Thoracic Involvement in IgG4-Related Disease.

Published on Apr 1, 2020in Seminars in Respiratory and Critical Care Medicine2.028
· DOI :10.1055/S-0039-1700995
Marta Casal Moura3
Estimated H-index: 3
(Mayo Clinic),
Ria Gripaldo (Emory University)+ 1 AuthorsJay H. Ryu80
Estimated H-index: 80
(Mayo Clinic)
Sources
Abstract
Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory disorder that has been recognized to involve virtually any organ in the body and typically manifests mass-like lesions (tumefactive). Although initial reports of this disease (autoimmune pancreatitis [AIP]) were described in the Japanese population, it has since been reported worldwide. It is most commonly seen in adults of middle age or older, more often men than women. The pathogenesis of IgG4-RD is largely unknown, but genetic factors, microorganisms, and autoimmunity are thought to play important roles. Serum IgG4 concentration is elevated in the majority of patients with IgG4-RD but is a nonspecific finding. Characteristic histopathologic features include dense lymphoplasmacytic infiltrate, fibrosis (often in storiform pattern), and obliterative phlebitis. Lung involvement in IgG4-RD was first reported in 2004 in two patients with AIP and coexisting interstitial lung disease. Since then, a wide spectrum of intrathoracic involvement has been reported and includes not only parenchymal lung diseases but also pleural, airway, vascular, and mediastinal lesions. Thoracic involvement in IgG4-RD is often found incidentally during the workup of extrathoracic lesions but can sometimes be the presenting abnormality. The diagnosis of IgG4-RD requires correlation of clinical, laboratory, imaging, and histopathologic features. Glucocorticoids are the first-line therapy but other options including B cell depletion are being investigated. IgG4-RD is generally associated with an indolent clinical course and most patients improve with glucocorticoid therapy.
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#2C. Terao (University of Shizuoka)H-Index: 5
AbstractIgG4-related disease (IgG4-RD) is an emerging concept of a novel clinical entity, characterized by the swelling of the affected organs, increase in serum total IgG and IgG4 levels, infiltration of plasmacyte and eosinophil, fibrosis of the affected lesions and good response to corticosteroid. IgG4-RD includes diseases with organ-specific fibrosis and infiltration of IgG4-positive plasmacyte, previously known as type 1 autoimmune pancreatitis, Mikulicz’s disease and others. Although the p...
6 CitationsSource
#1Angelica Torres Morales (UM: University of Miami)H-Index: 1
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Last. Mehdi Mirsaeidi (UM: University of Miami)H-Index: 29
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Abstract IgG4-related disease (IgG4-RD) is an autoimmune disorder characterized by substantial infiltration of plasma cells with IgG4 in target organs. Lung manifestations predominantly present as inflammatory pseudotumor, interstitial pneumonitis, organizing pneumonia, and lymphomatoid granulomatosis. There is no specific diagnostic test for IgG4-related lung disease (IgG4-RLD), and excluding diseases that mimic IgG4-RLD is important. Corticosteroids with or without disease-modifying anti-rheum...
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Last. Massimiliano PaciH-Index: 14
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We report a rare case of a IgG4-related disease presenting with recurrent pleural effusion, pleural thickness and multiple mediastinal lymphadenopathies and no involvement of other extrathoracic organs. A 65-year-old man with a previous asbestos exposure presented with cough and pain discomfort. A large right pleural effusion was detected and evacuated (siero-haematic liquid). With the suspicious of a pleural mesothelioma, a CT-scan before and a 18F-FDG PET/CT-scan later were performed revealing...
1 CitationsSource
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Objective IgG4-related disease (IgG4-RD) is a heterogeneous, multiorgan condition of unclear aetiology that can cause organ failure. Difficulty recognising IgG4-RD contributes to diagnostic delays. We sought to identify key IgG4-RD phenotypes. Methods We used two cross-sectional studies assembled by an international, multispecialty network of IgG4-RD specialists who submitted 765 cases to derive and replicate phenotypic groups. Phenotype groups of disease manifestations and key covariate distrib...
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#1Shoko Matsui (University of Toyama)H-Index: 22
AbstractIgG4-related diseases (IgG4-RDs), such as autoimmune pancreatitis and IgG4-related Mikulicz disease, are often accompanied by intrathoracic lesions, which are called IgG4-related respirator...
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: IgG4-related disease (IgG4-RD) is a fibroinflammatory disorder recognized as a novel clinical entity with either synchronous or metachronous multi-organ involvement. Patients with IgG4-RD show diffuse or focal organ enlargement and mass-forming or nodular/thickened lesions with abundant infiltration of IgG4-positive plasmacytes and fibrosis, and such patients respond well to steroid treatment. It should be differentiated from mimics by a combination of serum IgG4 level, imaging features, and h...
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#1Zachary S. Wallace (Harvard University)H-Index: 25
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Last. John H. Stone (Harvard University)H-Index: 76
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OBJECTIVE: IgG4-related disease (IgG4-RD) can cause fibroinflammatory lesions in nearly any organ, leading to organ dysfunction and failure. The IgG4-RD Responder Index (RI) was developed to help investigators assess the efficacy of treatment in a structured manner. The aim of this study was to validate the RI in a multinational investigation. METHODS: The RI guides investigators through assessments of disease activity and damage in 25 domains, incorporating higher weights for disease manifestat...
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#1Eunhee S. Yi (Mayo Clinic)H-Index: 47
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Last. Thomas V. Colby (Mayo Clinic)H-Index: 105
view all 5 authors...
Immunoglobulin(Ig)G4-related disease (IgG4-RD) is a fibroinflammatory condition that can affect virtually any organ and usually presents as tumefactive lesions involving multiple sites. Characteristic histopathology of IgG4-RD consists of dense lymphoplasmacytic infiltrate, fibrosis (often in storiform pattern), and obliterative phlebitis, accompanied by tissue infiltration of IgG4-positive plasma cells with or without elevation of serum IgG4 level. Despite a general similarity in the morphologi...
4 CitationsSource
#1Deng Hui (Peking Union Medical College)H-Index: 1
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Last. Xinying XueH-Index: 8
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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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