Ophthalmic immunoglobulin G4-related disease IgG4-RD Current concepts

Mulay, Kaustubh; Wick, Mark R.

doi:10.1053/j.semdp.2015.10.001

Cited by 12 publications

(8 citation statements)

References 62 publications

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“…In addition to autoimmune pancreatitis, they include Mickulicz disease, angiocentric fibrosis with eosinophilia, sclerosing sialadenitis (Kuttner tumor), Riedel thyroiditis, sclerosing cholangitis, and idiopathic retroperitoneal fibrosis, among others. In the orbit, IgG4-RD seems to account for a significant proportion of cases formerly diagnosed as idiopathic orbital inflammation (IOI) or reactive lymphoid hyperplasia (RLH) 5 . Although IgG4-RD remains idiopathic as its etiology is unclear, the clinicopathologic criteria for its diagnosis seem sufficiently specific to distinguish it from other forms of IOI, RLH, and many other forms of orbital inflammation, such as Sjögren's syndrome, granulomatosis with polyangiitis, and sarcoidosis.…”

Section: Discussionmentioning

confidence: 99%

“…Diagnosis depends on finding the appropriate histologic features and correlating them with the clinical history, imaging, and serum IgG4 levels. However, it is important to note that serum IgG4 levels may be normal in up to 40% of patients with IgG4-RD, which precludes its use as a sole diagnostic marker or as a measure of response to therapy in these patients 5, 8, 9…”

Section: Discussionmentioning

confidence: 99%

“…Although the true incidence is unknown, ophthalmic involvement by IgG4-RD is not rare, and a significant number of patients will have disease elsewhere at presentation, or develop it subsequently 5 . More than 50% have unilateral or bilateral lacrimal gland lesions, followed in decreasing frequency by trigeminal nerve, extraocular muscles, orbital fat, circumscribed orbital masses, eyelid lesions, nasolacrimal duct, lacrimal sac, and optic nerve 6, 7.…”

Section: Introductionmentioning

confidence: 99%

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Orbital progressive transformation of germinal centers as part of the spectrum of IgG4-related ophthalmic disease: Clinicopathologic features of three cases

Vizcaíno

Joseph

Eberhart

2018

Saudi Journal of Ophthalmology

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Progressive transformation of germinal centers (PTGC) is a form of follicular hyperplasia recently associated with immunoglobulin G4-related disease (IgG4-RD), but the ophthalmic manifestations of this combination are poorly described. In this retrospective case series, we present three cases of IgG4-related orbital disease (IgG4-ROD) showing varying degrees of PTGC involving the orbit and lacrimal gland. Three adult women presented with ill-defined lacrimal gland enlargement. Histologic sections showed variable fibrosis and large, irregular lymphoid follicles with prominent mantle zones penetrating the germinal centers, highlighted by Bcl-2 and/or IgD immunostains. The interfollicular areas contained a mixture of plasma cells, scattered histiocytes and eosinophils. Mixed T and B-cells were present, and no signs of monoclonality were identified. All cases showed more than 100 IgG4 positive cells per high power field. Epstein-Barr virus in situ hybridization performed in one case was negative. The serum IgG4 level was tested in one case and showed elevation above the normal range. After 2-10 months of follow-up, the patients showed either near-complete resolution or no remaining signs of ophthalmic disease. Increasing awareness of these PTGC in extra-nodal locations, including the orbit, may provide a better understanding of the histologic spectrum of this disease.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Orbital progressive transformation of germinal centers as part of the spectrum of IgG4-related ophthalmic disease: Clinicopathologic features of three cases

Vizcaíno

Joseph

Eberhart

2018

Saudi Journal of Ophthalmology

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“…Symptoms of IgG4-ROD may overlap those seen in other diseases, though in most patients, both eyes are affected. Likewise, CT or MRI scans of the orbital region also present unspecific features [109]. The presence of the high amount of IgG4+ cells in the biopsy cannot be the only feature in setting diagnosis, as they are also detected in other diseases like granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome), xanthogranulomatous disease, or lymphoma [110][111][112].…”

