Ocular adnexal IgG4-related disease: clinical features, outcome, and factors associated with response to systemic steroids

Yu, Wen-Chung; Kao, Sung‐Shuo; Yang, Ching‐Fen; Lee, Fenq-Lih; Tsai, Chieh Chih

doi:10.1007/s10384-014-0353-1

Cited by 38 publications

(31 citation statements)

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“…Sparse eosinophils were observed in only 3 soft tissue biopsies (that did not comprise LG) and obliterative phlebitis was never reported in the present series, as previously published in IgG4-ROD. [6,28] Yet, in all cases, a strict clinicopathologic correlation was performed to rigorously rule out diseases that mimic IgG4-RD, especially those presenting with tumefactive lesions and increased IgG4+ plasma cells on tissue biopsy. Two patients (patients 11 and 12) presented with no extra-orbital manifestations of IgG4-RD and normal serum IgG4 levels, but were included in order not to underestimate the true prevalence of IgG4-ROD or even overlook an entire pattern of patients with strictly localized orbital disease.…”

Section: Discussionmentioning

confidence: 99%

Ophthalmic manifestations in IgG4-related disease

et al. 2017

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Section: Discussionmentioning

confidence: 99%

Ophthalmic manifestations in IgG4-related disease

et al. 2017

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“…Currently, the cause and pathogenesis of BLEL remain unclear. Studies have shown that BLEL belongs to the category of IgG4-related diseases[ 4 – 5 ]. Clinically, BLEL can be treated with glucocorticoid therapy, but the effects are not ideal.…”

Section: Introductionmentioning

confidence: 99%

Complement System in the Pathogenesis of Benign Lymphoepithelial Lesions of the Lacrimal Gland

Wang

et al. 2016

PLoS ONE

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ObjectiveWe aimed to examine the potential involvement of local complement system gene expression in the pathogenesis of benign lymphoepithelial lesions (BLEL) of the lacrimal gland.MethodsWe collected data from 9 consecutive pathologically confirmed patients with BLEL of the lacrimal gland and 9 cases with orbital cavernous hemangioma as a control group, and adopted whole genome microarray to screen complement system-related differential genes, followed by RT-PCR verification and in-depth enrichment analysis (Gene Ontology analysis) of the gene sets.ResultsThe expression of 14 complement system-related genes in the pathologic tissue, including C2, C3, ITGB2, CR2, C1QB, CR1, ITGAX, CFP, C1QA, C4B|C4A, FANCA, C1QC, C3AR1 and CFHR4, were significantly upregulated while 7 other complement system-related genes, C5, CFI, CFHR1|CFH, CFH, CD55, CR1L and CFD were significantly downregulated in the lacrimal glands of BLEL patients. The microarray results were consistent with RT-PCR analysis results. Immunohistochemistry analysis of C3c and C1q complement component proteins in the resected tissue were positive in BLEL patients, while the control group had negative expression of these proteins. Gene ontology (GO) analysis revealed that activation of the genes of complement system-mediated signaling pathways were the most enriched differential gene group in BLEL patients.ConclusionsLocal expression of complement components is prominently abnormal in BLEL, and may well play a role in its pathogenesis.

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“…The incidence of enlarged EOMs in thyroid ophthalmopathy is from 80 to 90% [4], and that for IgG4-related ophthalmic disease is 10 to 89% [5, 6]. The incidence of enlarged EOMs is considerably higher for these diseases.…”

Section: Discussionmentioning

confidence: 99%

Orbital T-Cell Lymphoma with Discrete Enlargements of All Extraocular Muscles Bilaterally in Patient with Moon Face Countenance

Kawakami

Mochizuki

Goto

et al. 2017

Case Reports in Ophthalmological Medicine

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Purpose. To report our findings in a case of orbital T-cell lymphoma in which all of the extraocular muscles (EOMs) were bilaterally and discretely enlarged and the patient had a moon face countenance. Case. A 59-year-old woman presented with visual disturbances in her left eye, hyperemia in both eyes, and a moon face countenance. Examinations showed limited upward gaze in the right eye, blepharoptosis, hypertropia, and limited downward and rightward gaze in the left eye. Slit-lamp examination showed only chemosis and hyperemia of both eyes. Magnetic resonance imaging with contrast revealed discrete enlargements of the muscle bellies in all EOMs without abnormalities of the orbital fat in both eyes. Blood examinations excluded thyroid- and IgG4-related ophthalmopathy, and EOM biopsy revealed peripheral T-cell lymphoma. After beginning aggressive chemotherapy, the enlarged EOMs, limited eye motility, and moon face countenance improved. Unfortunately, the patient died of sepsis during the chemotherapy. Conclusions. A lymphoma should be included in the differential diagnosis of eyes with enlarged EOMs. Because lymphomas can lead to death, it is important for clinicians to consider lymphomas in eyes with enlarged EOMs.

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Ocular adnexal IgG4-related disease: clinical features, outcome, and factors associated with response to systemic steroids

Abstract: Ocular adnexal IgG4-related disease predominantly involved the lacrimal glands bilaterally and was usually associated with high serum IgG4 levels and multiple organ involvement. Most patients responded well to steroid therapy, which was accompanied by a marked decrease in serum IgG4.

Cited by 38 publications

References 32 publications

Ophthalmic manifestations in IgG4-related disease

Ophthalmic manifestations in IgG4-related disease

Complement System in the Pathogenesis of Benign Lymphoepithelial Lesions of the Lacrimal Gland

Orbital T-Cell Lymphoma with Discrete Enlargements of All Extraocular Muscles Bilaterally in Patient with Moon Face Countenance

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