Immunoglobulin G4-Related Disease and the Lung

Ryu, Jay H.; Yi, Eunhee S.

doi:10.1016/j.ccm.2016.04.017

Cited by 15 publications

(14 citation statements)

References 89 publications

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“…Most of the lung involvement presented in the form of nodular lesions in the lungs, non-specific infiltrates, "ground-glass" appearance with pleura thickening, and effusion ( Table 2). The pulmonary manifestation of IgG4-RD according to anatomic compartments can generally be categorised as parenchyma (nodules, masses, and interstitial lung disease), airway (stenosis, endobronchial mass, and bronchospastic disease), vasculature (vasculitis and pulmonary hypertension), mediastinum (lymphadenopathy and fibrosing mediastinitis), and pleural (thickening mass, and effusion) involvement [11].…”

Section: Discussionmentioning

confidence: 99%

“…Although GPA is not part of IgG4-RD, it is worth mentioning that in the context of OOI, it is an important differential diagnosis (which is why it was included in our analysis). It is important to exclude other diseases from the same group (diseases with multisystem involvement and diseases with pulmonary inflammation and increased IgG4 plasma cells), such as connective tissue diseases with lung involvement, sarcoidosis, eosinophilic granulomatous with polyangiitis (Churg-Strauss), multicentric Castelman's disease, and Rosai-Dorfman disease [11]. The results of the most relevant studies are presented in Tables 4 and 5.…”

Section: Discussionmentioning

confidence: 99%

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Cardiovascular and Lung Involvement in Patients with Autoimmune Pancreatitis

Nikolic

Brehmer

Panić

et al. 2020

JCM

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Introduction: Immunoglobulin G4-related disease (IgG4-RD) is a systemic immune-mediated disease characterised pathologically by the infiltration of IgG4-bearing plasma cells into the involved organs. Autoimmune pancreatitis (AIP) is a form of chronic pancreatitis with a heavy lymphocytic infiltration and two distinct histopathological subtypes, namely: lymphoplasmacytic sclerosing pancreatitis (AIP type 1) and idiopathic duct-centric pancreatitis (AIP type 2). Lung involvement and aortic involvement have been reported in 12% and 9% of patients with systemic IgG4-RD, respectively. In series including patients with AIP, both lung and aortic involvement were described in 2% of the patients. Most of the epidemiological data come from Japan, and there is a lack of information from Europe, especially the Scandinavian countries. Patients and methods: We performed a single-centre retrospective study on a prospectively collected cohort of patients diagnosed with AIP at the Department for Digestive Diseases at Karolinska University Hospital in Stockholm, Sweden, from 2004 to 2019. Demographic and clinical data were collected from the medical charts. Results: One hundred and thirty-three patients with AIP were analysed. Six patients were excluded because they lacked some of the clinical data relevant to the study. Demographic and clinical features of 127 patients were presented. There were 98 patients with AIP type 1-35 (35.7%) female and 63 (64.3%) male, with a mean age of 55.4 ± 18.2. Among them, 15 (15.3%) patients had lung and/or cardiovascular involvement-11 (11.2%) patients had lung involvement, 10 (10.2%) patients had cardiovascular involvement (six patients had both). Most of them (67.0%) had never smoked. The mean follow-up time of the patients with AIP type 1 was 49 months. Conclusions: Lung and/or cardiovascular involvement were diagnosed in 15 (15.3%) patients in our historical cohort of patients with AIP type 1. Most of the lung involvement was presented in the form of nodular lesions in the lungs, non-specific infiltrates, “ground-glass” appearance with pleura thickening, and effusion. Aortic involvement was a major form of vascular involvement in patients with AIP, as in previous published studies on patients with IgG4-RD.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Cardiovascular and Lung Involvement in Patients with Autoimmune Pancreatitis

Nikolic

Brehmer

Panić

et al. 2020

JCM

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“…Pulmonary function testing in patients with thoracic IgG4-RD may yield normal or abnormal results depending on the pattern of intrathoracic involvement. [122][123][124] Intrathoracic lymphadenopathy is unlikely to be associated with abnormal pulmonary function results, whereas parenchymal or pleural disease will likely be associated with a restrictive pattern of impairment. Airway disease will produce airflow obstruction, while pulmonary vascular disease may manifest only a reduced diffusing capacity.…”

Section: Laboratory and Pulmonary Function Testsmentioning

confidence: 99%

“…129 However, BAL data regarding IgG4-RLD are sparse, and the role of BAL in the evaluation and management of these patients remains to be clarified. 124 18 F-FDG-PET/CT can be used for a comprehensive assessment of organ involvement and has been proposed as an additional diagnostic tool in IgG4-RD. [131][132][133][134] Clinically, 18 F-FDG-PET/CT can also be useful for the selection of biopsy site that is most likely to be diagnostically informative as well as in assessing response to treatment and monitoring for disease relapse.…”

Section: Diagnosismentioning

confidence: 99%

Thoracic Involvement in IgG4-Related Disease

Moura

Gripaldo

Baqir

et al. 2020

Semin Respir Crit Care Med

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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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“…83 These manifestations can be asymptomatic, discovered on routine imaging, or present with nonspecific complaints of cough, chest pain, or dyspnea. 84 Imaging features (Figure 4), barring the finding of paravertebral band-like soft-tissue thickening, are nonspecific and can resemble those of almost any pulmonary disease.…”

Section: Non-gastrointestinal Tract Manifestationsmentioning

confidence: 99%

Gastrointestinal and Extra-Intestinal Manifestations of IgG4–Related Disease

et al. 2018

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IgG4-related disease (IgG4-RD) is a chronic relapsing multi-organ fibro-inflammatory syndrome of presumed autoimmune etiology. It is characterized by increased serum levels of IgG4 and tissue infiltration by IgG4 cells. Increased titers of autoantibodies against a spectrum of self-antigens and response to steroids have led to its characterization as an autoimmune disease. However, the pathognomonic antigens probably differ among manifestations, and different antigens or autoantibodies produce similar immune reactions in different organs. Little is known about the pathogenic effects, if any, of serum IgG4 or IgG4 plasma cells in tissues. Despite several animal models of the disease, none truly recapitulates human IgG4-RD. Histologic analyses of tissues from patients with IgG4-RD reveal a dense lymphoplasmacytic infiltrate rich in IgG4 plasma cells, storiform fibrosis, and obliterative phlebitis, although these features vary among organs. Typical presentation and imaging findings include mass-forming synchronous or metachronous lesions in almost any organ, but most commonly in the pancreas, bile duct, retroperitoneum, kidneys, lungs, salivary and lacrimal glands, orbit, and lymph nodes. In all organs, inflammation can be reduced by corticosteroids and drugs that deplete B cells, such as rituximab. Patients with IgG4-RD have relapses that respond to primary therapy. Intense fibrosis accompanies the inflammatory response, leading to permanent organ damage and insufficiency. Death from IgG4-RD is rare. IgG4-RD is a multi-organ disease with predominant pancreatico-biliary involvement. Despite its relapsing-remitting course, patients have an excellent prognosis.

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Immunoglobulin G4-Related Disease and the Lung

Cited by 15 publications

References 89 publications

Cardiovascular and Lung Involvement in Patients with Autoimmune Pancreatitis

Cardiovascular and Lung Involvement in Patients with Autoimmune Pancreatitis

Thoracic Involvement in IgG4-Related Disease

Gastrointestinal and Extra-Intestinal Manifestations of IgG4–Related Disease

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