Idiopathic fibrosing mediastinitis: spectrum of imaging findings with emphasis on its association with IgG4-related disease.

Published on Nov 1, 2015in Clinical Imaging1.109
· DOI :10.1016/J.CLINIMAG.2015.07.008
Luis Gorospe6
Estimated H-index: 6
Luis Gorospe1
Estimated H-index: 1
(Hospital Universitario de Canarias)
+ 4 AuthorsEva Mañas-Baena1
Estimated H-index: 1
Abstract Purpose The purpose of this study was to review the clinicoradiological features of idiopathic fibrosing mediastinitis (FM). Methods The records of six patients with idiopathic FM were retrospectively reviewed. Results The most common presenting symptom was cough. There were three localized forms and three diffuse forms. Definitive histological diagnosis was most commonly made with mediastinoscopy. Interestingly, three of our patients had disease manifestations of IgG4-related disease (IgG4-RD) outside the mediastinum. Conclusion The imaging findings of idiopathic FM vary depending on the involved mediastinal structures. Therapeutic options include medical treatment, stenting, or surgery. Radiologists should pay attention to the possible association of idiopathic FM with extrathoracic manifestations of the IgG4-RD spectrum.
📖 Papers frequently viewed together
1,443 Citations
4 Citations
46 Citations
#1Terumi KamisawaH-Index: 60
#2Yoh Zen (Kobe University)H-Index: 69
Last. John H. Stone (Harvard University)H-Index: 76
view all 4 authors...
Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is an immune-mediated condition that can affect almost any organ and is now being recognized with increasing frequency. IgG4-RD is characterized by a lymphoplasmacytic infiltrate composed of IgG4+ plasma cells, storiform fibrosis, obliterative phlebitis, and mild to moderate eosinophilia. The diagnosis of IgG4-RD unifies many eponymous fibroinflammatory conditions that had previously been thought to be confined to single organs. IgG4-RD lesions ...
561 CitationsSource
‘Downhill’ varices are located in the proximal part of the esophagus. Their etiology differs from the distal types, with most of them usually being related to superior vena cava obstruction. Although bleeding due to ‘downhill’ varices is very rare, it can be life-threatening. Here, we present a case of upper gastrointestinal bleeding due to mediastinal fibrosis associated with chest radiotherapy for seminoma metastasis sixteen years previously, which was successfully treated conservatively.
9 CitationsSource
#1Chase A Arbra (UK: University of Kentucky)H-Index: 1
#2Joseph D. Valentino (UK: University of Kentucky)H-Index: 5
Last. Jeremiah T. Martin (UK: University of Kentucky)H-Index: 17
view all 3 authors...
Fibrosing mediastinitis is a condition in which mediastinal fat is replaced by fibrous tissue. Complications may arise due to progressive fibrotic infiltration and compression of major vascular, respiratory, and nervous structures within the mediastinum. We describe 3 similar cases of fibrosing mediastinitis with pulmonary vessel involvement. Imaging and intraoperative observation revealed involvement of the pulmonary vasculature in all 3 patients. Perfusion studies showed decreased or absent pe...
2 CitationsSource
To the Editor: Fibrosing mediastinitis is an uncommon disorder characterized by inflammation and progressive fibrosis within the mediastinum (1–3). The resulting invasive obstruction of vital mediastinal structures is associated with significant morbidity (2, 4). Infectious and noninfectious inflammatory triggers have been reported (4). In North America, fibrosing mediastinitis is most commonly associated with histoplasmosis (4, 5). Although anecdotal success with antifungal, antiinflammatory, a...
20 CitationsSource
We read with keen interest the paper by Posligua et al. [(1)][1] in which the authors describe a young woman with left main and right coronary artery ostial stenosis due to an infiltrative fibrous mass involving the aortic root and arch, ascending aorta, and pulmonary artery, and extrinsically
3 CitationsSource
#1Rachel M. Owen (Emory University)H-Index: 6
#2Seth D. Force (Emory University)H-Index: 24
Last. Felix G. Fernandez (Emory University)H-Index: 25
view all 6 authors...
