Syndrome (Uveomeningitis)

BASICS

DESCRIPTION

• Bilateral granulomatous panuveitis with skin, meningeal, and auditory-vestibular involvement

• Usually occurs in more darkly pigmented individuals

EPIDEMIOLOGY

Incidence

• Rare

• More common in young patients (teens to early 40s)

• Slight female preponderance

• Occurs more often in the Spring and Fall

Prevalence

Very uncommon

RISK FACTORS

• Occurs most commonly in patients from the Middle East, South and East Asia, Latin America, and in Native Americans

• Rarely seen in Caucasians or patients from Africa

Genetics

HLA-DRB1*0405, HLA-DR4, and HLA-Dw53 have been identified as risk factors.

PATHOPHYSIOLOGY

T-cell mediated autoimmune disorder against melanocytes, specifically against tyrosinase and tyrosinase-related proteins.

ETIOLOGY

Unknown. Epstein-Barr virus has been suggested as a possible trigger, and injury to the skin has also been suggested as an inciting injury.

COMMONLY ASSOCIATED CONDITIONS

See Diagnosis, signs and symptoms.

DIAGNOSIS

HISTORY

Usually CNS findings first.

• History of blurred vision in one or both eyes, ocular pain, photophobia, and floaters.

• CNS: Heartache, stiff neck, periocular discomfort, vertigo, tinnitus, and dysacusis

• Later findings: Hair loss; skin/hair depigmentation, sensitivity to touch of the hair/skin

• The American Uveitis Society criteria for VKH are:

– No history of ocular trauma

– No other evidence of another disease process

– Early or late ocular disease as listed below

– Neurologic/auditory findings (subjective or objective)

– Skin findings: Poliosis, vitiligo, alopecia

PHYSICAL EXAM

• Bilateral granulomatous panuveitis, often with serous retinal detachments, dense vitreitis, optic disc edema, and Dalen-Fuchs nodules

– In chronic disease, disturbance of the retinal pigment epithelium can cause a sunset glow fundus

– Skin findings of poliosis, vitiligo, alopecia

– Auditory dysfunction

DIAGNOSTIC TESTS & INTERPRETATION

Lab

Rule out other causes: Syphilis (FTA-ABS), Lyme titers, Tuberculosis (PPD), Sarcoid (ACE, Chest x-ray/CT)

Imaging

Initial approach

• Fluorescein angiography (FA) is very helpful – delayed choroidal filling with pinpoint areas of hyperfluorescence later (starry sky) and optic disc leakage

• B-scan ultrasonography: Nonspecific choroidal thickening (helps to distinguish from posterior scleritis, uveal effusion syndrome, choroidal metastasis)

• Optical coherence tomography may show exudative retinal detachment (nonspecific).

Follow-up & special considerations

Weekly until inflammation under control, then every 3 months until off immunosuppression and inflammation free.

Diagnostic Procedures/Other

• Lumbar puncture: Pleocytosis occurs in up to 84% of patients.

• Detailed auditory testing to rule out other causes of central hearing loss

Pathological Findings

Diffuse granulomatous inflammation of the choroid with sparing of the choriocapillaris (in contrast to sympathetic ophthalmia).

DIFFERENTIAL DIAGNOSIS

• Sympathetic ophthalmia (history of ocular trauma/surgery)

• Central serous chorioretinopathy

• Uveal metastasis

• Idiopathic uveal effusion

• Posterior scleritis

• Sarcoidosis

• Syphilis

• Lyme

• Tuberculosis

• Ocular lymphoma

TREATMENT

MEDICATION

First Line

Prompt, aggressive corticosteroid treatment is critical (approximately 1–2 mg/kg/d initially). IV corticosteroids may hasten resolution and should be considered in severe cases. If inflammation present for more than 2–3 months on more than 10 mg daily, consider second line agent.

Second Line

• As patients may have inflammation for many months or years, given the risks of chronic prednisone use greater than 10 mg/d, early use of immunomodulatory therapy should be strongly considered.

• Agents such as azathioprine, mycophenolate mofetil (CellCept), cyclosporine, and anti-Tumor Necrosis Factor (TNF) agents (Remicade and Humira) are effective.

• A steroid implant (Retisert, fluocinolone acetonide, Bausch and Lomb) may be considered.

ADDITIONAL TREATMENT

Issues for Referral

Prompt referral to a uveitis specialist.

SURGERY/OTHER PROCEDURES

• Cataract surgery: The eye should be inflammation free for 3 months before surgery. Patients may still develop posterior synechiae.

• Uncommon. For complications of VKH: Draining chronic subretinal fluid, subfoveal choroidal neovascular membrane therapy with intravitreal injection of anti-VEGF agent, photodynamic therapy, or submacular surgery

IN-PATIENT CONSIDERATIONS

Initial Stabilization

High dose corticosteroids.

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Every week until inflammation free, then every 3 months until inflammation free off immunosuppressive medications.

PROGNOSIS

Guarded. Prompt, aggressive therapy with use of steroid sparing agents can be vision saving.

COMPLICATIONS

• Permanent vision loss

• Cataract formation, glaucoma, chronic retinal detachment, subretinal fibrosis, subfoveal choroidal neovascular membrane formation can occur.

ADDITIONAL READING

• Read RW, Holland GN, Rao NA, et al. Revised diagnostic criteria for Vogt-Koyanagi-Harada disease: report of an international committee on nomenclature. Am J Ophthalmol 2001;131(5):647–652.

• Lertsumitkul S, Whitcup SM, Nussenblatt RB, et al. Subretinal fibrosis and choroiral neovascularization in Vogt-Koyanagi-Harada syndome. Graefes Arch Clin Exp Ophthalmol 1999;237:1029–1045.

CODES

ICD9

• 360.12 Panuveitis

• 364.24 Vogt-koyanagi syndrome

CLINICAL PEARLS