Correlation between peripapillary retinal thickness and serum level of vascular endothelial growth factor in patients with POEMS syndrome

Yokouchi, Hirotaka; Baba, Takayuki; Misawa, Sonoko; Sawai, Setsu; Kitahashi, Masayasu; Oshitari, Toshiyuki; Kuwabara, Satoshi; Yamamoto, Shuichi

doi:10.1136/bjophthalmol-2015-307068

Cited by 16 publications

(7 citation statements)

References 22 publications

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“…VEGF is a cytokine produced by plasma cells that increases vascular permeability and causes capillary leak syndrome, leading to pleural effusion, ascites, and peripheral edema [ 7 ]. In addition, increased vascular permeability causes optic disc swelling and papilledema [ 8 ]. VEGF testing is useful for diagnosing and evaluating treatment response [ 5 ].…”

Section: Diagnosismentioning

confidence: 99%

Update on the POEMS syndrome

Kim

2022

Blood Res

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POEMS syndrome is an acronym for polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. It is a rare paraneoplastic disorder related to plasma cell neoplasm. However, its pathophysiology has not yet been clearly elucidated. The production of pro-inflammatory cytokines is thought to play an important role in the pathogenesis of POEMS syndrome. Vascular endothelial growth factor level reflects disease activity, which is helpful for the diagnosis and evaluation of treatment response. Conventional agents such as corticosteroids and melphalan are effective and safe combination regimens. Autologous hematopoietic stem cell transplantation is another option for high-risk transplant-eligible patients. Radiotherapy is effective in patients with localized lesions. The anti-myeloma agents lenalidomide, thalidomide, and bortezomib have shown good treatment outcomes for POEMS syndrome; however, large-scale studies with long-term follow-up are required. Early identification and active treatment can improve the outcomes of POEMS syndrome patients.

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Section: Diagnosismentioning

confidence: 99%

Update on the POEMS syndrome

Kim

2022

Blood Res

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“…17 The germinal centres of hyperplastic lymph nodes in CD have also been shown to produce significant amounts of IL-6, which stimulates VEGF production, leading to angiogenesis. 20,21 VEGF has specifically been implicated as a possible instigator of papilledema in POEMS, and the degree of papilledema 22 and peripapillary retinal thickness 23 has been shown to correlate with plasma VEGF levels and respond to anti-VEGF therapy in these patients. 24 Castleman tumourinduced increase in IL-6 and VEGF may explain the papilledema found across the spectrum of CD, from UCD as in our case, to MCD with POEMS syndrome.…”

Section: Discussionmentioning

confidence: 99%

Castleman Disease Presenting with Pseudotumour Cerebri and Myasthenia Gravis: A Case Report and Literature Review

Fein

Bittar

Shende

et al. 2018

Neuro-Ophthalmology

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Castleman disease (CD) is a rare lymphoproliferative disorder that may present with various autoimmune, inflammatory, or neurologic syndromes. This is a case of a 21-year-old woman who presented with signs and symptoms of pseudotumour cerebri (PTC) who subsequently developed myasthenia gravis (MG), and was incidentally found to have a large mass in the posterior mediastinum. Upon resection, the mass was classified as unicentric CD involved with follicular dendritic cell sarcoma. Following treatment with IVIG in the setting of progressive weakness and dyspnea, she has had complete symptom resolution while maintained on a low dose of pyridostigmine for the last two years. There are 13 cases of MG and five cases of optic disc edema described as PTC associated with CD in the literature, but to our knowledge, this is the sole case reported of the intersection of all three conditions in one patient. Increased serum levels of interleukin-6 and vascular endothelial growth factor may provide clues as to the association of CD with these neurologic syndromes.

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“…Increased vascular permeability secondary to elevated VEGF may also be important. In a study of 17 patients with POEMS syndrome, serum VEGF concentrations were significantly higher in patients with disc swelling compared to those without, and there was a positive correlation between serum VEGF levels and peripapillary retinal thickness on OCT [ 27 ]. None of the studied patients had elevated CSF opening pressures.…”

Section: Clinical Featuresmentioning

confidence: 99%

POEMS syndrome: clinical update

Brown

Ginsberg

2018

J Neurol

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POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) is a rare paraneoplastic syndrome, caused by a plasma cell proliferative disorder, which is most commonly lambda restricted. The neurological hallmark, which forms one of the mandatory criteria for diagnosis, is a subacute onset demyelinating neuropathy, which can be rapidly disabling and painful. A number of multi-system features are also characteristic of this disorder, and certainly not restricted to those included in its acronym, which though limited, remains a useful and memorable name, helping distinguish POEMS syndrome from other paraproteinaemic neuropathies. The discovery of vascular endothelial growth factor (VEGF) in association with POEMS syndrome has been extremely useful in aiding clinical diagnosis, and monitoring response to treatment, as well as helping understand the underlying mechanism of disease. Interestingly, however, treatment targeting VEGF has been disappointing, suggesting other disease mechanisms or inflammatory processes are also important. Current understanding of the pathogenesis of POEMS syndrome is outlined in detail in the accompanying article by Cerri et al. Here, we review the clinical features of POEMS syndrome, differential diagnosis and available treatment options, based on current literature.

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Correlation between peripapillary retinal thickness and serum level of vascular endothelial growth factor in patients with POEMS syndrome

Abstract: The significant correlation between the pRT and the serum level of VEGF suggests that the higher serum level of VEGF might be associated with the development of the optic disc oedema in patients with POEMS syndrome.

Cited by 16 publications

References 22 publications

Update on the POEMS syndrome

Update on the POEMS syndrome

Castleman Disease Presenting with Pseudotumour Cerebri and Myasthenia Gravis: A Case Report and Literature Review

POEMS syndrome: clinical update

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