Immunoglobulin G4–Related Disease Involving the Sclera

Ortiz, Alberto; Gallo, Jesica; Paira, Sergio; Paira, Noelia

doi:10.1097/rhu.0000000000000479

Cited by 4 publications

(2 citation statements)

References 13 publications

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“…There are a few reports of scleritis caused by IgG4-RD. Moreover, two IgG4-RD cases involving the sclera were misdiagnosed as intraocular tumor [12,13], and Ohno et al [12] reported as choroidal tumor and enucleation of the eyeball was performed. Contrary to the previous studies, our patient could have had the opportunity to avoid the discouraging surgery by simply performing serological tests.…”

Section: Discussionmentioning

confidence: 99%

IgG4-related disease presenting as recurrent scleritis combined with optic neuropathy

et al. 2021

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Background We report a case of atypical presentation of IgG4-related disease (IgG4-RD) with recurrent scleritis and optic nerve involvement. Case presentation A 61-year-old male presented with ocular pain and injection in his left eye for 2 months. Ocular examination together with ancillary testing led to the diagnosis of scleritis, which relapsed in spite of several courses of steroid treatment. After cessation of steroid, the patient complained of severe retro-orbital pain and blurred vision. His best corrected vision was count finger, the pupil was mid-dilated and a relative afferent pupillary defect was found. Funduscopic examination demonstrated disc swelling. Magnetic resonance imaging (MRI) showed enhancing soft tissue encasing the left globe, medial rectus muscle and optic nerve. Systemic work-up revealed multiple nodules in right lower lung and a biopsy showed histopathological characteristics of IgG4-RD. Long-term treatment with corticosteroids and a steroid-sparing agent (methotrexate) led to significant improvement in signs and symptoms with no recurrence for 2 years. Conclusions This case highlights the significance of IgG4-RD in the differential diagnosis of recurrent scleritis. IgG4-RD may cause optic neuropathy resulting in visual loss. Early diagnosis and proper treatment can prevent irreversible organ damage and devastating visual morbidity.

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Section: Discussionmentioning

confidence: 99%

IgG4-related disease presenting as recurrent scleritis combined with optic neuropathy

et al. 2021

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“…This phenomenon can occur in the setting of antigen excess (in this case, very high serum IgG4 concentrations), where assays do not provide accurate IgG4 measurements if the sample has not been diluted appropriately [ 9 , 11 ]. The prozone effect has been described in another case of IgG4-RD, with an initially normal serum IgG4 level that was found to be elevated after dilution of the sample [ 12 ]. The UK National External Quality Assessment Schemes (UK NEQAS) and other publications have shown that the antigen excess effect can occur at levels of IgG4 around the 95th centile of a normal population, causing missed IgG4 elevations due to prozoning [ 13 ].…”

Section: Discussionmentioning

confidence: 99%

A Challenging Diagnosis of IgG4-Related Disease When Understanding Limitations of Laboratory Testing Was Pivotal

Bell

Zahirieh

et al. 2017

Case Reports in Medicine

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A 76-year-old man was incidentally found on a CT scan to have lymphadenopathy and bilateral kidney enlargement suggestive of infiltrative renal disease. He was largely asymptomatic but had bilateral salivary and lacrimal gland enlargement. A grossly elevated serum IgG (>70 g/L) with concomitant suppression of other immunoglobulins, a small IgG restriction, and a parotid biopsy revealing lymphoplasmacytic infiltrate with slight kappa light chain excess all suggested a lymphoproliferative disorder (LPD). The diagnostic workup was further confounded by a normal serum IgG4 concentration. Moreover, bone marrow and renal biopsies did not reveal evidence of LPD. Discussion with the laboratory not only clarified that the markedly increased total IgG could not be accounted for by the small IgG restriction, but also identified a discrepancy in the IgG4 measurement. Repeat analysis of a follow-up sample revealed an elevated IgG4 of 5.94 (reference interval: 0.039–0.864) g/L, which prompted a repeat parotid biopsy that showed predominant IgG4+ lymphocytic infiltrates. Despite the deluding presentations, a final diagnosis of IgG4-related disease (IgG4-RD) was made based on elevated serum IgG4 concentrations and histopathological findings. This case highlights the importance of recognizing limitations of laboratory testing and the benefit of close communications among clinical subspecialties and the laboratory.

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IgG4-related orbital disease presenting as optic neuropathy: A rare presentation and review of literature

Harirajan,

Neena,

Pauly

et al. 2024

Indian Journal of Ophthalmology - Case Reports

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A 74-year-old male presented with a sudden drop of vision in the left eye for 5 days. He had a relative afferent pupillary defect and visual acuity of counting finger at 1 m in the left eye. Fundus showed a hyperaemic disc in the left eye. Ocular movements were full. He had uncontrolled diabetes mellitus. A possibility of anterior ischemic optic neuropathy was considered. The patient was lost for follow-up and came back 2 months later with visual acuity of perception of light in the left eye with features suggestive of orbital apex syndrome. Histopathology was confirmatory of immunoglobulin G4 (IgG4) disease. IgG4 disease presenting as optic neuropathy can be an early manifestation of orbital involvement.

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Immunoglobulin G4–Related Disease Involving the Sclera

Cited by 4 publications

References 13 publications

IgG4-related disease presenting as recurrent scleritis combined with optic neuropathy

IgG4-related disease presenting as recurrent scleritis combined with optic neuropathy

A Challenging Diagnosis of IgG4-Related Disease When Understanding Limitations of Laboratory Testing Was Pivotal

IgG4-related orbital disease presenting as optic neuropathy: A rare presentation and review of literature

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