Fibrosing mediastinitis (FM) and IgG4-related disease (IgG4-RD) are two fibroinflammatory disorders with potentially overlapping clinical and radiological features. In this paper, we looked for histopathologic features of IgG4-RD and enumerated infiltrating IgG4-positive plasma cells within mediastinal tissue biopsies from FM patients. We identified 15 consecutive FM surgical mediastinal tissue biopsies between 1985 and 2006. All patients satisfied the clinical and radiological diagnostic criteria for FM. All patients had either serological or radiological evidence of prior histoplasmosis or granulomatous disease, respectively. Formalin-fixed paraffin-embedded tissue sections of all patients were stained for H&E, IgG, and IgG4. Three samples met the predefined diagnostic criteria for IgG4-RD. In addition, characteristic histopathologic changes of IgG4-RD in the absence of diagnostic numbers of tissue infiltrating IgG4-positive plasma cells were seen in a number of additional cases (storiform cell-rich fibrosis in 11 cases, lymphoplasmacytic infiltrate in 7 cases, and obliterative phlebitis/arteritis in 2 cases). We conclude that up to one-third of histoplasmosis or granulomatous-disease-associated FM cases demonstrate histopathological features of IgG4-RD spectrum. Whether these changes occur as the host immune response against Histoplasma or represent a manifestation of IgG4-RD remains to be determined. Studies to prospectively identify these cases and evaluate their therapeutic responses to glucocorticoids and/or other immunosuppressive agents such as rituximab are warranted.
IgG4-related systemic disease (IgG4-RSD) is an emerging clinical entity about which much remains to be elucidated, in terms of its aetiology, pathogenesis, diagnosis, treatment and outcome. Autoimmune pancreatitis (AIP) and Mikulicz disease (MD) are the two major, well-studied constituents of IgG4-RSD. AIP and MD have common characteristics of forming tumour-mimicking lesions that consist of lymphoplasmacytic infiltrates and fibrosclerosis with numerous immunoglobulin G4 (IgG4)-positive plasma c...
Objective T-helper type 2 responses are crucial in Churg–Strauss syndrome (CSS) and may enhance the production of IgG4 antibodies. The authors assessed the IgG4 immune response in CSS patients. Methods The authors included 46 consecutive patients with CSS (24 with active and 22 with quiescent disease), 26 with granulomatosis with polyangiitis (GPA, Wegener9s), 25 with atopic asthma and 20 healthy controls and determined serum IgG, IgM, IgA, IgE and IgG subclass levels. Tissue infiltration by IgG...
Summary Retroperitoneal fibrosis, inflammatory aortic aneurysm, and pericardial and mediastinal fibrosis are characterized by infiltration of immuno-inflammatory cells and deposition of thickened fibrous tissues. Several recent studies suggested that an immunoglobulin-G4 (IgG4)-related immunological mechanism may play a role in these diseases. By searching the clinical database of patients admitted to our department between 2000 and 2010, we summarized the clinical data of 11 patients who were d...
PURPOSE OF REVIEW: Provide an update of the developments in IgG4 related disease within the last year in terms of nomenclature, diagnostic criteria and clinical features. RECENT FINDINGS: The number of diseases associated with IgG4 related disease continues to grow. The entities of idiopathic orbital inflammation, idiopathic cervical fibrosis, eosinophilic angiocentric fibrosis, reactive nodular fibrous pseudotumor, sclerosing mesenteritis, and membranous glomerulonephritis should all be added t...
AbstractIgG4-related systemic disease is now recognized as a systemic disease that may affect various organs. The diagnosis is usually made in patients who present with elevated IgG4 in serum and tissue infiltration of diseased organs by numerous IgG4+ plasma cells, in the absence of validated diagn
Fibrosing mediastinitis (FM) is a rare disorder characterized by the invasive proliferation of fibrous tissue within the mediastinum. FM frequently results in the compression of vital mediastinal structures and has been associated with substantial morbidity and mortality. Its pathogenesis remains unknown. However, in North America most cases are thought to represent an immune-mediated hypersensitivity response to Histoplasma capsulatum infection. To characterize the clinical disease spectrum, na...
The rapidly emerging disorder now known as IgG4-related disease (IgG4-RD) includes a variety of clinical entities once regarded as being entirely separate diseases. Manifestations of IgG4-RD have now been reported in essentially all organ systems. Regardless of which organ is involved, tissue biopsies reveal striking histopathological similarities. The hallmark pathology findings are diffuse lymphoplasmacytic infiltrates, abundant IgG4-positive plasma cells, modest tissue eosinophilia, and exten...
