IgG4-related disease presenting as hypertrophic pachymeningitis and compressive optic neuropathy

Hwang, Gyuhwan; Jin, So-Young; Kim, Hyun‐Sook

doi:10.1016/j.jbspin.2015.07.016

Cited by 15 publications

(6 citation statements)

References 10 publications

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“…Since lymphoplasmocyic infiltration with CD20+ cells is one of the hallmarks of the disease, rituximab (an anti-CD20 monoclonal antibody) may be a treatment of choice, with a response rate of 90% in refractory cases [18,19]. Although treatment was started within 1 week of developing visual disturbance in this case, visual acuity was not recovered completely similar to other reports [17,20] because of the involvement of optic nerve. The optic neuropathy leading to vision impairment may be due to a compressive or inflammatory process or both.…”

Section: Discussionsupporting

confidence: 78%

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: Discussionsupporting

confidence: 78%

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

et al. 2021

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“…MTX was specifically assessed in a recent retrospective small study by Della-Torre et al and was reported to be effective in maintaining clinical and serological responses induced by glucocorticoids, enabling a substantial reduction in the overall dose of steroids in all patients and their withdrawal in six of ten patients within 6–12 months [161]. None of these patients had neurological disease secondary to IgG4-RD, and it should be noted that others have reported conflicting results of SSA use (predominantly MTX) in neurological disease caused by IgG4-RD [15, 63, 64, 69–71, 83, 118, 162, 163]. …”

Section: Treatmentmentioning

confidence: 99%

Neurological Manifestations of IgG4-Related Disease

Baptista

Casian

Gunawardena

et al. 2017

Curr Treat Options Neurol

106

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Opinion statementIgG4-related disease (IgG4-RD) is a multisystem inflammatory disorder. Early recognition of IgG4-RD is important to avoid permanent organ dysfunction and disability. Neurological involvement by IgG4-RD is relatively uncommon, but well recognised—hypertrophic pachymeningitis and hypophysitis are the most frequent manifestations. Although the nervous system may be involved in isolation, this more frequently occurs in conjunction with involvement of other systems. Elevated circulating levels of IgG4 are suggestive of the condition, but these are not pathognomonic and exclusion of other inflammatory disorders including vasculitis is required. Wherever possible, a tissue diagnosis should be established. The characteristic histopathological changes include a lymphoplasmacytoid infiltrate, storiform fibrosis and obliterative phlebitis. IgG4-RD typically responds well to treatment with glucocorticoids, although relapse is relatively common and treatment with a steroid-sparing agent or rituximab may be required. Improved understanding of the pathogenesis of IgG4-RD is likely to lead to the development of more specific disease treatments in the future.

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“…[ 35 ] In some cases a meningeal infiltration of the optic nerve is found on imaging, suggesting perineuritis. [ 30 , 31 , 33 , 39 ] Only one case of unilateral IgG4-related optic neuropathy occurred in the absence of any infiltrating mass. [ 32 ] In view of this only case, it seems that IgG4-related optic neuropathy may rarely be due to direct infiltration of the optic nerve by IgG4-related inflammation (by analogy with what is found in the biopsies of the branches of the trigeminal nerve, but this hypothesis is subject to confirmation by an actual optic nerve biopsy).…”

Section: Discussionmentioning

confidence: 99%

Colon cancer and IgG4-related disease with orbital inflammation and bilateral optic perineuritis

Lemaître

Esquerda²,

Guardiola

et al. 2018

Medicine

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Rationale:Immunoglobulin G4-related disease (IgG4-RD) is an inflammatory condition of unknown cause. Cancer might be related to the development of certain IgG4-RD but to date, little literature documents it.Patient concerns:A 78-year old man presented with unilateral proptosis responsive to steroids, initially attributed to nonspecific orbital inflammation.Diagnosis:Right hemicolectomy was performed because of a suspicious lesion which turned out to be tubulovillous adenoma on histological analysis. Eight months after the surgery, a mass infiltrating the mesentery was found and biopsy revealed IgG4-RD.Interventions:Both the orbital inflammation and abdominal mass infiltrating the mesentery were responsive to steroids and rituximab administered to treat IgG4-RD.Outcomes:In the course of IgG4-RD, the patient developed bilateral optic perineuritis, causing bilateral visual loss. Colon cancer with synchronous multiple liver metastases was found 1 year after rituximab treatment.Lessons:This case raises the possibility of IgG4-RD being a paraneoplastic syndrome in some patients. Cancer screening should probably be performed in some elderly patients diagnosed with IgG4-RD.

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IgG4-related disease presenting as hypertrophic pachymeningitis and compressive optic neuropathy

Cited by 15 publications

References 10 publications

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

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

Neurological Manifestations of IgG4-Related Disease

Colon cancer and IgG4-related disease with orbital inflammation and bilateral optic perineuritis

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