A perplexing case of bilateral Vogt-Koyanagi-Harada syndrome

Rehna Rasheed; Kannisha Shah; Gopal S Pillai; CB Mithun; Natasha Radhakrishnan; Pooja Kandula

doi:10.4103/ijo.IJO_1522_21

CASE REPORT

Year : 2022 | Volume : 2 | Issue : 1 | Page : 120-122

A perplexing case of bilateral Vogt-Koyanagi-Harada syndrome

Rehna Rasheed, Kannisha Shah, Gopal S Pillai, CB Mithun, Natasha Radhakrishnan, Pooja Kandula
Department of Ophthalmology, Amrita Institute of Medical Sciences and Research Centre, Kochi, Kerala, India

Date of Submission	01-Jun-2021
Date of Acceptance	23-Jul-2021
Date of Web Publication	07-Jan-2022

Correspondence Address:
Dr. Kannisha Shah
Department of Ophthalmology, Amrita Institute of Medical Sciences, Ponekkara, Kochi - 682 041, Kerala
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_1522_21

Abstract

Vogt–Koyanagi–Harada (VKH) disease presents as a bilateral granulomatous panuveitis with systemic manifestations. We report a 60-year-old female who presented with features suggestive of VKH and was treated with steroids and immunosuppression. Although thorough investigation ruled out tuberculosis (TB) at presentation, she developed neurotuberculosis 2 months after initiating treatment. The primary diagnosis of VKH was hence revisited. Since both VKH and TB uveitis can present as chronic granulomatous panuveitis, it is important to differentiate between them. Once started on immunosuppression, patients should be kept on close follow-up for early detection of development of infections or reactivation of latent TB.

Keywords: Corticosteroid, granulomatous uveitis, immunosuppression, neurotuberculosis, VKH

How to cite this article:
Rasheed R, Shah K, Pillai GS, Mithun C B, Radhakrishnan N, Kandula P. A perplexing case of bilateral Vogt-Koyanagi-Harada syndrome. Indian J Ophthalmol Case Rep 2022;2:120-2

How to cite this URL:
Rasheed R, Shah K, Pillai GS, Mithun C B, Radhakrishnan N, Kandula P. A perplexing case of bilateral Vogt-Koyanagi-Harada syndrome. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Aug 14];2:120-2. Available from: https://www.ijoreports.in/text.asp?2022/2/1/120/334885

Vogt–Koyanagi–Harada (VKH) is a bilateral granulomatous panuveitis typically associated with choroiditis, serous retinal detachments, and disc edema. Treatment for VKH is systemic steroids with long-term immunosuppression.^[1] Tuberculosis (TB) is known to cause a multisystemic granulomatous inflammation. In the eye, it can cause bilateral chronic granulomatous panuveitis mimicking VKH and needs antitubercular treatment (ATT). Reactivation of latent TB is more commonly seen in immunosuppressed.^[2],[3] Here, we report an interesting case of a 60-year-old female who presented with features suggestive of VKH. After ruling out TB, she was treated with corticosteroids and immunosuppression. However, she developed neurotuberculosis (neuroTB), creating dilemma in our diagnosis. This case highlights the diagnostic dilemma between VKH and TB, and the possibility of reactivation of latent TB on immunosuppression. We also want to underline the fact that in selected cases, it may not be entirely possible to exclude the presence of underlying latent TB, even with extensive investigations.

Case Report

A 60-year-old woman presented with sudden diminution of vision in both eyes of 2 weeks duration associated with intermittent fever and headache. On evaluation, her best-corrected visual acuity (BCVA) was 20/70 in both eyes and intraocular pressure was normal. Slit-lamp examination revealed 2+ cells and flare in the anterior chamber (SUN classification) with retrolental cells. Fundus showed bilateral marked disc edema with extensive exudative retinal detachment involving the macula [Figure 1]a and [Figure 1]b. Optical coherence tomography (OCT) showed large sensorineural detachments with choroidal undulations, pigment epithelial detachments, and retinal folds [Figure 1]c and [Figure 1]d. Fundus fluorescein angiography showed multiple pinpoint leakages in both eyes and disc leak typical of VKH [Figure 1]e, [Figure 1]f, [Figure 1]g, [Figure 1]h. There was marked choroidal thickening on B scan ultrasonography (2.3 mm). Thus, a diagnosis of VKH was made. Pure tone audiometry revealed mild sensorineural hearing loss.

Figure 1: (a and b) – Fundus photos showing bilateral disc edema with multiple exudative sensori-neural detachments involving the macula. (c and d) – Optical coherence tomography of the macula showing sensorineural detachments along with choroidal undulations. (e and f) –Early phases of fundus fluoroscein angiography showing disc hyperfluoroscence suggestive of disc leakage and multiple pinpoint leakages. (g and h) – Late phases of fundus fluoroscein angiography showing increase in intensity and size of disc leak

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However, with the history of fever, headache, and meningismus, she was investigated to rule out TB. Cerebrospinal fluid study including acid fast bacilli microscopy and GeneXpert were negative for TB. Computed tomography of chest and abdomen and magnetic resonance imaging (MRI) of brain failed to reveal any features of TB. TB QuantiFERON Gold and Mantoux test were also negative.

