Concurrent acute Vogt-Koyanagi-Harada disease in one eye and chronic disease in the fellow eye
© Khochtali et al. 2015
Received: 9 July 2015
Accepted: 14 August 2015
Published: 3 September 2015
Vogt-Koyanagi-Harada (VKH) disease is a granulomatous panuveitis, usually involving both eyes at the same time or within a few days or weeks. Acute and chronic diseases are characterized by distinct clinical features, treatment modalities, and visual outcomes. We report an atypical case of probable VKH disease, with features of acute disease in one eye and chronic disease in the fellow eye.
A 53-year-old female presented with exudative retinal detachment associated with mild vitritis in the right eye and anterior uveitis, vitritis, and sunset-glow fundus in the left eye. Based on clinical findings and results of multimodal imaging including fundus photography, spectral-domain optical coherence tomography, fluorescein angiography, indocyanine green angiography, and B-scan ultrasonography, a diagnosis of acute VKH disease in the right eye and chronic VKH disease in the left eye was made. The patient received systemic corticosteroids and mycophenolate mofetil. After a 15-month follow-up, the right fundus eye was normal, and there was a sunset-glow fundus in the left eye.
VKH disease may begin with asymptomatic unilateral ocular involvement. The patient may present only when the fellow eye is affected. A significant delay before involvement of the second eye leads to atypical features of acute disease in one eye and chronic disease in the other eye.
KeywordsVogt-Koyanagi-Harada disease Exudative retinal detachment Sunset-glow fundus
Vogt-Koyanagi-Harada (VKH) disease is often defined as a bilateral, chronic granulomatous panuveitis associated with central nervous system, auditory, and integumentary manifestations. The diagnosis is established on the basis of the Revised Diagnostic Criteria for VKH disease . The disease is bilateral and naturally evolves through four consecutive stages. The prodromal stage including neurological and auditory manifestations is followed after few days by the acute uveitic stage. Acute VKH disease typically presents in the form of localized or multifocal exudative retinal detachment (ERD) associated with intraocular inflammatory reaction. It usually affects both eyes at the same time. However, onset may be unilateral, and the second eye may become involved 2 to 4 weeks later [1–3]. The chronic convalescent stage occurs several weeks after the acute stage and is characterized by the development of ocular depigmentation, the sunset-glow fundus being the most specific feature, associated or not with integumentary depigmentation. The chronic recurrent stage usually manifests with granulomatous anterior uveitis and is associated with higher risks of complications and vision loss [2, 3].
We report herein an atypical case of probable VKH disease with concurrent findings of acute disease in one eye and chronic disease in the fellow eye, at first presentation.
A 53-year-old female with a history of arterial hypertension presented to our department, with bilateral vision blurring for 5 days. She reported no previous ocular complaints or neurological or auditory symptoms.
Spectral-domain optical coherence tomography (SD-OCT) showed ERD with a few subretinal septa and undulations of the retinal pigment epithelium (RPE) in the RE. Findings were unremarkable in the LE (Fig. 1).
Medical history excluded ocular trauma or surgery. Findings in each eye apart were very suggestive of VKH disease, in its acute phase in the RE and in its chronic phase in the LE. Such asymmetry being atypical, a workup was ordered to rule out conditions mimicking VKH disease. Results of systemic examination by the internist, complete blood cell count, sedimentation rate, C-reactive protein, syphilis serology, skin tuberculin test, chest X-ray, and oculo-cerebral magnetic resonance imaging were unremarkable.
This case report was approved by the ethics committee of Fattouma Bourguiba University Hospital, Monastir, Tunisia.
Our patient had an unusual presentation of VKH disease with features of acute disease in one eye and chronic disease in the fellow eye. Other causes of ocular inflammation including malignant hemopathies or other neoplasms, syphilis, tuberculosis, or sarcoidosis were excluded in our patient. The diagnosis of posterior scleritis could be ruled out based on the lack of ocular pain and clinical and ultrasonographic findings.
