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

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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“…Mediastinal involvement in IgG4-RD can also manifest mediastinal mass or fibrosing mediastinitis. 94,[112][113][114][115]…”

Section: Mediastinal Diseasementioning

confidence: 99%

Thoracic Involvement in IgG4-Related Disease

Moura

Gripaldo

Baqir

et al. 2020

Semin Respir Crit Care Med

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“…■ Because the symptom severity of patients with FM is variable, treatment should be individualized, and the therapeutic approach should be tailored to alleviate compression of the affected mediastinal structures. A subset of patients with nongranulomatous FM exhibit histopathologic and immunologic features that fall within the spectrum of immunoglobulin G4-related disease (IgG4-RD) and have additional associated diseases such as retroperitoneal fibrosis, sclerosing cholangitis, autoimmune pancreatitis, and Riedel thyroiditis (16,(24)(25)(26). IgG4-RD is a protean multiorgan immune-mediated fibroinflammatory condition that may mimic various infections, inflammatory processes, or malignancies and may affect nearly any organ.…”

Section: Teaching Pointsmentioning

confidence: 99%

Multimodality Imaging of Focal and Diffuse Fibrosing Mediastinitis

Garrana

Buckley

Rosado-de-Christenson

et al. 2019

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Fibrosing mediastinitis is a rare benign but potentially life-threatening process that occurs because of proliferation of fibrotic tissue in the mediastinum. The focal subtype is more common and typically is associated with an abnormal immunologic response to Histoplasma capsulatum infection. Affected patients are typically young at presentation, but a wide age range has been reported, without a predilection for either sex. The diffuse form may be idiopathic or associated with autoimmunity, usually affects middle-aged and/or elderly patients, and is more common in men. For both subtypes, patients present with signs and symptoms related to obstruction or compression of vital mediastinal structures. The most common presenting signs and symptoms are cough, dyspnea, recurrent pneumonia, hemoptysis, and pleuritic chest pain. Patients with the diffuse subtype may have additional extrathoracic symptoms depending on the other organ systems involved. Because symptom severity is variable, treatment should be individualized with therapies tailored to alleviate compression of the affected mediastinal structures. Characteristic imaging features of fibrosing mediastinitis include infiltrative mediastinal soft tissue (with or without calcification) with compression or obstruction of mediastinal vascular structures and/or the aerodigestive tract. When identified in the appropriate clinical setting, these characteristic features allow the radiologist to suggest the diagnosis of fibrosing mediastinitis. Careful assessment is crucial at initial and follow-up imaging for exclusion of underlying malignancy, assessment of disease progression, identification of complications, and evaluation of treatment response.

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“…The majority of cases of FM are thought to result from infection with the dimorphic fungus Histoplasmosis capsulatum which commonly lives in the soil of the southeastern and central United States. Other potential triggers include tuberculosis, sarcoidosis, autoimmune, and idiopathic processes such as IgG4 related disease . Histoplasmosis infection is near ubiquitous in residents of endemic regions and is typically asymptomatic .…”

Section: Etiologymentioning

confidence: 99%

“…Other potential triggers include tuberculosis, sarcoidosis, autoimmune, and idiopathic processes such as IgG4 related disease. [7][8][9][10] Histoplasmosis infection is near ubiquitous in residents of endemic regions and is typically asymptomatic. 3,11 Infection triggers a potent cell-mediated immunologic response within the mediastinal lymph nodes.…”

Section: Etiologymentioning

confidence: 99%

Catheter based treatments for fibrosing mediastinitis

Fender

Widmer

Koepsel

et al. 2019

Cathet Cardio Intervent

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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 interventions aimed at alleviating obstruction can provide significant palliation, surgical interventions are challenging with high mortality and clinical experience with percutaneous interventions is limited. Here, we will review the presentation, natural history, and treatment of fibrosing mediastinitis, placing particular emphasis on catheter‐based therapies.

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

Cited by 30 publications

References 32 publications

Thoracic Involvement in IgG4-Related Disease

Thoracic Involvement in IgG4-Related Disease

Multimodality Imaging of Focal and Diffuse Fibrosing Mediastinitis

Catheter based treatments for fibrosing mediastinitis

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