RESULTS: There were 32 patients, 19 men (59%) and 13 women, with a mean age of 48 years (17–78). Indications for pneumonectomy included pulmonary tuberculosis in 10 patients (31%), chronic septic lung disease in seven (22%), invasive opportunistic infections in five (16%), fibrosing mediastinitis in four (12%) and other in six (19%). Pneumonectomies were left-sided in 17 (53%) and right-sided in 15 patients; nine (28%) were completion pneumonectomies. Intraoperatively, intrapericardial isolation...
16 CitationsSource
#1Michael F. McNeeley (UW: University of Washington)H-Index: 9
#2Jonathan H. Chung (University of Colorado Denver)H-Index: 28
Last. J. David Godwin (UW: University of Washington)H-Index: 19
view all 4 authors...
OBJECTIVE. The purpose of this article is to review and illustrate the pathogenesis, natural history, imaging features, and treatment of granulomatous fibrosing mediastinitis. CONCLUSION. Granulomatous fibrosing mediastinitis is an infiltrative disorder characterized by encasement of the mediastinal viscera; in most cases in the United States, it represents an idiosyncratic immune response to histoplasma. Familiarity with the various imaging features of fibrosing mediastinitis is important for p...
32 CitationsSource
#1Tobias Peikert (Mayo Clinic)H-Index: 29
#2Bijayee Shrestha (Mayo Clinic)H-Index: 3
Last. Eunhee S. Yi (Mayo Clinic)H-Index: 47
view all 9 authors...
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 criter...
46 CitationsSource
#1Aiko Sakamoto (UTokyo: University of Tokyo)H-Index: 8
#2Ryozo Nagai (UTokyo: University of Tokyo)H-Index: 113
Last. Nobukazu Ishizaka (UTokyo: University of Tokyo)H-Index: 34
view all 17 authors...
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...
57 CitationsSource
#1Hisanori Umehara (Kanazawa Medical University)H-Index: 43
#2Kazuichi Okazaki (Kansai Medical University)H-Index: 68
Last. Hirotaka Ohara (Nagoya City University)H-Index: 42
view all 26 authors...
Background IgG4-related disease (IgG4-RD) is a novel clinical disease entity characterized by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4+ plasma cells. Although IgG4-RD is not rare and is clinically important, its clinical diagnostic criteria have not been established. Comprehensive diagnostic criteria for IgG4-RD, including the involvement of various organs, are intended for the practical use of general physicians and nonspecialists.
1,063 CitationsSource
Cited By13
#1Federica MaritatiH-Index: 13
#2Francesco PeyronelH-Index: 4
Last. Augusto Vaglio (UniFI: University of Florence)H-Index: 42
view all 3 authors...
IgG4-related disease (IgG4-RD) is a recently recognized fibro-inflammatory disorder that can affect almost any organ. Common presentations include major salivary and lacrimal gland enlargement, orbital disease, autoimmune pancreatitis, retroperitoneal fibrosis and tubulointerstitial nephritis. The main histopathological features are a dense, polyclonal, lymphoplasmacytic infiltrate rich in IgG4+ plasma cells, storiform fibrosis and obliterative phlebitis. The precise pathogenic mechanisms of IgG...
6 CitationsSource
#1Marta Casal Moura (Mayo Clinic)H-Index: 3
#2Ria Gripaldo (Emory University)
Last. Jay H. Ryu (Mayo Clinic)H-Index: 80
view all 4 authors...
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...
1 CitationsSource
#1Erin A. Fender (Mayo Clinic)H-Index: 9
#2R. Jay Widmer (Scott & White Hospital)H-Index: 2
Last. David R. Holmes (Mayo Clinic)H-Index: 146
view all 8 authors...
