Multimodal Analysis and Electroretinogram in VKH From Acute Onset - Part I

NCT03811366 | Completed Results

• 1 other identifier: Brazilian VKH Study Group I
• Study Type: interventional
• Target: 12
• Locations: 1 country
• Sites: 1

Brief Summary

Patients with acute onset Vogt-Koyanagi-Harada disease (VKHD) were prospectively included in this study. They were systematically followed with clinical, posterior segment imaging exams and full-field electroretinogram during a minimum 24-month of follow-up. All patients were treated with 3-day methylprednisolone pulse therapy followed by 1mg/day oral prednisone with a slow tapper during a median of 13 months. Non-steroidal immunosuppressive therapy (IMT) was introduced in cases of refractory disease or in cases of prednisone intolerance. Outcome measured by full-field electroretinogram was analyzed and patient was grouped as electroretinogram stable or electroretinogram worsening. Clinical data was analyzed in these two electroretinogram-based groups.

Conditions

Uveomeningoencephalitic Syndrome; Inflammation; Choroid Disease; Visual Impairment

Outcome Measures

Primary Outcomes (3)

• Median Values of ERG Scotopic Parameters at 12-month

  Full-field electroretinogram (ERG) scotopic parameters were evaluated at 12 -month (scotopic parameters: amplitude of scotopic a and b wave, amplitude of maximum scotopic a and b wave, oscillatory potential).

  assessed at 12-month

• Median Values of ERG Scotopic Parameters at 24-month

  Full-field electroretinogram (ERG) scotopic parameters were evaluated at 24 -month (scotopic parameters: amplitude of scotopic a and b wave, amplitude of maximum scotopic a and b wave, oscillatory potential).

  assessed at 24-month

• Variation (Worsening or Improvement) Between 24 and 12 Months of ERG Scotopic Parameters Comparing 24 and 12-months

  Full-field electroretinogram (ERG) scotopic parameters at 12 and 24 months were compared (scotopic parameters: amplitude of scotopic a and b wave, amplitude of maximum scotopic a and b wave, oscillatory potential). The worsening of the ERG parameters was defined as a value reduction of ≥ 30 % in any these scotopic ERG parameters at month 12 and month 24. We report the change between the (value at month 24/ the value at month 12)\*100.

  12 and 24-months

Secondary Outcomes (7)

• Recurrence or Worsening of Cells in Anterior Chamber

  6 to 24 months from disease onset.

• Increase in the Score of Dark Dots on Indocyanine Green Angiography

  6 to 24 months from disease onset

• Change in Subfoveal Choroidal Thickness on Enhanced Depth Optical Coherence Tomography

  6 to 24 months from disease onset

• Change in Perivascular Leakage on Fluorescein Angiography

  6 to 24 months after disease onset

• Choroidal Neovascularization

  6 to 24 months after disease onset

Study Arms (1)

Corticosteroid monotherapy

OTHER

All patients in the acute phase will be treated with corticosteroid monotherapy, unless presents a contraindication or persistence/ recurrence of inflammation with regressive corticosteroid monotherapy.This is the unique arm of the present study. Treatment protocol includes a three-day course of intravenous methylprednisolone (1000 mg per day) followed by a high dose of oral prednisone (1.0 mg/kg/day) with a slow tapering with dose equal or less than 0.1mg/kg/day after 10 months. The prednisone dose is scheduled to be reduced in a 0.1 mg/kg-step ladder until it reaches 0.3 mg/kg (i.e. for an individual with 60 kg, dose would be 20 mg): from 1 to 0.8 mg/kg every two to three weeks; at 0.7 mg/kg for 4 weeks; at 0.6 mg/kg for two to three weeks; from 0.5 mg/kg every 4 weeks; from 0.3 to 0.15 mg/kg every 6 weeks; and 0.1 mg/kg, 0.075 mg/kg and 0.05 mg/kg for 8 weeks each.

Drug: Corticosteroid monotherapy

Interventions

Corticosteroid monotherapy DRUG

Patients will receive corticosteroid monotherapy as a pulsetherapy (1000mg/ day for 3 days) followed by oral corticosteroid.

