IgG4-related orbital disease masquerading as thyroid eye disease, vice versa, or both?

Khandji, Joyce; Campbell, Ashley A.; Callahan, Alison B.; Sirinek, Portia; Kazim, Michael

doi:10.1080/01676830.2017.1383478

Cited by 6 publications

(10 citation statements)

References 7 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…This needs to be interpreted with caution as the study analyzed mostly specimens obtained from orbital fat, while only four biopsies were obtained from the lacrimal gland and none from extraorbital muscles [85]. This is confirmed by the case report of a patient with GO, in which no staining for IgG4 in the lacri-mal gland and orbital fat was demonstrated but 24 IgG4(+) cells in HPF and fibrosis in an enlarged extraorbital muscle biopsy [86].…”

Section: Role Of Igg4 In Graves' Disease and Orbitopathymentioning

confidence: 74%

“…There are currently a handful of case reports describing patients with GO, and the biopsy procedure confirmed IgG4(+) plasma cell infiltration of the orbital tissue [86][87][88]. In those cases, biopsies were obtained most often not from the orbital fat but from the extraorbital muscle, lacrimal gland, or infiltrative orbital masses.…”

Section: Role Of Igg4 In Graves' Disease and Orbitopathymentioning

confidence: 99%

See 1 more Smart Citation

IgG4 as a Biomarker in Graves’ Orbitopathy

Olejarz

Szczepanek‐Parulska

Dadej

et al. 2021

Mediators of Inflammation

View full text Add to dashboard Cite

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.

show abstract

Section: Role Of Igg4 In Graves' Disease and Orbitopathymentioning

confidence: 74%

Section: Role Of Igg4 In Graves' Disease and Orbitopathymentioning

confidence: 99%

IgG4 as a Biomarker in Graves’ Orbitopathy

Olejarz

Szczepanek‐Parulska

Dadej

et al. 2021

Mediators of Inflammation

View full text Add to dashboard Cite

show abstract

“…29 Both IgG4-ROD and TED can have bilateral and multiple EOM enlargement radiologically. 30,31 Komatsu et al 32 reported that up to 27% of autoimmune pancreatitis patients were associated with hypothyroidism, indicative of thyroid involvement in IgG4-RD. Tooley et al 33 suggested that the key features to distinguish IgG4-ROD from TED were disproportionately large lateral rectus muscle and the enlarged infraorbital nerves.…”

Section: Discussionmentioning

confidence: 99%

Radiological Determinants of Complicated Immunoglobulin G4–Related Ophthalmic Disease: A Territory-Wide Cohort Study

Lai

Chu

et al. 2022

Asia-Pacific Journal of Ophthalmology

View full text Add to dashboard Cite

Purpose: To evaluate the presenting radiological features of immunoglobulin G4–related ophthalmic disease (IgG4-ROD) and their associations with IgG4-related optic neuropathy (IgG4-RON), and IgG4-related ocular adnexal lymphoma (IgG4-ROL). Methods: A territory-wide, biopsy-proven, Chinese cohort. Masked review of orbital images, medical records, and histopathology reports. Results: A total of 115 (94%) of the 122 patients in our cohort had preoperative orbital images (computed tomography=105, magnetic resonance imaging=40). Among them, 103/115 (90%) showed enlarged lacrimal glands, and 91 (88%) were bilateral. Nerve enlargement was observed: infraorbital in 31/115 (27%) patients and frontal in 17/115 (15%), 10 and 9 being bilateral, respectively. At least 1 or more extraocular muscle (EOM) enlargement was found in 41/115 (37%) patients, bilaterally in 20. Lateral rectus occurred in 30 (73%) of these 41 EOM patients and inferior rectus in 28 (68%). Two adjacent EOMs (inferior and lateral recti in 11 patients, inferior and medial recti in 7 patients) or multiple EOMs (at least 3) were enlarged in 23/41 (56%) and 13/41 (32%) of the patients, respectively. Intraconal lesions (67% vs 11%, P<0.05), infraorbital (83% vs 23%, P<0.005), or frontal (50% vs 15%, P<0.05) nerve enlargement was significantly associated with IgG4-RON (6 patients) by univariate analyses. Asymmetric lacrimal gland enlargement and discrete orbital mass (both P<0.05) were associated with IgG4-ROL (9 patients) by multivariate analyses. Conclusions: In this IgG4-ROD cohort, most patients had bilateral enlarged lacrimal glands, and the lateral rectus is the most frequently involved EOM. For the first time, unique radiological patterns associated with the development of IgG4-RON and IgG4-ROL are found.

show abstract

“…Case reports describing eye diseases masquerading as Graves’ ophthalmopathy can be found in the literature; 5 , 6 however, only a few cases demonstrated idiopathic orbital inflammation and thyroid eye disease occurring concurrently. While clinicians frequently order imaging in these cases, imaging is often inconclusive.…”

Section: Discussionmentioning

confidence: 99%