: Fibrosing mediastinitis is a rare, often debilitating and potentially lethal disease characterized by an exuberant fibroinflammatory response within the mediastinum. Patients typically present with insidious symptoms related to compression of adjacent structures including the esophagus, heart, airways, and cardiac vessels. Fibrosing mediastinitis is most often triggered by Histoplasmosis infection; however, antifungal and anti-inflammatory therapies are largely ineffective. While structural in...
2 CitationsSource
#1F. Yangui (Tunis University)H-Index: 2
#2A Ayadi (Tunis University)
Last. Mohamed Ridha Charfi (Tunis University)H-Index: 3
view all 4 authors...
#1Kaleigh E. Lindholm (University of Texas MD Anderson Cancer Center)H-Index: 2
#2Patricia M. de Groot (University of Texas MD Anderson Cancer Center)H-Index: 16
Last. Cesar A. MoranH-Index: 59
view all 3 authors...
: Fibrosing lesions of the mediastinum represent a small but challenging group of lesions that range in etiology from infectious to idiopathic to neoplastic. The diagnosis of such lesions becomes more challenging in the setting of mediastinoscopic biopsies. In addition, over the years, there has been further accumulation of knowledge of the clinical aspects of these lesions that needs to be incorporated into their evaluation. Therefore, it is essential that in the general evaluation of these fib...
1 CitationsSource
#1Hee Kang (Kosin University Gospel Hospital)
#2Min Jung Jung (Kosin University Gospel Hospital)
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 ...
#1Ying KanH-Index: 7
#2Leilei YuanH-Index: 6
Last. Jigang YangH-Index: 12
view all 4 authors...
: A 66-year-old man presented to our hospital because of abdominal pain for 5 days. A contrast abdominal CT raised the possibility of pancreatic carcinoma. FDG PET/CT showed increased FDG accumulation not only in the pancreas and the retroperitoneum, but also in the posterior mediastinum, which was not typical of pancreatic carcinoma. The patient was subsequently diagnosed having immunoglobulin G4-related disease following the histopathologic examination.
3 CitationsSource
Mediastinal fibrosis is a rare disease characterised by fibrous proliferation in the mediastinum. It can be idiopathic or secondary to several conditions such as infections and malignancies. Anecdotal reports have described idiopathic mediastinal fibrosis (IMF) in association with other fibro-inflammatory or autoimmune diseases. We report nine new IMF cases recently seen at our Fibro-Inflammatory Disease Clinic and reviewed the IMF cases reported in the English language literature throughout 200...
13 CitationsSource
#2Andy Abril (Mayo Clinic)H-Index: 16
Last. José A. Gómez-Puerta (UdeA: University of Antioquia)H-Index: 32
view all 3 authors...
IgG4-related disease is the term used to refer to a condition characterized by a lymphoplasmacytic infiltrate, fibrosis and an increased number of IgG4+ cells present in tissue, in most cases, with an elevated serum IgG4 level. This disease frequently affects the pancreas, salivary glands and lymph nodes, but can involve almost any tissue. Its etiology and the exact role of the different inflammatory cells in the damage to the target organ is still unclear. As yet, there is no international cons...
8 CitationsSource
#1Gabriele A. Krombach (University of Giessen)H-Index: 36
IgG4-assoziierte Erkrankungen des Bindegewebes beruhen auf einer allergenvermittelten Autoimmunreaktion und konnen das Retroperitoneum, das Mesenterium, das Mediastinum sowie seltener andere Lokalisationen (Hals, Orbita und periartikulares Bindgewebe) betreffen. Die inflammatorische Fibrosierung mit zunachst lymphozyten- und plasmazellreichem Infiltrat und zellarmer storiformer Fibrose im spateren Stadium kann durch die Stenosierung oder den Verschluss von Gefasen, Ureteren oder Darmschlingen sc...
1 CitationsSource