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PubMed This is a summary of 14 peer-reviewed journal articles Updated
Ophthalmology

The Four Phases of VKH: What to Expect

At a Glance

Vogt-Koyanagi-Harada (VKH) disease progresses through four phases: a flu-like prodromal stage, an acute uveitic stage with severe eye inflammation and vision loss, a convalescent stage causing skin and hair pigment loss, and a chronic recurrent stage if not aggressively treated early.

Vogt-Koyanagi-Harada (VKH) disease is unique because it often follows a predictable path through four distinct stages. While it is primarily known for affecting the eyes, it is a multisystem condition, meaning it impacts various parts of the body including the skin, ears, and the lining of the brain [1][2].

Phase 1: The Prodromal Phase (The “Flu-like” Start)

The disease typically begins with symptoms that feel like a severe flu or a viral infection. This phase usually lasts a few days to several weeks [1].

  • Neurological Symptoms: You may experience intense headaches, a stiff neck (meningismus), fever, and nausea [3][4].
  • Audiovestibular Symptoms: Many patients notice ringing in the ears (tinnitus), dizziness or spinning sensations (vertigo), and sensitive hearing or mild hearing loss [5][6].

Phase 2: The Acute Uveitic Phase (Eye Involvement)

Shortly after the flu-like symptoms, the immune system begins a concentrated attack on the eyes. This phase is defined by two major clinical findings:

  • Bilateral Granulomatous Uveitis: This is a specific type of severe, chronic inflammation that affects the uvea (the middle layer of the eye) in both eyes simultaneously [7][2]. “Granulomatous” refers to the way the inflammatory cells clump together under a microscope [2].
  • Exudative Retinal Detachment: Because the inflammation is so intense, fluid begins to leak and collect underneath the retina [8]. This “exudative” (fluid-related) detachment causes sudden, significant blurring or loss of vision [9].

Phase 3: The Convalescent Phase (Skin and Color Changes)

Weeks or months later, as the initial intense inflammation subsides, the “convalescent” or recovery phase begins. This is when changes to body pigment often appear because the melanocytes (pigment cells) have been damaged [1][8].

  • Dermatological Symptoms: You may notice patches of skin losing color (vitiligo), patches of hair or eyelashes turning white (poliosis), or patches of hair loss (alopecia) [10][11].
  • Sunset Glow Fundus: Inside the eye, the loss of pigment can make the back of the eye (the fundus) look orange-red, a classic sign doctors call a “sunset glow fundus” [1][12].

Phase 4: The Chronic Recurrent Phase

If the disease is not fully controlled during the earlier stages, it may enter a chronic phase. This stage is characterized by repeated “flares” of inflammation [1]. These recurrences often focus on the front of the eye rather than the back and can lead to complications like cataracts or increased eye pressure (glaucoma) if not managed with long-term therapy [8][12].

Phase Primary Symptoms Timing
Prodromal Headache, fever, tinnitus, vertigo Days to weeks
Acute Uveitic Blurred vision, eye pain, fluid behind retina Weeks
Convalescent White hair patches, skin color loss, “sunset glow” eye Months
Chronic Recurrent Repeated eye redness/inflammation, vision fluctuations Months to years

Understanding which phase you are in helps your medical team decide how aggressive your treatment needs to be [13]. Early intervention during the “Acute Uveitic” phase is considered the “window of opportunity” to prevent the disease from progressing to the chronic stage [14].

Common questions in this guide

What are the first signs of VKH disease?
VKH disease often begins with a prodromal phase that feels like a severe flu or viral infection. Common early symptoms include intense headaches, a stiff neck, fever, nausea, ringing in the ears, and dizziness.
How does VKH disease affect my eyes and vision?
During the acute phase, VKH causes severe, chronic inflammation in the middle layer of both eyes, known as bilateral granulomatous uveitis. This intense inflammation can cause fluid to leak and collect under the retina, resulting in sudden blurred vision or vision loss.
Why does VKH cause my hair and skin to change color?
In the convalescent phase of VKH, the immune system damages the body's pigment cells, called melanocytes. This can result in patches of skin losing color, hair or eyelashes turning white, or an orange-red appearance in the back of the eye known as sunset glow fundus.
Can VKH symptoms come back after the initial flare-up?
Yes, if the early stages of VKH are not fully controlled, the disease can enter a chronic recurrent phase. This phase is marked by repeated flares of eye inflammation that can lead to cataracts or glaucoma if not managed with long-term therapy.
Should I see an audiologist if I experience ringing in my ears?
Because ringing in the ears and dizziness are common during the early stages of VKH, it is important to bring these symptoms up with your doctor. They may recommend seeing an audiologist to properly document and monitor any hearing changes or hearing loss.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Is the inflammation I'm experiencing considered 'granulomatous,' and how does that affect our treatment plan?
  2. 2.I’ve noticed some ringing in my ears; should I see an audiologist to document any hearing loss?
  3. 3.What are the signs that my disease is moving from the acute phase into the convalescent phase?
  4. 4.If I develop 'sunset glow fundus' or vitiligo, does that mean my treatment isn't working, or is that part of the disease's natural progression?

