Thoracic involvement in IgG4-related disease in a UK-based patient cohort

Corcoran, John; Culver, Emma L.; Anstey, R M; Talwar, Arunabh; Manganis, Charis; Cargill, Tamsin; Hallifax, Robert; Psallidas, Ioannis; Rahman, Najib M.; Barnes, Eleanor

doi:10.1016/j.rmed.2017.10.005

Cited by 30 publications

(46 citation statements)

References 19 publications

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“…Patients with IgG4-RD are referred from local, regional, and national hospitals, and video-linked multidisciplinary team meetings are held every 6 weeks between the London and Oxford groups. Patients with autoimmune pancreatitis, IgG4-sclerosing cholangitis, IgG4-related lung disease, and 1 patient with IgG4-RPF in this cohort have previously been reported 15, 16, 17…”

Section: Methodsmentioning

confidence: 68%

Clinical Manifestations and Long-term Outcomes of IgG4-Related Kidney and Retroperitoneal Involvement in a United Kingdom IgG4-Related Disease Cohort

Evans

Cargill

Goodchild

et al. 2019

Kidney International Reports

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IntroductionIgG4-related disease (IgG4-RD) is a relapsing multisystem fibro-inflammatory disease, which may involve the kidney (IgG4-related kidney disease [IgG4-RKD]) and retroperitoneum (IgG4-related retroperitoneal fibrosis [IgG4-RPF]). The aim of this study was to describe IgG4-RKD and IgG4-RPF in the United Kingdom.MethodsWe conducted a retrospective observational study of patients with IgG4-RKD and IgG4-RPF in a multicenter IgG4-RD cohort. Data were collected through review of medical records. We describe clinical parameters at baseline, histological and radiological findings, treatment, and patient outcomes.ResultsOf 154 patients with IgG4-RD, 14 (9.1%) had IgG4-RKD, 10 (6.5%) had IgG4-RPF, and 4 (2.6%) had both. Patients were aged 58.2 ± 14.2 years, and 26 (92.9%) were male. Creatinine at presentation was worse in those with intrinsic renal disease (229 μmol/l vs. 110 μmol/l; P = 0.0076). Serum IgG4 was elevated in the majority of patients (87.5%), and hypocomplementemia was present in half of those with IgG4-RKD. Fifteen patients underwent renal biopsy; tubulointerstitial nephritis with abundant IgG4+ plasma cells was the most common finding (n = 14; 93.3%), and 4 (26.7%) patients had membranous nephropathy. Most patients (89.3%) were treated with corticosteroids, and 4 (16.0%) with additional azathioprine as initial management. Thirteen patients (46.4%) relapsed over 60 ± 48 months of follow-up, at median 18 (12–36) months after renal/RPF diagnosis; 61.5% of relapses were in the kidney. Renal function deteriorated in 5 patients (20.8%), including 2 (8.3%) who reached end-stage renal disease (ESRD).ConclusionIgG4-RKD and IgG4-RPF represent major organ manifestations of IgG4-RD, and should be identified early with prompt treatment to prevent progression to ESRD.

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Section: Methodsmentioning

confidence: 68%

Clinical Manifestations and Long-term Outcomes of IgG4-Related Kidney and Retroperitoneal Involvement in a United Kingdom IgG4-Related Disease Cohort

Evans

Cargill

Goodchild

et al. 2019

Kidney International Reports

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“…Since then, it has been recognized as an immune-mediated condition involving several organs, particularly exocrine organs such as the pancreas, salivary glands, and biliary tract [1]. Several studies have reported that intrathoracic involvement may be seen in about 10 to 50% [2,4,5], which commonly includes interstitial lung disease, inflammatory pseudotumors, fibrosing mediastinitis, and lymphadenopathy. The formation of a mass in the anterior mediastinum, mimicking an anterior mediastinal tumor, is very rare.…”

