Deconstructing IgG4-related disease involvement of midline structures: Comparison to common mimickers

Lanzillotta, Marco; Campochiaro, Corrado; Trimarchi, Matteo; Arrigoni, Gianluigi; Gerevini, Simonetta; Milani, Raffaella; Bozzolo, Enrica; Biafora, Matteo; Venturini, Elena; Cicalese, Maria Pia; Stone, John H.; Sabbadini, Maria Grazia; Della‐Torre, Emanuel

doi:10.1080/14397595.2016.1227026

Cited by 34 publications

(38 citation statements)

References 29 publications

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“…This classification algorithm showed excellent accuracy in distinguishing IgG4-RD from multiple mimickers, including malignant disorders, granulomatous conditions, and small and large vessel vasculitidies,1516 with a specificity of 97.8% and a sensitivity of 82.0%. Moreover, high specificity and sensitivity were maintained even when exclusion and inclusion criteria related to biopsy or serum IgG4 domains were removed from the algorithm, indicating that combinations of highly suggestive clinical and/or radiological manifestations alone can accurately classify patients after entry and exclusion criteria have been passed 2223.…”

Section: Guidelinesmentioning

confidence: 98%

“…Notably, the first three histopathological features are critical for diagnosis, whereas immunohistochemistry is secondary in importance because mimicker neoplastic and inflammatory disorders can also show increased tissue IgG4 counts. Neutrophils, leukocytoclastic vasculitis, granulomas, and tissue necrosis do not belong to the spectrum of pathological features of IgG4-RD and, even if accompanied by a prominent IgG4 positive plasma cell infiltrate, should prompt exclusion of other inflammatory conditions such as ANCA associated vasculitis and sarcoidosis 1669707172737475…”

Section: Guidelinesmentioning

confidence: 99%

“…Since then, IgG4-RD has been recognized with increasing frequency by both generalists and specialists worldwide. However, awareness of the diagnostic and therapeutic tools for the management of patients with IgG4-RD remains confined to tertiary care centers, and the disease is still misdiagnosed as neoplastic, inflammatory, and infectious conditions 1314151617181920…”

Section: Introductionmentioning

confidence: 99%

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Advances in the diagnosis and management of IgG4 related disease

Lanzillotta

Mancuso

Della‐Torre

2020

BMJ

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213

172

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IgG4 related disease was recognized as a unified disease entity only 15 years ago. Awareness of IgG4 related disease has increased worldwide since then, and specialists are now familiar with most of its clinical manifestations. Involvement of the pancreato-biliary tract, retroperitoneum/aorta, head and neck, and salivary glands are the most frequently observed disease phenotypes, differing in epidemiological features, serological findings, and prognostic outcomes. In view of this multifaceted presentation, IgG4 related disease represents a great mimicker of many neoplastic, inflammatory, and infectious conditions. Histopathology remains key to diagnosis because reliable biomarkers are lacking. Recently released classification criteria will be invaluable in improving early recognition of the disease. IgG4 related disease is highly treatable and responds promptly to glucocorticoids, but it can lead to end stage organ failure and even death if unrecognized. Prolonged courses of corticosteroids are often needed to maintain remission because the disease relapses in most patients. Rapid advancement in our understanding of the pathophysiology of IgG4 related disease is leading to the identification of novel therapeutic targets and possible personalized approaches to treatment.

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Section: Guidelinesmentioning

confidence: 98%

Section: Guidelinesmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Advances in the diagnosis and management of IgG4 related disease

Lanzillotta

Mancuso

Della‐Torre

2020

BMJ

Self Cite

213

172

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“…The obstruction can be distinguished as congenital or acquired. Primary acquired nasolacrimal duct obstruction is determined in most cases by idiopathic fibrosis or inflammation, while infection, traumatic events, surgery, or neoplasms may cause secondary acquired NLDO 17‐22 …”

Section: Discussionmentioning

confidence: 99%

A case of endonasal balloon‐assisted dacryoplasty after failure of endonasal dacryocystorhinostomy

Indelicato

Vinciguerra

Resti

et al. 2020

Clinical Case Reports

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“…in the sinonasal cavity and mucosal infiltration mimicking chronic rhinosinusitis. 109,110 IgG4-RD accounts for some of the cases of midline destructive lesions (also known as lethal midline granuloma). 111…”

Section: Airway 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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Deconstructing IgG4-related disease involvement of midline structures: Comparison to common mimickers

Abstract: Histological examination of bioptic samples from the sinonasal area and oral cavity represents the mainstay for the diagnosis of IgG4-RD involvement of the midline structures.

Cited by 34 publications

References 29 publications

Advances in the diagnosis and management of IgG4 related disease

Advances in the diagnosis and management of IgG4 related disease

A case of endonasal balloon‐assisted dacryoplasty after failure of endonasal dacryocystorhinostomy

Thoracic Involvement in IgG4-Related Disease

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