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      Drugs & Diseases > Ophthalmology

      Vogt-Koyanagi-Harada (VKH) Disease Treatment & Management

      Updated: Mar 09, 2023
      • Author: Fatma Zaguia, MD; Chief Editor: Hampton Roy, Sr, MD  more...
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      Treatment

      Approach Considerations

      The key to successful therapy for Vogt-Koyanagi-Harada (VKH) disease is early and aggressive systemic treatment. [1, 2] Those patients who are treated later in the course of the disorder have a more guarded prognosis for recovery of visual acuity and probably have a greater risk for chronic inflammation.

      For pigmentary changes in VKH disease, treatment options mirror those for vitiligo. Chronic VKH disease requires monitoring for many years.

      Surgical care

      Surgical therapy for glaucoma is necessary in some patients. Surgical intervention includes laser iridotomy, surgical iridectomy, and trabeculectomy.

      Consultations

      Ophthalmologic and neurologic consultations are necessary in the treatment of VKH disease.

      Next:

      Systemic Therapy

      For most patients with bilateral serous detachments and severe visual loss, begin therapy with systemic prednisone (1-2 mg/kg/day). In the most severe cases, some clinicians use intravenous methylprednisolone (up to 1 g/day) for several days before beginning oral prednisone (1 mg/kg/day). However, the addition of intravenous therapy does not appear to alter the visual outcome or the development of significant ocular complications.

      The length of treatment and subsequent taper must be individualized for each patient. Most patients require therapy for 6 months and occasionally up to 1 year before successful tapering of systemic corticosteroids. In general, systemic therapy should not be discontinued during the 3 months following the onset of the disease because of the risk for recurrence.

      For patients who fail to respond to high-dose systemic corticosteroids or who develop intolerable adverse effects, immunomodulatory therapy, such as treatment with cyclosporine, tacrolimus, mycophenolate mofetil, azathioprine, cyclophosphamide, or chlorambucil, should be instituted. Increasing evidence supports the use of immunomodulatory therapy in virtually all patients with VKH. [3, 69, 70, 71, 72, 73]  There is a growing body of evidence suggesting corticosteroid monotherapy in early-stage VKH is inadequate, [74, 75]  with significant rates of chronic recurrent disease, subretinal fibrosis, worse visual acuity, and  progression to sunset glow fundus. [76, 77]  Case series have shown that patients who received prompt immunosuppressive agents had a better visual outcome than those who received prolonged treatment with corticosteroids. [78]

      Case reports suggest that intravenous immunoglobulins (IVIGs) [79, 80] and infliximab [81, 82] may be of interest in the treatment for VKH syndrome. Further trials are needed to assess the efficacy of these agents.

      If systemic medications are not effective, [83] subtenon injections may be considered before intraocular treatment modalities are used. [84]

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      Topical Therapy

      Topical corticosteroids, such as prednisolone acetate, are used for the treatment of anterior uveitis. In severe cases, begin therapy with 1 drop up to every hour and slowly taper based on the therapeutic response. In patients with mild to moderate anterior uveitis, begin with 1 drop 4-6 times daily and taper slowly.

      Cycloplegic-mydriatic eye drops are used symptomatically. Topical cycloplegics are useful for the relief of the discomfort of ciliary spasm and for the prevention of the formation of posterior synechiae. Discontinue as the inflammation wanes and symptoms resolve.

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