IgG4-Related Disease Presenting as Isolated Scleritis

Berkowitz, Eran; Arnon, Ella; Yaakobi, Alona; Tiosano, Beatrice

doi:10.1155/2017/4876587

Cited by 5 publications

(3 citation statements)

References 10 publications

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“…In most cases, scleritis was found to be an IgG4-related disease after performing blood sampling for a detailed examination. Characteristically, it is often unilateral and does not involve other organs [7][8][9][10][11][12][13][14].…”

Section: Discussionmentioning

confidence: 99%

A Case of Immunoglobulin G4-Related Scleritis and Pneumonia Initially Diagnosed as Eosinophilic Pneumonia

Fukushima¹,

Tabuchi²

2022

Cureus

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Immunoglobulin G4 (IgG4)-positive plasma cells play a pivotal role in the pathogenesis of IgG4-related diseases, in which fibrosis is observed in various organs. Lesions often develop in the lacrimal gland and orbit; however, uveitis and scleritis are also noticed. We present the case of a 55-year-old woman who was diagnosed with eosinophilic pneumonia in November 2021 at the Department of Collagen Disease of another hospital. She was treated with 25 mg of oral prednisolone. On January 11, 2022, when the dose of prednisolone was reduced to 7.5 mg, she began complaining of conjunctival hyperemia in the left eye, and a few days later, eye pain was also reported. On January 17, she visited an ophthalmology clinic and was prescribed betamethasone and tacrolimus eye drops, and was subsequently admitted to our hospital. The blood test results showed a high IgG4 level. We consulted the collagen disease physician to evaluate her previous data. The serum IgG4 level collected on December 6, 2021, was 608 mg/dL, and a re-examination of the bronchial biopsy tissues on December 2, 2021, confirmed 48% of IgG4-positive cells. Thus, pulmonary lesions appeared to be IgG4-related pathologies. Increasing the oral prednisolone dose to 30 mg improved the scleritis. As seen in this case, the possibility of an IgG4-related disease should be considered when scleritis is observed in a patient diagnosed with eosinophilic pneumonia.

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Section: Discussionmentioning

confidence: 99%

A Case of Immunoglobulin G4-Related Scleritis and Pneumonia Initially Diagnosed as Eosinophilic Pneumonia

Fukushima¹,

Tabuchi²

2022

Cureus

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“…The lacrimal gland is most commonly involved but other orbital structures affected include the EOM, eyelid, the supraorbital and infraorbital nerves [7][8][9]. IgG4-ROD is a new and rare entity, which can be difficult to diagnose, especially in case of atypical features such as scleritis and conjunctival infiltration [10,11]. There are a few reports of scleritis caused by IgG4-RD.…”

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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“…The chronic inflammation and fibrosis often cause diffuse enlargement of affected tissues mimicking a neoplastic etiology. IgG4-ROD orbital manifestations include lacrimal gland enlargement, extraocular muscle enlargement and it most commonly presents as inflammation of orbital and scleral tissues, but is also a rare cause of uveitis [195][196][197][198][199][200][201][202].…”

Section: Inflammatory Systemic Disease: Scleritis and Intraocular Inv...mentioning

confidence: 99%

Mimickers of anterior uveitis, scleritis and misdiagnoses- tips and tricks for the cornea specialist

Caplash,

Paez-Escamilla,

Westcott

et al. 2024

J Ophthal Inflamm Infect

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Background Anterior uveitis, inflammation of the anterior chamber and related structures, is a cohort of diseases that can present to almost any general or sub-specialty Ophthalmology practice. Its features classically involve anterior chamber cell and flare. Below the surface of these two signs exist a panoply of diagnoses. Body The purpose of this review is to provide a general framework for diagnoses of anterior uveitis that are often missed as well as non-uveitic pathologies that often mimic anterior uveitis. Diagnostic deviation in either direction can have vision-threatening and rarely life-threatening consequences for patients. Using a comprehensive literature review we have collected a broad spectrum of etiologies of anterior uveitis that are easily missed and non-uveitic pathologies that can masquerade as anterior uveitis. Conclusions We present a focused review on specific misdiagnosed anterior uveitis pathologies and some of the conditions that can masquerade as anterior uveitis and scleritis.

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IgG4-Related Disease Presenting as Isolated Scleritis

Cited by 5 publications

References 10 publications

A Case of Immunoglobulin G4-Related Scleritis and Pneumonia Initially Diagnosed as Eosinophilic Pneumonia

A Case of Immunoglobulin G4-Related Scleritis and Pneumonia Initially Diagnosed as Eosinophilic Pneumonia

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

Mimickers of anterior uveitis, scleritis and misdiagnoses- tips and tricks for the cornea specialist

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