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 Table of Contents  
Year : 2022  |  Volume : 2  |  Issue : 4  |  Page : 906-908

Adalimumab in Vogt–Koyanagi–Harada disease: A case report

1 Medical Department of Nanchang University, Nanchang, 330000, China
2 Department of Ophthalmology, The Second Hospital Affiliated to Nanchang University, Nanchang, 330000, China

Date of Submission16-Nov-2021
Date of Acceptance31-Jul-2022
Date of Web Publication11-Oct-2022

Correspondence Address:
Dr. Guodong Li
No. 1, Mingde Road, Donghu District, Nanchang City, Jiangxi Province, 330000
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_2902_21

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A 30-year-old Chinese woman presented to the ophthalmology department with bilateral blurry vision.; she was diagnosed with incomplete Vogt–Koyanagi–Harada (VKH). The treatment consisted of prednisolone infusion followed by a change of oral hormones. However, adalimumab subcutaneous injection was used instead because of the ineffectiveness of hormone therapy and the patient's reluctance to take oral hormones. After effective monotherapy with adalimumab, the inflammation was stable and inactive without recurrence at follow-up.

Keywords: Adalimumab, TNF-a, tumor necrosis factor-alpha inhibitors, Vogt–Koyanagi–Harada

How to cite this article:
Chen T, Li G, Mao Q, Zhang Y. Adalimumab in Vogt–Koyanagi–Harada disease: A case report. Indian J Ophthalmol Case Rep 2022;2:906-8

How to cite this URL:
Chen T, Li G, Mao Q, Zhang Y. Adalimumab in Vogt–Koyanagi–Harada disease: A case report. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Nov 30];2:906-8. Available from: https://www.ijoreports.in/text.asp?2022/2/4/906/358167

 Vogt-Koyanagi-Harada syndrome More Details is a T-lymphocyte-mediated autoimmune disease with eye manifestations of chronic granulomatous bilateral total uveitis. The ocular symptoms present as serous retinal detachment, optic neuritis, and vitreous inflammation. There is simultaneous involvement in the central nervous system, cochlear vagus, and skin system. The prevalence of VKH varies by region and ethnicity, often being found in Asia, the Middle East, Spain, and America. The main clinical therapies for VKH are glucocorticoids and immunosuppressants. However, these traditional methods have numerous side effects including systemic toxicity. Over the years, tumor necrosis factor-alpha (TNF-α) inhibitors have become a widespread treatment for non-infectious uveitis (NIU), in this paper, we will describe the successful treatment of Vogt-Koyanagi-Harada syndrome.

  Case Report Top

A 30-year-old woman visited our hospital because of bilateral blurry vision following ophthalmodynia. She showed the cephalic symptom of the common cold and had been vaccinated against HPV several years ago. She denied a history of eye surgery or penetrating ocular trauma. Her best-corrected visual acuity (BCVA) in two eyes was 20/200. Slit-lamp biomicroscopy revealed 4+ anterior chamber cells and vitreous inflammation. Posterior pupillary adhesions were also detected [Figure 1]a and [Figure 1]b. Fundus photographs depicted mild optic disc and retinal edema [Figure 1]c and [Figure 1]d. Optical coherence tomography showed increased choroidal thickness and multiple PED [Figure 1]e and [Figure 1]f. Fluorescein angiography revealed bilateral multiple early pinpoint leakages and late pooling. Intraocular pressure was 14 mmHg right eye and 13 mmHg left eye. According to basic medical history information and consultation, the patient denied that she had suspicious symptoms of tuberculosis: cough and sputum for ≥2 weeks, or sputum blood, hemoptysis, hypothermia, and night sweats. There was no exposure history of tuberculosis (TB) contact and her chest X-ray finding was normal. The patient was tested for sputum pathogenic laboratory tests for a precise diagnosis, which showed no abnormalities. The patient was eventually excluded from the diagnosis of tuberculosis. No abnormalities were observed during the laboratory tests, HLA-B27, HIV, and PPD testing were both negative. Finally, she was diagnosed with incomplete VKH disease. Topical atropine and prednisolone acetate eye drops were used to control inflammation. Meanwhile, we began with intravenous methylprednisolone at a dosage of 1 g/day for an immediate effect, oral hormone was started after 3 days. The initial dose was 40 mg, which was calculated based on her bodyweight. During the next 14 weeks, the oral dose was decreased by 5 mg/day every 2 weeks. The patient's subjective symptoms improved, and anterior chamber inflammation had disappeared. At this point, we stopped using eye drops. The hormone tapering schedule for steroid administration was based on the guidelines of an international expert panel.[1] However, when we exerted a decline to less than 5 mg/day, there was a recurrence of inflammation. The patient reported tinnitus and routine pure tone audiometric threshold tests performed by the otolaryngology department. The patient's hearing abnormality occurred at a high frequency of 4000 Hz, with a pure tone audiometric value of 23 dB at 4000 Hz on the right side and 27 dB at 4000 Hz on the left side. The patient developed a gastric ulcer after taking glucocorticosteroids and was advised by the gastroenterologist to temporarily stop taking the oral medication. A large proportion of immunosuppressants have gastrointestinal side effects. At the same time, she suffered side effects such as obesity and increased blood sugar, which directly caused her reluctance to take oral hormones. For safety reasons, adalimumab injection was chosen as a convenient way to avoid gastrointestinal administration. After the exclusion of the hepatitis examination, adalimumab subcutaneous injection was initiated. The initial dose was 80 mg, another subcutaneous injection was 40 mg given a week later. All subsequent biologics therapy was subcutaneously injected at 40 mg every 2 weeks. As adalimumab is fully humanized, it is less likely to produce autoantibodies than other tumor necrosis factor inhibitors. Intraocular adalimumab antibodies cannot be analyzed at this time due to limited laboratory conditions at our hospital. After 28 months of follow-up from injection of adalimumab, there was no evidence of active uveitis [Figure 2]a and [Figure 2]b, the fundus photography showed the subsiding of retinal exudation [Figure 2]c and [Figure 2]d, and repeat OCT imaging demonstrated PED reset [Figure 2]e and [Figure 2]f. Hyperfluorescent pinpoint leakage was improved on early phase FA, suggesting mitigation of choroidal inflammation. And it didn't witness adverse cases related to adalimumab.
Figure 1: Before a dalimumab therapy, slit-lamp biomicroscopic examination revealed anterior chamber cell and posterior pupillary adhesions (a and b). Color retinal photographs showed serous retinal detachments and hyperemic optic discs with choroidal folds disc (c and d). Optical coherence tomography scanned exudative detachment of the neurosensory retina (e and f). FA photographs depicted bilateral multiple early pinpoint leakages and late pooling

