Neuro-ophthalmic sarcoidosis

Baughman, Robert P.; Weiss, Kenneth L.; Golnik, Karl C.

doi:10.2147/eb.s29401

Cited by 32 publications

(18 citation statements)

References 82 publications

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“…Some single associations between two involved organs have been published, e.g. liver and spleen or CNS and eye [40,41]. Utilising MCA, we were able to comprehensively analyse associations in organ involvement and to identify five distinct clinical phenotypes of organ involvement: an abdominal, an "OCCC", a musculoskeletal-cutaneous, a pulmonary-lymphonodal and an extrapulmonary phenotype.…”

Section: Discussionmentioning

confidence: 99%

Phenotypes of organ involvement in sarcoidosis

et al. 2018

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Sarcoidosis is a highly variable, systemic granulomatous disease of hitherto unknown aetiology. The GenPhenReSa (Genotype-Phenotype Relationship in Sarcoidosis) project represents a European multicentre study to investigate the influence of genotype on disease phenotypes in sarcoidosis.The baseline phenotype module of GenPhenReSa comprised 2163 Caucasian patients with sarcoidosis who were phenotyped at 31 study centres according to a standardised protocol.From this module, we found that patients with acute onset were mainly female, young and of Scadding type I or II. Female patients showed a significantly higher frequency of eye and skin involvement, and complained more of fatigue. Based on multidimensional correspondence analysis and subsequent cluster analysis, patients could be clearly stratified into five distinct, yet undescribed, subgroups according to predominant organ involvement: 1) abdominal organ involvement, 2) ocular-cardiac-cutaneous-central nervous system disease involvement, 3) musculoskeletal-cutaneous involvement, 4) pulmonary and intrathoracic lymph node involvement, and 5) extrapulmonary involvement.These five new clinical phenotypes will be useful to recruit homogenous cohorts in future biomedical studies.

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Section: Discussionmentioning

confidence: 99%

Phenotypes of organ involvement in sarcoidosis

et al. 2018

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“…158 For this neuro-ophthalmic manifestation, one should approach treatment in the same manner as neurosarcoidosis. 169 Systemic CSs remain the most rapid and effective treatment for patients with an inflammatory optic neuropathy but appear less helpful in those with granulomas. 158 In some cases, i.v.…”

Section: Optic Nerve Involvementmentioning

confidence: 99%

“…Some authors suggest starting a cytotoxic agent early in the treatment course unless the patient is highly responsive to initial CS. 169 Relapses occur in 25% of the patients, requiring an increase or an escalation of treatment, but is not a poor prognostic factor. 158 According to Kidd et al, the presence of neurosarcoidosis requires more prolonged higher-dose treatment with steroids and immunosuppression from the outset, with early recourse to biological agents, should the clinical and radiologic features of the disorder fail to improve quickly.…”

Section: Optic Nerve Involvementmentioning

confidence: 99%

Ocular Sarcoidosis

Sève

Jamilloux

Tilikete

et al. 2020

Semin Respir Crit Care Med

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Sarcoidosis is one of the leading causes of inflammatory eye disease. Any part of the eye and its adnexal tissues can be involved. Uveitis and optic neuropathy are the main manifestations, which may require systemic treatment. Two groups of patients with sarcoid uveitis can be distinguished: one of either sex and any ethnicity in which ophthalmological findings are various and another group of elderly Caucasian women with mostly chronic posterior uveitis. Clinically isolated uveitis revealing sarcoidosis remains a strictly ocular condition in a large majority of cases. Although it can be a serious condition involving functional prognosis, early recognition in addition to a growing therapeutic arsenal (including intravitreal implant) has improved the visual prognosis of the disease in recent years. Systemic corticosteroids are indicated when uveitis does not respond to topical corticosteroids or when there is bilateral posterior involvement, especially macular edema. In up to 30% of the cases that require an unacceptable dosage of corticosteroids to maintain remission, additional immunosuppression is used, especially methotrexate. As with other forms of severe noninfectious uveitis, monoclonal antibodies against tumor necrosis factor-α have been used. However, only very rarely does sarcoid uveitis fail to respond to combined corticosteroids and methotrexate therapy, a situation that should suggest either poor adherence or another granulomatous disease. Optic neuropathy often affects women of African and Caribbean origins. Some authors recommend that patients should be treated with high-dose of corticosteroids and concurrent immunosuppression from the onset of this manifestation, which is associated with a poorer outcome.

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“…Sarcoidosis is a multisystem disease that involves the central nervous system in about 5-10% of patients and neuro-ophthalmic involvement occurs in about half of these. [ 18 ] African Americans and Japanese have an increased rate of sarcoidosis. The diagnosis is suggested by positive angiotensin converting enzyme (60-70% of patients) and chest X-ray with hilar adenopathy (90% of patients), but definitive diagnosis is based on biopsy showing noncaseating granulomas.…”

Section: Optic Neuropathy Associated With Systemic Diseasesmentioning

confidence: 99%

“…The diagnosis is suggested by positive angiotensin converting enzyme (60-70% of patients) and chest X-ray with hilar adenopathy (90% of patients), but definitive diagnosis is based on biopsy showing noncaseating granulomas. [ 18 ] Optic nerve involvement may present as retrobulbar optic neuropathy, mild to severe disc swelling, NR and when discovered late’ optic atrophy. The optic neuropathy of sarcoidosis typically is painless or presents with mild pain, and visual loss is subacute.…”

Section: Optic Neuropathy Associated With Systemic Diseasesmentioning

confidence: 99%

Treatment options for atypical optic neuritis

Malik

Ahmed

Golnik

2014

Indian J Ophthalmol

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Context:Optic neuritis (ON) is defined as inflammation of the optic nerve and can have various etiologies. The most common presentation in the US is demyelinating, or “typical” ON, usually associated with multiple sclerosis. This is in contrast to “atypical” causes of ON, which differ in their clinical presentation, management, and prognosis. These atypical cases are characterized by lack of eye pain, exudates, and hemorrhages on exam, very severe, bilateral or progressive visual loss, or with failure to recover vision.Aims:The aim was to describe the clinical presentations of atypical ON and their treatments.Settings and Design:Review article.Materials and Methods:Literature review.Results:Types of atypical ON identified include neuromyelitis optica, autoimmune optic neuropathy, chronic relapsing inflammatory optic neuropathy, idiopathic recurrent neuroretinitis, and optic neuropathy associated with systemic diseases. Atypical ON usually requires corticosteroid treatment and often will require aggressive immunosuppression.Conclusions:Unlike demyelinating ON, atypical ON requires treatment to preserve vision.

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Neuro-ophthalmic sarcoidosis

Cited by 32 publications

References 82 publications

Phenotypes of organ involvement in sarcoidosis

Phenotypes of organ involvement in sarcoidosis

Ocular Sarcoidosis

Treatment options for atypical optic neuritis

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