Clinical Manifestations of IgG4-Related Disease in the Pharynx

Reder, Lindsay; Della‐Torre, Emanuel; Stone, John H.; Mori, Matthew; Song, Phil Hyun

doi:10.1177/0003489414549574

Cited by 34 publications

(31 citation statements)

References 18 publications

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“…[30] These observations suggest that patients with IgG4-RD and positive ANCA might be more at risk of developing a systemic form of AAV. Indeed, head and neck manifestations of IgG4-RD are typically ANCA negative [31–33] raising the possibility that ANCA in these patients might sustain an underlying vasculitic process rather than being primarily linked to IgG4-RD. Additional cases and a longer follow-up are needed in order to confirm this hypothesis.…”

Section: Discussionmentioning

confidence: 99%

Antineutrophil cytoplasmic antibody positivity in IgG4-related disease

et al. 2016

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Background:IgG4-related disease (IgG4-RD) is a fibroinflammatory condition characterized by serum IgG4 elevation and tissue infiltration of IgG4-positive plasma cells. Substantial overlap between IgG4-RD and antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV) exists in terms of organ involvement and histopathological features. A positive ANCA assay is regarded as a highly specific finding in favor of an AAV, and generally influences away from a diagnosis of IgG4-RD. Recent reports, however, have raised the possibility that some patients with IgG4-RD are ANCA positive, thus suggesting reconsideration of the role of ANCA in the diagnostic workup. In the present work, we describe the first case of concomitant biopsy-proven IgG4-RD and granulomatosis with polyangiitis (GPA), demonstrating antiproteinase 3 (PR3) ANCA of the IgG4 subclass in the patient's serum. We also review the literature in order to provide clinicians with tools for interpreting ANCA positivity in IgG4-RD patients.Case summary:A 51-year-old woman was referred for left exopthalmos due to lacrimal gland enlargement and increased serum IgG4 concentration. IgG4-RD was suspected and further imaging studies disclosed multiple pulmonary masses in the right lung. Histological analysis of the left lacrimal gland was diagnostic for IgG4-RD, but lung biopsy showed typical features of GPA. ANCA assay was positive for anti-PR3 antibodies. Further immunofluorescence studies demonstrated anti-PR3 antibodies of IgG1 and IgG4 subclass. Treatment with rituximab induced swift remission of both IgG4-RD and GPA manifestations. We identified 9 other reports of patients with IgG4-RD and positive ANCA in the English literature, 5 cases with biopsy-proven IgG4-RD and 4 cases in whom IgG4-RD was diagnosed presumptively. Four patients had also histological evidence of concomitant AAV.Conclusion:The present work demonstrates that ANCA positivity in patients with biopsy-proven IgG4-RD should prompt the exclusion of a concomitant vasculitic process; a positive ANCA does not exclude the diagnosis of IgG4-RD; confirmation through immunoenzymatic assays of the ANCA specificity, clinical-pathological correlation, and histopathological evaluation remain crucial steps for the differential diagnosis between AAV and IgG4-RD.

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Section: Discussionmentioning

confidence: 99%

Antineutrophil cytoplasmic antibody positivity in IgG4-related disease

et al. 2016

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“…Thyroid gland [19] IgG4-related thyroiditis (Riedel's rhyroiditis) Neck pressure, neck mass, malaise, recurrent laryngeal nerve palsy, hypothyroidism Ear, nose and throat [17,36] IgG4-related sinusitis/midline destructive lesion/pharyngitis Nasal crusting, nasal obstruction, rhinorrhoea, polyposis, sinusitis, nasal septum perforation, laryngeal obstruction, middle ear effusion Thorax Lungs [11,12] IgG4-related lung disease Cough, sputum, dyspnoea, chest pain Pleura [12] IgG4-related pleural disease Chest pain, dyspnoea, pleural effusion Mediastinum [46] IgG4-related mediastinitis (fibrosing mediastinitis)…”

Section: Pathologymentioning

confidence: 99%

“…Indeed, IgG4-RD might be asymptomatic or present with signs and symptoms related to the mechanical compression exerted by the fibrotic masses on local structures. IgG4-RD has been described in virtually every anatomical region (Table 1) [11][12][13][14][15][16][17][18][19], but the most common manifestations include type I AIP, chronic periaortitis, retroperitoneal fibrosis (Ormond's disease) and salivary or lacrymal gland swelling (Mikulicz's disease), conditions regarded as single independent entities for decades.…”

Section: Overview Of Clinical Manifestationsmentioning

confidence: 99%

Immunology of IgG4-related disease

Della‐Torre

Lanzillotta

Doglioni

2015

Clinical and Experimental Immunology

188

215

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SummaryImmunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition that derives its name from the characteristic finding of abundant IgG4 1 plasma cells in affected tissues, as well as the presence of elevated serum IgG4 concentrations in many patients. In contrast to fibrotic disorders, such as systemic sclerosis or idiopathic pulmonary fibrosis in which the tissues fibrosis has remained largely intractable to treatment, many IgG4-RD patients appear to have a condition in which the collagen deposition is reversible. The mechanisms underlying this peculiar feature remain unknown, but the remarkable efficacy of B cell depletion in these patients supports an important pathogenic role of B cell/T cell collaboration. In particular, aberrant T helper type 2 (Th2)/ regulatory T cells sustained by putative autoreactive B cells have been proposed to drive collagen deposition through the production of profibrotic cytokines, but definitive demonstrations of this hypothesis are lacking. Indeed, a number of unsolved questions need to be addressed in order to fully understand the pathogenesis of IgG4-RD. These include the identification of an antigenic trigger(s), the implications (if any) of IgG4 antibodies for pathophysiology and the precise immunological mechanisms leading to fibrosis. Recent investigations have also raised the possibility that innate immunity might precede adaptive immunity, thus further complicating the pathological scenario. Here, we aim to review the most recent insights on the immunology of IgG4-RD, focusing on the relative contribution of innate and adaptive immune responses to the full pathological phenotype of this fibrotic condition. Clinical, histological and therapeutic features are also addressed.

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“…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%

Advances in the diagnosis and management of IgG4 related disease

Lanzillotta

Mancuso

Della‐Torre

2020

BMJ

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216

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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Clinical Manifestations of IgG4-Related Disease in the Pharynx

Cited by 34 publications

References 18 publications

Antineutrophil cytoplasmic antibody positivity in IgG4-related disease

Antineutrophil cytoplasmic antibody positivity in IgG4-related disease

Immunology of IgG4-related disease

Advances in the diagnosis and management of IgG4 related disease

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