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Figure 2: Ophthalmic imaging changes at 32-month follow up; The inflammation in the anterior chamber had subsided (a and b). The optic disc edema had resolved (c and d) and there was a significant improvement of PED and hyperfluorescent pinpoint leakage. Subretinal fluid in macular area of both eyes almost completely absorbed (e and f)

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  Discussion Top

Vogt–Koyanagi–Harada syndrome (VKHS) is the most common anatomical type in the etiology of specific types of noninfectious panuveitis. Currently, VKHS pathogenesis is considered to be an immunological, genetic, and infectious hypothesis.[2] Among the inflammatory factors, TNF-α is involved in the VKHS inflammatory response through direct action and mediation of other pro-inflammatory cytokines, which are originally from Th1 and Th17 cells. TNF-α connects with tumor necrosis factor receptor-1 (TNFR-1) to participate in inflammatory pathways. The TNF-α inhibitor is a specific recombinant fully human immunoglobulin IgG1 monoclonal antibody with a structure and function similar to that of human IgG1.[3] As a multisystem disorder, unequivocal diagnosis requires evidence of the involvement of more than the ocular system. There are diagnostic criteria revised by an international committee on nomenclature.[4],[5]

  1. No history of penetrating ocular trauma or surgery
  2. Exclusion of other ocular disease entities
  3. Bilateral ocular involvement
  4. Neurological/auditory findings (meningismus, tinnitus, cerebrospinal fluid pleocytosis)
  5. Integumentary finding (alopecia, poliosis, vitiligo)

Classification: Complete VKHS (criteria 1 to 5 must be present); incomplete VKHS (criteria 1 to 3 and either 4 or 5 must be present); probable VKHS (isolated ocular disease; criteria 1 to 3 must be present)