She was treated with intravenous methylprednisolone pulse dose for 3 days followed by oral steroids. Mycophenolate mofetil (MMF) was also started. Her BCVA in both eyes improved to 20/30 with resolution of disc edema and sensorineural detachments [Figure 2]a, [Figure 2]b, [Figure 2]c, [Figure 2]d.

Figure 2: Posttreatment (a and b) – Fundus photo – bilateral resolved disc edema and sensorineural detachments. (c and d) – Optical coherence tomography of the macula showing normal foveal contour with resolved sensorineural detachments (e and f) – Magnetic resonance imaging of the brain showing multiple T2 FLAIR hyperintense ring enhancing lesions in right pons, mid brain, bilateral frontal and parietal lobes (yellow arrows), and bilateral basal ganglia

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Two months later, she presented with fever, headache, fatiguability, and loss of balance. Both eyes were quiet. MRI brain showed multiple T2 FLAIR hyperintense ring enhancing lesions in bilateral frontal and parietal lobes and bilateral basal ganglia [Figure 2]e and [Figure 2]f and Mantoux test became strongly positive. She was diagnosed as neuroTB and started on ATT along with oral steroids. MMF was stopped with the suspicion of TB being the cause for initial ocular presentation.

Over a period of 6 months, systemic steroids were tapered and stopped following which she presented with redness and pain in both eyes. BCVA was 20/40. Anterior segment showed Koeppe's and Busacca nodules with 2+ cells and flare. Fundus showed a typical sunset glow fundus [Figure 3]a, [Figure 3]b, [Figure 3]c, [Figure 3]d, [Figure 3]e. OCT retinal nerve fiber layer (RNFL) showed peripapillary RNFL thickening in both eyes [Figure 3]f. This indicated a recurrence of uveitis when immunosuppression was stopped. Repeat MRI however showed resolution of the ring enhancing lesions. Hence, she was restarted on topical steroids, systemic steroids, and MMF. After 6 months of treatment, her BCVA improved to 20/30, anterior chamber became quiet, and disc edema resolved.

Figure 3: After 6 months – following stoppage of systemic steroids (a-c) – Anterior segment showing Busaccas (black arrow showing nodule on iris surface) and Koeppes nodules (black arrow showing nodule at the pupillary margin) suggestive of anterior chamber reaction (d and e) – Fundus photos showing bilateral sunset glow fundus. (f) – Optical coherence tomography of the peripapillary retinal nerve fiber layer showing thickening bilaterally

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Discussion

VKH is an inflammatory, chronic granulomatous multisystem disorder that is characterized by bilateral panuveitis, exudative retinal detachment, and optic disc swelling. The acute stage of VKH needs to be treated with high-dose corticosteroid with or without immunosuppressive agents.^[4]

TB is a multisystemic granulomatous disease caused by mycobacterium TB, which can have pulmonary and extrapulmonary manifestations.^[5] A well-known complication of patients on long-term corticosteroids is reactivation of latent TB.^[6] Such patients are at a higher risk of developing disseminated infection and/or extrapulmonary TB with tuberculous lymphadenopathy being the most common manifestation followed by pleural, bone, abdominal, genitourinary, neuroTB, as well as ocular TB.^[7]

Our patient initially presented with bilateral disc edema, exudative retinal detachments, and neurosensory deafness typical of VKH. Based on this, our main differential was ocular TB.^[8] Apart from these ocular features, our patient also had systemic features like fever and meningismus which are common to both TB and VKH. Ocular TB can have different clinical presentations ranging from anterior granulomatous uveitis to choroidal tuberculomas and choroiditis. Exudative retinal detachment although rare is also documented in literature.^[8]

Although TB was ruled out initially, the diagnosis of VKH was revisited when she developed neuroTB while on immunosuppression. Nonetheless, the absence of ocular signs when she developed neuroTB, flare up of uveitis after stopping immunosuppression, and generalized sunset glow fundus are features in favor of VKH than ocular TB. It also suggests that neuroTB has occurred secondary to reactivation of an underlying latent TB. Another similar case has been reported in literature where a patient with VKH on corticosteroids developed TB-associated uveitis secondary to reactivation of latent TB.^[9] However, our case developed reactivation of latent TB in the form of neuroTB while on treatment with corticosteroids for VKH syndrome. Our case also highlights that patients started on immunomodulatory therapy for VKH should be kept on close follow-up to look for infections and TB especially in the Indian scenario.

Conclusion

This case shows us the perplexity of diagnosis between two granulomatous conditions – VKH and TB, with similar presentations but separate pathophysiology and treatments. This case also highlights the importance of close monitoring of patients for infections like TB while on immunosuppression.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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