Optic neuritis as an initial presentation of Wegener's granulomatosis

Moubayed, Sami P.; Black, Daniel Ovid

doi:10.3129/i09-145

Cited by 11 publications

(4 citation statements)

References 5 publications

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“…Granulomatous vasculitis including sarcoidosis and Wegener's granulomatosis was also found to be an independent risk factor for optic neuritis after adjusting for other confounders (adjusted OR: 18.28, 95% CI: 2.21-151.45, p = 0.0071). This finding is consistent with results of several previous studies which reports the association between optic neuritis and Wegener's granulomatosis (10,(18)(19)(20), or sarcoidosis (38,39). Sarcoidosis is a chronic multisystemic inflammatory disease that commonly affects the visual and neurological systems: the most common neuro-ophthalmic manifestation is optic neuropathy.…”

Section: Discussionsupporting

confidence: 93%

“…Although the pathophysiology of optic neuritis remains unclear, it is considered an immune-mediated disease (6) and could be a risk factor or the first clinical manifestation of autoimmune disease following systemic involvement (7). People may have a greater propensity for optic neuritis if they have underlying autoimmune diseases, including Sjögren syndrome (8,9,10), rheumatoid arthritis (8,11), ankylosing spondylitis (7,11,12), multiple sclerosis (10,(13)(14)(15), systemic lupus erythematosus (7,16,17), and granulomatous vasculitis (10,(18)(19)(20). Moreover, some systemic infections, such as human herpes (21-24) and tuberculosis 10,12,25), with the ability to induce autoimmune reactions, may trigger optic neuritis attacks.…”

Section: Introductionmentioning

confidence: 99%

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Association between Sjögren syndrome, sociodemographic factors, comorbid conditions, and optic neuritis: a Taiwanese population-based study

Jan,

Ho,

Sung

et al. 2024

Front. Neurol.

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PurposeOur study aimed to explore the correlation between Sjögren syndrome, sociodemographic factors, comorbid conditions, and optic neuritis.MethodsThis retrospective, nationwide, population-based, matched case–control investigation involved 33,190 individuals diagnosed with optic neuritis, identified using the International Classification of Diseases, Ninth Revision, Clinical Modification codes 377.30 for optic neuritis or 377.32 for retrobulbar neuritis. Patient data were extracted from the Taiwan National Health Insurance Research Database. Demographic characteristics, the presence of Sjögren syndrome, and pre-existing comorbid conditions were analyzed using univariate logistic regression. Continuous variables were assessed with a paired t-test. Adjusted logistic regression was employed to compare the prognosis odds ratio (OR) of patients with optic neuritis to controls.ResultsAfter adjusting for confounding variables, individuals with Sjögren syndrome exhibited a significantly higher likelihood of developing optic neuritis compared to controls (adjusted OR, 9.79; 95% confidence interval [CI], 7.28–12.98; p < 0.0001). Other conditions associated with increased odds of optic neuritis included rheumatoid arthritis, ankylosing spondylitis, multiple sclerosis, systemic lupus erythematosus, and granulomatous vasculitis (adjusted OR: 1.57, 95% CI: 1.33–1.86; adjusted OR: 2.02, 95% CI: 1.65–2.48; adjusted OR: 140.77, 95% CI: 35.02–565.85; adjusted OR: 2.38, 95% CI: 1.71–3.30; adjusted OR: 18.28, 95% CI: 2.21–151.45, respectively), as well as systemic infections such as human herpes viral infection and tuberculosis infection (adjusted OR: 1.50, 95% CI: 1.35–1.66; adjusted OR: 4.60, 95% CI: 3.81–5.56, respectively).DiscussionOur findings strongly support the existence of an association between Sjögren syndrome, rheumatoid arthritis, ankylosing spondylitis, multiple sclerosis, systemic lupus erythematosus, granulomatous vasculitis, human herpes viral infection, tuberculosis, and optic neuritis.

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Section: Discussionsupporting

confidence: 93%

Section: Introductionmentioning

confidence: 99%

Association between Sjögren syndrome, sociodemographic factors, comorbid conditions, and optic neuritis: a Taiwanese population-based study

Jan,

Ho,

Sung

et al. 2024

Front. Neurol.