Questions For You

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References

References (14)
  1. 1

    Vitiligo as a First Sign of Vogt-Koyanagi-Harada Disease.

    Vukojević M, Vukojevic N, Vuković A, et al.

    Acta dermatovenerologica Croatica : ADC 2023; (31(4)):229-231.

    PMID: 38651852
  2. 2

    Evaluation of Vestibular Functions in Patients with Vogt-Koyanagi-Harada Disease.

    Fujiwara K, Morita S, Hoshino K, et al.

    Audiology & neuro-otology 2017; (22(3)):190-195 doi:10.1159/000481426.

    PMID: 29080887
  3. 3

    F-18 FDG PET Findings for Vogt-Koyanagi-Harada Disease.

    Park HL, Yoo IR, Park SY

    Nuclear medicine and molecular imaging 2017; (51(2)):190-192 doi:10.1007/s13139-015-0384-7.

    PMID: 28559946
  4. 4

    Vogt-Koyanagi-Harada Disease, a Rare Entity in Spain: The Challenge of Worldwide Immigration and Globalization.

    Fernández AB, Moyano SP, Husein-ElAhmed H, Juárez AMA

    European journal of case reports in internal medicine 2018; (5(7)):000886 doi:10.12890/2018_000886.

    PMID: 30756049
  5. 5

    A Case of Vogt-Koyanagi-Harada Disease: Diagnosis Based on the Presence of Vitiligo and Sunset Glow Fundus Without Ocular Symptoms.

    Tsutsui H, Fukuda K, Nakajima I, Yamashiro K

    Cureus 2025; (17(1)):e78209 doi:10.7759/cureus.78209.

    PMID: 40026970
  6. 6

    Vogt-Koyanagi-Harada disease.

    Street D, Sivaguru A, Sreekantam S, Mollan SP

    Practical neurology 2019; (19(4)):364-367 doi:10.1136/practneurol-2018-002152.

    PMID: 30890584
  7. 7

    Choroidal circulation impairment during the anterior recurrence of Vogt-Koyanagi-Harada disease confirmed with indocyanine green angiography and laser speckle flowgraphy.

    Takemoto Y, Namba K, Mizuuchi K, et al.

    Acta ophthalmologica 2016; (94(7)):e629-e636 doi:10.1111/aos.13024.

    PMID: 27079805
  8. 8

    Vogt-Koyanagi-Harada disease.

    O'Keefe GA, Rao NA

    Survey of ophthalmology 2017; (62(1)):1-25 doi:10.1016/j.survophthal.2016.05.002.

    PMID: 27241814
  9. 9

    Retromode Infrared Scanning Laser Ophthalmoscopy in Vogt-Koyanagi-Harada Disease.

    Yasuda S, Yoshida T, Suzuki Y, et al.

    Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie 2025; (263(4)):1089-1103 doi:10.1007/s00417-024-06693-6.

    PMID: 39585405
  10. 10

    Case of probable Vogt-Koyanagi-Harada syndrome: A rare presentation.

    Mehta KN, Daigavane S

    Indian journal of ophthalmology 2022; (70(7)):2684-2686 doi:10.4103/ijo.IJO_495_22.

    PMID: 35791205
  11. 11

    A Case of Vogt-Koyanagi-Harada Syndrome Mimicking Optic Neuritis.

    Amir MM, Masood A, Khan Z

    Journal of the College of Physicians and Surgeons--Pakistan : JCPSP 2018; (28(4)):325-326 doi:10.29271/jcpsp.2018.04.325.

    PMID: 29615180
  12. 12

    [A rare case of ocular scarrings in a patient with Vogt-Koyanagi-Harada disease].

    Belmokhtar A, Daoudi R

    The Pan African medical journal 2017; (28()):313 doi:10.11604/pamj.2017.28.313.4547.

    PMID: 29721143
  13. 13

    Vogt-Koyanagi-Harada Syndrome: A Diagnostic Conundrum.

    Hussain A, Khurana R

    Cureus 2021; (13(12)):e20138 doi:10.7759/cureus.20138.

    PMID: 35003972
  14. 14

    Catching the therapeutic window of opportunity in early initial-onset Vogt-Koyanagi-Harada uveitis can cure the disease.

    Herbort CP, Abu El Asrar AM, Takeuchi M, et al.

    International ophthalmology 2019; (39(6)):1419-1425 doi:10.1007/s10792-018-0949-4.

    PMID: 29948499

This page explains the stages and progression of VKH disease for educational purposes only. Always consult your ophthalmologist or specialist for an accurate diagnosis, symptom monitoring, and treatment plan.

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