controlled trials are needed in IgG4-ND not only with rituximab but also with the other anti-B cell agents that target CD19/20, especially those like obexelimab and obinutuzumab, that concurrently activate the inhibitory FcγRIIb receptors which have low binding affinity to IgG4, exerting a more prolonged anti-B cell action affecting also antigen presentation and cytotoxic T cells. Antibody therapies targeting FcRn, testing those anti-FcRn inhibitors that effectively catabolize the IgG4 antibody subclass, may be especially promising.

Dataset Information

IgG4-related aortitis/periaortitis and periarteritis: a distinct spectrum of IgG4-related disease.

Publications

IgG4-related aortitis/periaortitis and periarteritis: a distinct spectrum of IgG4-related disease.

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure

Tweets