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“…In the past 20 years, only 16 well-documented cases of isolated optic neuropathies without pseudotumor have been reported in the literature (Table 3). 5–14 All presented with sudden or rapidly progressive severe visual loss, seven patients had retrobulbar pain, and two developed oculomotor palsy. CRP was less than 10 mg/L for five out of the six patients tested.…”

Section: Resultsmentioning

confidence: 99%

Inflammatory optic neuropathy in granulomatosis with polyangiitis can mimick isolated idiopathic optic neuritis

Clément

Néel

Toulgoat

et al. 2019

European Journal of Ophthalmology

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Objective: We describe a clinico-radiological presentation of inflammatory optic neuropathy that mimicked optic neuritis. Methods: Retrospective single-center case series and literature review of optic neuropathy without orbital pseudotumor. Results: Five local patients fulfilled the inclusion criteria. Clinical presentation revealed rapidly progressive severe unilateral visual loss, retrobulbar pain (n = 4), and paralytic strabismus (simultaneous = 2, protracted = 2) without proptosis. Optic nerve abnormality was not appreciated on initial scan review. Patients did not have any general activity of the granulomatosis with polyangiitis. Upon follow-up magnetic resonance imaging and initial imaging review, all patients revealed orbital apex anomalies. Visual acuity improved in three patients who received high-dose intravenous glucocorticosteroids immediately. Relapse was frequent and visual outcome was poor (final vision > 20/40 in two patients only). Literature review identified 16 well-documented cases of granulomatosis with polyangiitis–related isolated optic neuropathies. Magnetic resonance imaging revealed no abnormality (n = 6), optic nerve and/or sheath involvement (n = 9), apex infiltration (n = 3), and/or pachymeningitis (n = 7). Conclusion: Granulomatosis with polyangiitis is a rare yet potentially blinding cause of inflammatory optic neuropathy. Optic neuropathy in granulomatosis with polyangiitis may occur in the absence of systemic symptoms of disease activity and is challenging to distinguish from other inflammatory and non-inflammatory disorders affecting visual acuity. Several clinical and imaging clues suggest that optic neuropathy results from the development of an extravascular granulomatous process within the optic nerve sheath in the orbital apex, a place that is difficult to image. In a granulomatosis with polyangiitis patient with unexplained visual loss and a seemingly normal workup (fundoscopy, biology, and imaging), clinician should keep a high index of suspicion.

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“…Some complications are rare in this disorder, including central nervous system (CNS) involvement especially optic neuritis, cranial nerve paralysis, pachymeningitis, or pituitary disorders while in some cases the presentation of the optic nerve disease was the first feature. [2][3][4][5] Management protocols, including corticosteroid and cyclophosphamide or rituximab therapy, can control the progress of the disease. Medical treatments are the mainstay in ocular or CNS involvement, too.…”

Section: Introductionmentioning

confidence: 99%

Pachymeningitis in a Patient With Granulomatosis With Polyangiitis; A Case Report

Alishiri¹,

Rahmanian

Ramezanpour

2021

Hosp Pract Res

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Introduction: Granulomatosis with polyangiitis (GPA) is a systematic and necrotizing vasculitis with positive autoimmune antibodies. Some studies have reported the prevalence of eye involvement between 40%-50% of cases. Retro orbital granuloma is a rare complication of GPA which should be treated by surgical involvements, while pachymeningitis can be diagnosed by MRI and treated by medical management. In this study, we tried to present a case of GPA with optic neuritis and typical central nervous system (CNS) involvement, while there were no definite features of sinusitis or kidney injuries. Case Presentation: A 15-year-old girl was admitted because of blurred vision in her left eye. She was a known case of GPA three years ago with initial features, including left facial nerve paresis due to pan-sinusitis and pulmonary cavity. Neurologic evaluations, including sensory and motor features, were normal, too. Ophthalmologic examinations showed that visual acuity of the right eye was good, while the visual acuity in the left eye decreased to the point of finger counting at a distance of 20 cm. The left eye Marcus gunn test was positive (3+); anterior and posterior eye segments were normal. The patient was evaluated by brain MRI with gadolinium and a pathologic enhancement in the left cavernous was seen which had a pressure effect on the optic nerve. She was treated by intravenous methylprednisolone followed by rituximab. Conclusion: Reporting orbital mass in a patient who had GPA can be supposed as granuloma which needs a biopsy to confirm a diagnosis. In our case, the imaging manifestation was heterodox for granuloma, while neurosurgical consultation recommended drug treatment for pachymeningitis.

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Optic neuritis as an initial presentation of Wegener's granulomatosis

Cited by 11 publications

References 5 publications

Association between Sjögren syndrome, sociodemographic factors, comorbid conditions, and optic neuritis: a Taiwanese population-based study

Association between Sjögren syndrome, sociodemographic factors, comorbid conditions, and optic neuritis: a Taiwanese population-based study

Inflammatory optic neuropathy in granulomatosis with polyangiitis can mimick isolated idiopathic optic neuritis

Pachymeningitis in a Patient With Granulomatosis With Polyangiitis; A Case Report

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