VKH disease is usually characterized by simultaneous onset of inflammation in both eyes, although the involvement of the second eye may occur 2 to 4 weeks after the first eye [1–3]. There have been very rare cases of VKH disease that remained unilateral after a long follow-up period. However, subclinical disease in the fellow eye was not ruled out in these patients by indocyanine green angiography or B-scan ultrasonography, prior to the initiation of systemic corticosteroids . Unilateral features of acute VKH disease have also been described in a 4-year-old boy. However, ultrasonography in this case revealed choroidal thickening in both eyes, which was consistent with bilateral involvement . A long delay between the first and second eye involvement, ranging from 11 months to 6 years, has been previously reported in four patients with a history of unilateral panuveitis before the definitive diagnosis of VKH disease was established. Baseline subclinical inflammation of the initially unaffected eye might have been overlooked in these cases [6, 7]. On the other hand, prolonged initial unilateral ocular involvement in VKH disease may be misdiagnosed as posterior scleritis . Among these patients with a long interval before the second eye was affected, concomitant sunset-glow fundus in one side and ERD consistent with acute VKH disease in the other side has been noted in only two patients [6, 7].
To the best of our knowledge, our report is the first to describe concurrent acute VKH in one eye and sunset-glow fundus in the fellow eye at initial presentation. This striking contrast between the right and the left eyes might suggest a significant delay before the second eye was affected. However, our patient did not report any ocular complaints when the first eye was affected. She presented only when vision blurring became bilateral. The mechanism of delayed involvement of the second eye remains unclear. Early bilateral involvement with minimal inflammation in the RE seems unlikely in our patient. In fact, without any treatment, sunset-glow fundus would have developed in both eyes.
In summary, patients with VKH disease may present with atypical features of chronic disease in one eye and acute disease in the fellow eye. Initial unilateral ocular inflammation, progressing relentlessly to chronic disease, may remain asymptomatic until the fellow eye is involved. Accurate diagnosis is essential for prompt initiation of corticosteroid and immunosuppressive therapy to reduce the risk of visual morbidity.
best corrected visual acuity
exudative retinal detachment
retinal pigment epithelium
spectral-domain optical coherence tomography
This work has been supported by the Ministry of Higher Education and Research of Tunisia.
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- Read RW, Holland GN, Rao NA, Tabbara KF, Ohno S, Arellanes-Garcia L, Pivetti-Pezzi P, Tessler HH, Usui M (2001) Revised diagnostic criteria for Vogt-Koyanagi-Harada disease: report of an international committee on nomenclature. Am J Ophthalmol 131(5):647–52View ArticlePubMedGoogle Scholar
- Attia S, Khochtali S, Kahloun R, Zaouali S, Khairallah M (2012) Vogt–Koyanagi–Harada disease. Expert Rev Ophthalmol 7(6):565–585View ArticleGoogle Scholar
- Rao NA, Gupta A, Dustin L, Chee SP, Okada AA, Khairallah M, Bodaghi B, Lehoang P, Accorinti M, Mochizuki M, Prabriputaloong T, Read RW (2010) Frequency of distinguishing clinical features in Vogt-Koyanagi-Harada disease. Ophthalmology 117(3):591–9.e1PubMed CentralView ArticlePubMedGoogle Scholar
- Usui Y, Goto H, Sakai J, Takeuchi M, Usui M, Rao NA (2009) Presumed Vogt-Koyanagi-Harada disease with unilateral ocular involvement: report of three cases. Graefes Arch Clin Exp Ophthalmol 247(8):1127–32View ArticlePubMedGoogle Scholar
- Forster DJ, Green RL, Rao NA (1991) Unilateral manifestation of the Vogt-Koyanagi-Harada syndrome in a 7-year-old child. Am J Ophthalmol 15;111(3):380–2View ArticleGoogle Scholar
- Roe RH, Rathinam SR, Wong RW, McDonald HR, Jumper JM, Cunningham ET Jr (2009) Delayed fellow eye involvement in patients with Vogt-Koyanagi-Harada disease. Br J Ophthalmol 93(5):701–2View ArticlePubMedGoogle Scholar
- Far NY, Liu DT (2012) Probable Vogt-Koyanagi-Harada disease with initial unilateral ocular manifestation in a hepatitis C carrier. J Ophthalmic Inflamm Infect 2(4):235–7PubMed CentralView ArticlePubMedGoogle Scholar
- Kouda N, Sasaki H, Harada S, Yamada Y, Takahashi N, Sasaki K (2002) Early manifestation of Vogt–Koyanagi–Harada disease as unilateral posterior scleritis. Jpn J Ophthalmol 46:590–3View ArticlePubMedGoogle Scholar