Rare:  important cause of non-infectious uveitis in populations with a pigmented skin. Systemic autoimmune disease involving the tissues rich in melanocytes as the eye, the inner ear, meninges, hair and skin. The autoimmune process is directed against antigens associated with melanocytes and seems triggered by a viral infection, particularly in patients carrying the HLA-DRB1*0405 allele. The main differential diagnosis is sympathetic ophthalmia: this disease is characterized by the absence of history of surgery or ocular trauma.

Different stages:

-        a prodromal phase (3-5 days): clinical picture of viral infection sometimes accompanied by orbital pain, dizziness, photophobia and tearing

-        a stage of acute uveitis (weeks): blurred vision in one then both eyes; sometimes involvement of the anterior segment with granulomatous iridocyclitis (risk of acute closed angle glaucoma); sometimes meningeal signs (CSF: pleocytosis; sometimes: hemiparesis, impairment of the cranial nerves, aphasia) and tinnitus (70%) (with dysacousis/dysacusis)

-        a so-called convalescent phase (2-3 months later): depigmentation of the skin (vitiligo, alopecia, poliosis) and the choroid (zones of depigmentation at ophthalmoscopy)

-        a chronic or recurrent phase: which appears in 17 to 73% of cases: cataracts, glaucoma, neovascularization of the choroid and retinal fibrosis.

Treatment: high systemic doses of cortisone as soon as the diagnosis is made, cycloplegic eyedrops. Sometimes, methotrexate, cyclosporin A, infliximab.

Anesthetic implications:

side effects of treatment, eye protection, possible glaucoma

References : 

-         Lavezzo MM, Sakata VM, Morita C, Caso Rodriguez EE et al.
Vogt-Koyanagi-Harada disease : review of a rare autoimmune disease targeting antigens of melanocytes.
Orphanet J Rare Diseases 2016; 11:29

-        Escarza BS, Austin TM, Lam HV.
Anesthesia for a patient with Vogt-Koyanagi-Harada disease.
J Clin Anesth 2019 ; 58, 131.

Updated: September 2019