What is the treatment and management of multifocal choroiditis with panuveitis?

Multifocal Choroiditis with Panuveitis: Clinical Features and Management

Clinical Presentation and Demographics

Multifocal choroiditis with panuveitis (MCP) is a bilateral inflammatory disorder predominantly affecting young to middle-aged women, characterized by multiple yellow-white or gray focal lesions at the level of the retinal pigment epithelium and choroid, accompanied by inflammation in all uveal compartments. 1

• The disease typically presents bilaterally, though rare unilateral cases exist 1
• Visual acuity at presentation is frequently compromised, with 55% of affected eyes having vision of 20/50 or worse and 38% having 20/200 or worse 2
• Patients present with floaters, blurred vision, photophobia, and visual field changes 3

Diagnostic Workup

Multimodal imaging is essential to establish diagnosis and guide treatment decisions. 1

• Optical coherence tomography (OCT) identifies subretinal fluid, outer retinal disruption, cystoid macular edema (CME), and subretinal fibrosis 1
• Fluorescein angiography (FA) demonstrates focal leakage points and choroidal neovascularization (CNV) 1
• Indocyanine green angiography (ICGA) shows hypofluorescent changes on late-phase imaging characteristic of white dot syndromes 1
• OCT-angiography detects neovascular networks when present 1

Treatment Algorithm

Acute Management

Systemic corticosteroids must always be combined with immunosuppressive agents and never used as monotherapy for posterior uveitis. 1, 4

• Local corticosteroids (topical drops, periocular injections) may be used for acute inflammation control 1
• High-dose systemic corticosteroids provide rapid suppression during acute attacks but require concurrent immunosuppression 3

First-Line Systemic Immunosuppression

Patients with MCP affecting the posterior segment should be on systemic immunomodulatory therapy with azathioprine, cyclosporine-A, interferon-alpha, or monoclonal anti-TNF antibodies. 4

• Azathioprine has level IB evidence for posterior segment inflammation 3, 4
• Cyclosporine-A has level IB evidence for preserving visual acuity and preventing relapses 3, 4
• Interferon-alpha has level IIA evidence for effectiveness in posterior uveitis 3, 4
• Monoclonal anti-TNF antibodies (infliximab or adalimumab) have level IIA evidence and should be considered for refractory cases 3, 4

Evidence for Immunosuppression Benefits

Immunosuppressive drug therapy reduces the risk of posterior pole complications by 83% and the risk of vision loss to 20/200 or worse by 92% in affected eyes. 2

• Treatment with immunosuppressives significantly reduces new-onset and recurrent choroidal neovascularization 2
• Low-dose corticosteroid monotherapy does not provide the same protective benefit 2

Management of Choroidal Neovascularization

Intravitreal anti-VEGF injections are the primary treatment for choroidal neovascularization, which occurs in 16-22% of eyes and is the leading cause of vision loss in MCP. 1, 5, 2

• CNV accounts for approximately 45% of incident vision loss during follow-up 2
• Photodynamic therapy may be considered as adjunctive treatment in select cases 1, 6
• Periocular corticosteroids can be used for acute exacerbations in conjunction with systemic therapy 6

Long-Term Complications and Monitoring

Common Complications

• Cystoid macular edema occurs in 29.7% of eyes and is the most frequent macular abnormality 5
• Cataract (posterior subcapsular and/or nuclear) is the most common long-term complication, occurring in 40% of affected eyes 5
• Glaucoma develops in 10.8% of eyes 5
• Epiretinal membrane formation is associated with vision loss during follow-up 2
• Cataract surgery improves visual acuity in 83.3% of operated eyes 5

Visual Prognosis

Visual acuity decreases over time in patients with MCP, with the incidence rate of vision loss to 20/50 or worse being 0.19 per eye-year in affected eyes. 5, 2

• The incidence of vision loss to 20/200 or worse is 0.12 per eye-year in affected eyes 2
• Vision loss results from both the inflammatory process itself and iatrogenic complications from chronic corticosteroid use 5

Monitoring Strategy

Regular ophthalmologic surveillance is mandatory, with monitoring for at least 3 years after achieving remission to detect recurrence early. 1, 4

• After stopping immunosuppression, patients should be screened by an ophthalmologist at least every 3 months for a minimum of 1 year 3
• The period of uveitis inactivity should be greater than 2 years before withdrawing immunosuppressive therapy 3
• Patients should have 2 years of inactive disease while not using topical steroids before reducing systemic immunosuppression 3

Critical Pitfalls to Avoid

Abrupt discontinuation of immunosuppression can trigger severe rebound inflammation with multilevel inflammatory activity in the posterior pole, including new inflammatory lesions, ellipsoid zone loss, and widespread inflammatory cell infiltration into the outer retina. 6

• Before initiating systemic immunomodulatory therapy, screen for latent or active infections, particularly tuberculosis 4
• Perform baseline organ function tests before starting immunosuppressive agents 4
• Multidisciplinary coordination between ophthalmologists and other specialists (rheumatologists, internists) is essential for long-term management 4
• Unlike anterior uveitis, posterior uveitis can be asymptomatic but nonetheless proceed to visual loss, requiring vigilant monitoring 3