Section: Other Inflammatory Eye Diseases To Be Included In the Differential Diagnosismentioning

confidence: 99%

IgG4 as a Biomarker in Graves’ Orbitopathy

Olejarz

Szczepanek‐Parulska

Dadej

et al. 2021

Mediators of Inflammation

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Immunoglobulin G4-related disease (IgG4-RD) is a chronic inflammatory disorder associated with fibrosis and abundant tissue lymphoplasmacytic infiltrations. It typically affects the pancreas, the salivary glands, and the retroperitoneal space. However, it might also involve multiple other organs, including the orbit and the thyroid. Recent studies have suggested that IgG4 plays a role in the pathophysiology of autoimmune thyroid diseases. This ultimately led to the establishment of new clinical entities called IgG4-related thyroid disease and thyroid disease with an elevation of IgG4. The aim of this paper is to describe the pathophysiological, histopathological, and clinical features of Graves’ Disease (GD) and Graves’ Orbitopathy (GO) with elevated IgG4 levels. Multiple studies have demonstrated higher IgG4 serum concentrations in GD patients than in healthy euthyroid controls. Depending on the studied population, elevated serum IgG4 levels occur in 6.4-23% (average: 10.3%) of all patients with GD, 8.3-37.5% (average: 17.6%) of patients with GO, and 0-9.8% (average: 5.4%) of patients with GD without GO, while GO patients comprise 37.5-100% (average: 65.8%) of all GD patients with elevated IgG4 levels. Characteristic features of GD with elevated IgG4 levels include lower echogenicity of the thyroid gland on ultrasound examination, peripheral blood eosinophilia, higher prevalence of orbitopathy, and better response to antithyroid drugs with a tendency to develop hypothyroidism when compared to patients with GD and normal levels of IgG4. Typical signs of GO accompanied by increased concentration of IgG4 include younger age at diagnosis, and more severe course of the disease with a higher Clinical Activity Score (CAS).. We strongly recommend considering the diagnosis of GO with elevated IgG4 in patients with an established diagnosis of GD, elevated serum IgG4 levels, and clinical features of ophthalmic disease overlapping with those of IgG4-related orbital disease.

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“…It is important to note that IgG4-RD is an idiopathic, multiorgan inflammatory state, possibly manifestin gas chronic, relapsing inflammation in virtually any organ [23]. For current concepts on ophthalmic IgG4-RD, see review by Mulay and Wick [24]. For background and pathology of IgG4-related ophthalmic disease, see review by McNab and McKelvie [25].…”

Section: Introductionmentioning

confidence: 99%

Orbital IgG4-associated diseases

Závorková¹,

Richter²,

Větvička³

et al. 2017

Pathol Discov

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IgG4-related disease (IgG4-RD) is a distinct entity that frequently occurs in an ophthalmic location. IgG4-RD is not limited to the orbit, but may also involve other anatomical structures in and around the eye. Careful clinicoradiologic examination and the use of immunohistological examination are key diagnostic methods. Serum IgG4 levels are neither sensitive nor specific enough for the diagnosis of IgG4-RD and should not be relied upon solely. Careful evaluation of histologic and immunophenotypic features and clinical correlation are required to distinguish orbital IgG4-RD from other inflammatory lesions in the orbit. Glucocorticoids are the primary therapeutic choice for treatment of IgG4-RD. Azathioprine or Mofetil can be used as a second possibility. Rituximab can be effective in the patients with relapse IgG4-RD.

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Ophthalmic immunoglobulin G4-related disease IgG4-RD Current concepts

Cited by 12 publications

References 62 publications

Orbital progressive transformation of germinal centers as part of the spectrum of IgG4-related ophthalmic disease: Clinicopathologic features of three cases

Orbital progressive transformation of germinal centers as part of the spectrum of IgG4-related ophthalmic disease: Clinicopathologic features of three cases

IgG4 as a Biomarker in Graves’ Orbitopathy

Orbital IgG4-associated diseases

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