Section: Discussionmentioning

confidence: 99%

“…IgG4-related disease (IgG4-RD) is a systemic disease characterized by an elevated serum IgG4 level and lymphoplasmacytic infiltration of multiple organs, such as the pancreas, salivary glands, and biliary tract [1]. In the respiratory system, it commonly presents as pulmonary nodules, lymphadenopathy, or sclerosing mediastinitis [2]. We present a case of IgG4-related disease forming a mass in the anterior mediastinum.…”

Section: Introductionmentioning

confidence: 99%

A case of IgG4-related anterior mediastinal sclerosing disease coexisting with autoimmune pancreatitis

et al. 2020

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Background: IgG4-related disease (IgG4-RD) is a systemic fibro-inflammatory condition that predominantly involves exocrine organs. Concerning its thoracic presentation, it often manifests as interstitial lung disease or fibrosing mediastinitis. It is very rare for IgG4-RD to form a well-defined mass in the anterior mediastinum, mimicking an encapsulated thymoma. Case presentation: An 82-year-old man with autoimmune pancreatitis under treatment with oral corticosteroids was found to have peripancreatic lymphadenopathy on computed tomography. Subsequent positron emission tomography revealed abnormal uptake (maximal value 3.6) by a thymic mass as well as the peripancreatic lymph nodes. Exacerbation of IgG4-RD was suspected, and we increased the oral steroid dosage. As a result, the peripancreatic lymph nodes, and the anterior mediastinal mass, decreased in size. The thymic mass was suspected to be an encapsulated thymoma because of its lobulated shape, degree of fluorodeoxyglucose accumulation, and response to steroids, and the patient was referred to our department. The serum anti-acetylcholine receptor antibody test was negative. A thoracoscopic tumor resection was performed as diagnostic therapy. Histopathological analysis revealed dense lymphoplasmacytic infiltration with sclerotic stroma within the tumor. Immunohistochemical analysis revealed abundant IgG4-positive plasma cell infiltrates and over 50% IgG4/IgGpositive plasma cells. We did not see either keratin-positive thymocytes or terminal deoxynucleotidyl transferasepositive lymphocytes. Furthermore, deviation in the kappa chain and lambda chain-positive plasma cells was not noted. Accordingly, IgG4-related sclerosing disease was diagnosed. Conclusions: IgG4-related sclerosing masses in the anterior mediastinum are very rare, and the effect of tumor resection on prognosis remains unclear. IgG4-RD had potentially been categorized as Castleman's disease.

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“…30 These reports, however, do not include mediastinal and airway manifestations and thereby underestimate the frequency of thoracic involvement which may be nearly one-half of patients. 5,15 Most cases of thoracic involvement with IgG4-RD are found incidentally during the workup of extrathoracic lesions. As such, patients with thoracic IgG4-RD may not manifest overt respiratory symptoms, for example, mediastinal lymphadenopathy.…”

Section: Thoracic Manifestations Of Igg4-rdmentioning

confidence: 99%

“…Intrathoracic lymphadenopathy involving the mediastinum and hilum has been reported to be the most common form of thoracic involvement in IgG4-RD in some studies but not in others. 5,15 In these cases of IgG4-RD-related intrathoracic lymphadenopathy, the diagnosis is often based on imaging without histopathologic confirmation in the presence of documented IgG4-RD elsewhere in the body. Mediastinal involvement in IgG4-RD can also manifest mediastinal mass or fibrosing mediastinitis.…”

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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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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Thoracic involvement in IgG4-related disease in a UK-based patient cohort

Cited by 30 publications

References 19 publications

Clinical Manifestations and Long-term Outcomes of IgG4-Related Kidney and Retroperitoneal Involvement in a United Kingdom IgG4-Related Disease Cohort

Clinical Manifestations and Long-term Outcomes of IgG4-Related Kidney and Retroperitoneal Involvement in a United Kingdom IgG4-Related Disease Cohort

A case of IgG4-related anterior mediastinal sclerosing disease coexisting with autoimmune pancreatitis

Thoracic Involvement in IgG4-Related Disease

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