Background IgG4-related systemic fibrosclerosis is a recently defined disorder characterised by a diffuse or tumefactive inflammatory reaction rich in IgG4-positive plasma cells associated with sclerosis and obliterative phlebitis. Although characteristic histopathological features are essential for the diagnosis of these disorders, to date there exists no consensus regarding the cut-off values used to define a ‘significant IgG4-positive plasma cell count,’ and data regarding the distribution of...
Purpose of review IgG4-related systemic disease (ISD) is a recently recognized syndrome affecting multiple organs. Autoimmune pancreatitis (AIP) is the pancreatic manifestation of ISD and mimics pancreatic cancer. Current data show frequent association with serum IgG4 elevation and other serologic abnormalities. Here we explore the diagnostic and possible prognostic utility and pathogenetic implications of serologic abnormalities in ISD. Recent findings Serum IgG4 elevations (>140 mg/dl) are see...
Purpose of reviewTo summarize the existing knowledge of various clinical presentations of IgG4-related systemic disease (IgG4-RSD) and to review the evolving list of organs affected by IgG4-RSD.Recent findingsThe term IgG4-RSD encompasses a variety of clinical entities once regarded as being entirel
Although fibrotic disorders are frequently assumed to be linked to TH2 cells, quantitative tissue interrogation studies have rarely been performed to establish this link and certainly many fibrotic diseases do not fall within the type 2/allergic disease spectrum. We have previously linked two human autoimmune fibrotic diseases, IgG4-related disease and systemic sclerosis, to the clonal expansion and lesional accumulation of CD4+CTLs. In both these diseases TH2 cell accumulation was found to be s...
#1Ha Nguyen(City of Hope National Medical Center)H-Index: 1
#2Joo Y. Song(City of Hope National Medical Center)H-Index: 17
Abstract There are multiple entities that involve the lung that have radiographic, clinical, and morphologic overlaps with pulmonary lymphoma. In this review, we will discuss these entities in detail and provide relevant updates.
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, bu...
The chapter on Nonneoplastic Thymic Lesions will cover the epidemiological, etiological and clinicopathological features of common thymic lesions (congenital and acquired cysts, including dysplasia); thymic follicular, rebound and “true” hyperplasia) as well as rare or recently described non-neoplastic thymic alterations that may form mass lesions and can mimic thymic neoplasms (“Lesa-like thymic hyperplasia”; “Cholesteroloma”; Fibrosing mediastinitis, including IgG4-related disease). Criteria c...
#1Franklin A. Argueta(University of Texas Health Science Center at San Antonio)H-Index: 1
#2David Villafuerte(University of Texas Health Science Center at San Antonio)H-Index: 1
Last. Carlos S. Restrepo(University of Texas Health Science Center at San Antonio)H-Index: 22
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Abstract Fibrosing mediastinitis is a rare disorder characterized by the invasive proliferation of fibrous tissue within the mediastinum. This fibrosis can result in compression of intrathoracic structures including the pulmonary vasculature leading to clinical symptoms and complications like pulmonary hypertension. Here, we present two cases of young patients with fibrosing mediastinitis complicated by pulmonary artery stenosis requiring medical and endovascular management with excellent result...
Thymic immunoglobulin G4 (IgG4) is a rare entity that can mimic anterior mediastinal tumors. Preoperative diagnosis is essential to prevent unnecessary surgery. Little is known about disease presentation, diagnosis, and response to therapy. A 40-year-old man presented with retrosternal chest pain. A roentgenogram showed an anterior mediastinal mass. Intraoperatively, dense adhesions to the phrenic nerve, aorta, and pulmonary artery deemed the tumor unresectable. Thymic IgG4 disease was diagnosed...
Abstract Fibrosing mediastinitis is an uncommon benign disorder in which a chronic inflammatory reaction results in diffuse fibrosis of the mediastinum, potentially compromising the airways, great vessels and other mediastinal structures. Herein we describe a progressive course of fibrosing mediastinitis in a 72-year-old man. Computed tomography images depicted a diffuse, infiltrative, soft tissue mass involving the esophagus and superior vena cava in the mediastinum. Magnetic resonance imaging ...
#1Yong Zhou(SRRSH: Sir Run Run Shaw Hospital)H-Index: 1
#1Yong Zhou(UCSF: University of California, San Francisco)H-Index: 3
Last. Xiaohong Wu(SRRSH: Sir Run Run Shaw Hospital)H-Index: 5
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AbstractBackground:Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized, immune-mediated chronic fibrotic inflammation that can involve almost all organs, causing tumefaction and dysfunction. Its presence in pulmonary circulation is underestimated and has not yet been investigated.Ob
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...