Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

acute episode

1st line – pulse dose corticosteroid

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ACUTE
|
acute episode
1st line – 

pulse dose corticosteroid

Corticosteroids are the treatment of choice for an acute episode of optic neuritis, whether that be a first episode or relapse. Corticosteroids are prescribed with the aim of reducing the duration of the acute phase and the risk of recurrence. Acute treatment involves high-dose intravenous corticosteroids (e.g., methylprednisolone) as per the Optic Neuritis Treatment Trial (ONTT) protocol, or no treatment based on the patient’s benefit versus risk ratio.[12]​​[20]

The ONTT has examined the role of corticosteroid treatment in the management of acute optic neuritis (ON) and the subsequent risk of development of multiple sclerosis (MS).

The ONTT confirmed that pulse dose intravenous methylprednisolone showed a better visual outcome at 6 months (the benefit disappeared at 2 years) and a lower risk of recurrence (conversion to clinically definite MS) than placebo or oral prednisone.[20]

It has been recommended that treatment should be initiated ideally in the first 48 hours of the onset of the symptoms, as it could be sight saving in ON that is not associated with MS (e.g., neuromyelitis optica spectrum disorder [NMOSD], ON associated with systemic lupus erythematosus [SLE) and that it should be introduced before blood test results are known (e.g., aquaporin-4 or MOG antibodies).[49]

Intravenous methylprednisolone is as safe, well-tolerated and effective as oral methylprednisolone, with the latter being more convenient.[47][48] Dose regimens may vary. Consult a specialist for guidance on the most appropriate treatment regimen. Some experts may recommend alternative high dose oral corticosteroids, some may omit the oral reducing dose after intravenous treatment, and some may recommend a 5-day treatment course if there is only a partial response to initial treatment.

In general, gastric irritation is not a problem with this corticosteroid regimen, but protective medications (e.g., proton-pump inhibitors) can be used as appropriate.

Primary options

methylprednisolone: 1000 mg orally/intravenously once daily for 3 days initially, followed by oral prednisone

and

prednisone: 1 mg/kg orally once daily for 7-11 days after initial dosing with intravenous methylprednisolone

2nd line – plasma exchange

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ACUTE
|
acute episode
2nd line – 

plasma exchange

If ON is refractory to corticosteroids treatment with plasma exchange has been shown to be effective.[3][30] Intravenous immune globulin (IVIG) may be an option, but it has not specifically been evaluated in refractory ON and is rarely used in practice.[30]

ONGOING

coexisting inflammatory diseases (e.g., NMOSD, MOGAD, SLE or sarcoidosis)

1st line – immunosuppressive treatment

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ONGOING
|
coexisting inflammatory diseases (e.g., NMOSD, MOGAD, SLE or sarcoidosis)
1st line – 

immunosuppressive treatment

When optic neuritis (ON) is present as a part of neuromyelitis optica spectrum disorder (NMOSD) or myelin oligodendrocyte glycoprotein antibody associated neurologic disease (MOGAD) there is continuation of the corticosteroid treatment beyond the acute intravenous treatment, usually in the form of oral prednisone for several months. In addition, for NMOSD and MOGAD with negative prognostic features (e.g., persistently positive MOG antibodies) a corticosteroid-sparing immunosuppressive agent is introduced (e.g., azathioprine, mycophenolate).

In patients with proven or suspected NMOSD or MOGAD, plasma exchange or intravenous immune globulin (IVIG) can also be introduced in conjunction with oral corticosteroids.[30] In patients with NMOSD for an acute or relapse attack, not responding to corticosteroids alone, plasma exchange has shown benefit as an adjunct including promise when initiated early during an attack and in patients taking preventive immunosuppressants.[19][50][51][52]​​​​[53][54][55][56]

Intravenous immune globulin has also been used for corticosteroid-resistant relapses of ON.[57] Eculizumab (a complement inhibitor) has shown in patients with AQP4-IgG-positive NMOSD a significantly lower risk of relapse compared with placebo.[58]​ 

When ON is present as a part of other systemic or CNS inflammatory diseases (e.g., systemic lupus erythematosus, sarcoidosis), the treatment is aimed at the underlying inflammatory disease. This is usually with corticosteroids in various regimens, often including high-dose intravenous or oral corticosteroids for protracted periods of time. Corticosteroid-sparing immunosuppressive treatments (e.g., azathioprine, cyclophosphamide) are often used.

When conditions such as sarcoidosis are treated with tumor necrosis factor (TNF) alpha inhibitors (e.g., infliximab), the development of ON may represent a rare complication of anti-TNF treatment rather than a manifestation of sarcoidosis.

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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