The reason for being diagnosed with incomplete VKHS is the typical clinical ocular manifestation and the concomitant systemic symptom. During the acute phase, the most obvious evidence was hyperfluorescent spots and late leakage on FA. Along with disc hyperfluorescence, we also witnessed exudative retinal detachment and choroidal thickening. The diagnosis is also supported by the patient's history of non-ocular symptoms such as previous headaches and hearing loss. The acute phase is often treated with high-dose steroid corticosteroid shock therapy to control inflammation. Additionally, it can also be used in combination with immunosuppressive drugs. We have presented a treatment about adalimumab. Díaz Llopis M. described a patient with VKHS who successfully suppressed inflammation using adalimumab.[6] Kei Takayama treated another elderly patient with chronic recurrent VKHS who was not relieved by oral corticosteroids and cyclosporine. Central serous chorioretinopathy (CSC) was induced by corticosteroids and the inflammation was effectively stabilized by adalimumab.[7] Similarly, Emily Su used adalimumab (ADA) to improve ocular symptoms of a 12-year-old female patient when corticosteroids and mycophenolate were ineffective.[8] Besides these case reports, Couto Cristóbal systematically followed 14 young patients with VKHS. All patients treated with adalimumab had remission of inflammation after 6 months of close follow-up, and relevant data suggested that adalimumab treatment reduces the need for oral corticosteroids and conventional immunosuppressive therapy in patients with VKHS.[9] A 78-week extension study of ADA safety and efficacy in 371 adult patients who completed VISUAL- I and II showed that ADA increased mean BCVA in patients with active Non-infectious uveitis (NIU) while enabling 66% of inactive NIU hormone dose reductions, indicating that ADA is effective for NIU.[10] Burmester et al. studied the safety data from 77 ADA clinical trials, which covered multiple indications. It was the largest safety assessment on ADA to date, enrolling 29,967 patients. Emergency adverse events occurred in 12.9% of patients (8. 7/100 PY) and serious infections (3.7/100 PY) were the most common serious adverse events, with the most common serious infections being pneumonia (0.6/100 PY) and cellulitis (0.2/100 PY), in addition to an overall incidence of severe demyelinating disease, lupus-like syndrome, and nodular disease of less than 0.1/100 PY.[11] Adalimumab has had remarkable effects in the clinical application of NIU and VKHS, including the advantages of inhibiting inflammation and reducing recurrence rate. Recent drug formulations are well tolerated and convenient. It can significantly alleviate ocular symptoms and improve visual prognosis. It will provide additional options for VKHS patients who have limited success with steroid therapy or are not qualified candidates for hormones. However, there are still problems such as causing adverse events and ineffective treatment. It is believed that adalimumab will provide new options for VKHS patients as the efficacy and safety of clinical, large-sample, controlled trials for VKHS alone are further established.

  Conclusion Top

Biological agent such as adalimumab is an effective therapy for Vogt–Koyanagi–Harada disease, patients treated with adalimumab appear to demonstrate good visual outcomes when the traditional Corticosteroids are ineffective.

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.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Jabs DA, Rosenbaum JT, Foster CS, Holland GN, Jaffe GJ, Louie JS, et al. Guidelines for the use of immunosuppressive drugs in patients with ocular inflammatory disorders: Recommendations of an expert panel. Am J Ophthalmol 2000;130:492-513.  Back to cited text no. 1
Greco A, Fusconi M, Gallo A, Turchetta R, Marinelli C, Macri GF, et al. Vogt-Koyanagi-Harada syndrome. Autoimmun Rev 2013;12:1033-8.  Back to cited text no. 2
Tomita N, Morishita R, Tomita S, Kaneda Y, Higaki J, Ogihara T, et al. Inhibition of TNF-alpha, induced cytokine and adhesion molecule. Expression in glomerular cells in vitro and in vivo by transcription factor decoy for NFkappaB. Exp Nephrol 2001;9:181-90.  Back to cited text no. 3
Read R, Holland GN, Rao NA, Tabbara KF, Ohno S, Arellanes-Garcia L, et al. Revised diagnostic criteria for Vogt-Koyanagi-Harada disease: Report of an international committee on nomenclature. Am J Ophthalmol 2001;131:647-52.  Back to cited text no. 4
Yang P, Zhong Y, Du L, Chi W, Chen L, Zhang R, et al. Development and evaluation of diagnostic criteria for Vogt-Koyanagi-Harada disease. JAMA Ophthalmol 2018 136:1025-31.  Back to cited text no. 5
Díaz Llopis M, Amselem L, Romero FJ, García-Delpech S, Hernández ML. [Adalimumab therapy for Vogt-Koyanagi-Harada syndrome]. Arch Soc Esp Oftalmol 2007;82:131-2.  Back to cited text no. 6
Takayama K, Obata H, Takeuchi M. Efficacy of adalimumab for chronic Vogt-Koyanagi-Harada disease refractory to conventional corticosteroids and immunosuppressive therapy and complicated by central serous chorioretinopathy. Ocul Immunol Inflamm 2020;28:509-12.  Back to cited text no. 7
Su E, Oza VS, Latkany P. A case of recalcitrant pediatric Vogt-Koyanagi-Harada disease successfully controlled with adalimumab. J Formos Med Assoc 2019;118:945-50.  Back to cited text no. 8
Couto C, Schlaen A, Frick M, Khoury M, Lopez M, Hurtado E, et al. Adalimumab treatment in patients with Vogt-Koyanagi-Harada disease. Ocul Immunol Inflamm 2018;26:485-9.  Back to cited text no. 9
Suhler E, Adán A, Brézin AP, Fortin E, Goto H, Jaffe GJ, et al. Safety and efficacy of adalimumab in patients with noninfectious uveitis in an ongoing open-label study: VISUAL III. Ophthalmology 2018;125:1075-87.  Back to cited text no. 10
Burmester GR, Gordon KB, Rosenbaum JT, Arikan D, Lau WL, Li P, et al. Long-term safety of adalimumab in 29,967 adult patients from global clinical trials across multiple indications: An updated analysis. Adv Ther 2020;37:364-80.  Back to cited text no. 11


  [Figure 1], [Figure 2]


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