Also known as: Prednisone arm, Corticosteroid pulsetherapy arm, Methilprednisolone arm

Corticosteroid monotherapy

Eligibility Criteria

• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)

You may qualify if:

• clinical diagnosis of Vogt-Koyanagi-Harada disease
• acute onset with no previous treatment

You may not qualify if:

• non-acute VKHD
• media opacities

Sponsors & Collaborators

• University of Sao Paulo: lead

Study Sites (1)

Hospital das Clinicas HCFMUSP, Faculdade de Medicina Universidade de Sao Paulo

São Paulo, São Paulo, 05403-000, Brazil

Location

Related Publications (15)

• Du L, Kijlstra A, Yang P. Vogt-Koyanagi-Harada disease: Novel insights into pathophysiology, diagnosis and treatment. Prog Retin Eye Res. 2016 May;52:84-111. doi: 10.1016/j.preteyeres.2016.02.002. Epub 2016 Feb 11.

  PMID: 26875727 BACKGROUND

• Lavezzo MM, Sakata VM, Morita C, Rodriguez EE, Abdallah SF, da Silva FT, Hirata CE, Yamamoto JH. Vogt-Koyanagi-Harada disease: review of a rare autoimmune disease targeting antigens of melanocytes. Orphanet J Rare Dis. 2016 Mar 24;11:29. doi: 10.1186/s13023-016-0412-4.

  PMID: 27008848 BACKGROUND

• Rao NA. Pathology of Vogt-Koyanagi-Harada disease. Int Ophthalmol. 2007 Apr-Jun;27(2-3):81-5. doi: 10.1007/s10792-006-9029-2. Epub 2007 Apr 14.

  PMID: 17435969 BACKGROUND

• Rubsamen PE, Gass JD. Vogt-Koyanagi-Harada syndrome. Clinical course, therapy, and long-term visual outcome. Arch Ophthalmol. 1991 May;109(5):682-7. doi: 10.1001/archopht.1991.01080050096037.

  PMID: 2025171 BACKGROUND

• Herbort CP Jr, Abu El Asrar AM, Takeuchi M, Pavesio CE, Couto C, Hedayatfar A, Maruyama K, Rao X, Silpa-Archa S, Somkijrungroj T. Catching the therapeutic window of opportunity in early initial-onset Vogt-Koyanagi-Harada uveitis can cure the disease. Int Ophthalmol. 2019 Jun;39(6):1419-1425. doi: 10.1007/s10792-018-0949-4. Epub 2018 Jun 11.

  PMID: 29948499 BACKGROUND

• Yang P, Fang W, Wang L, Wen F, Wu W, Kijlstra A. Study of macular function by multifocal electroretinography in patients with Vogt-Koyanagi-Harada syndrome. Am J Ophthalmol. 2008 Nov;146(5):767-71. doi: 10.1016/j.ajo.2008.05.044. Epub 2008 Jul 30.

  PMID: 18672226 BACKGROUND

• Chee SP, Jap A, Bacsal K. Spectrum of Vogt-Koyanagi-Harada disease in Singapore. Int Ophthalmol. 2007 Apr-Jun;27(2-3):137-42. doi: 10.1007/s10792-006-9009-6. Epub 2006 Nov 11.

  PMID: 17103022 BACKGROUND

• Yuan W, Zhou C, Cao Q, Du Z, Hu R, Wang Y, Kijlstra A, Yang P. Longitudinal Study of Visual Function in Vogt-Koyanagi-Harada Disease Using Full-Field Electroretinography. Am J Ophthalmol. 2018 Jul;191:92-99. doi: 10.1016/j.ajo.2018.04.013. Epub 2018 Apr 25.

  PMID: 29702075 BACKGROUND

• Chee SP, Luu CD, Cheng CL, Lim WK, Jap A. Visual function in Vogt-Koyanagi-Harada patients. Graefes Arch Clin Exp Ophthalmol. 2005 Aug;243(8):785-90. doi: 10.1007/s00417-005-1156-3. Epub 2005 Mar 11.

  PMID: 15761760 BACKGROUND

• da Silva FT, Hirata CE, Olivalves E, Oyamada MK, Yamamoto JH. Fundus-based and electroretinographic strategies for stratification of late-stage Vogt-Koyanagi-Harada disease patients. Am J Ophthalmol. 2009 Dec;148(6):939-45.e3. doi: 10.1016/j.ajo.2009.06.029. Epub 2009 Sep 24.

  PMID: 19781687 BACKGROUND

• Tugal-Tutkun I, Herbort CP, Khairallah M; Angiography Scoring for Uveitis Working Group (ASUWOG). Scoring of dual fluorescein and ICG inflammatory angiographic signs for the grading of posterior segment inflammation (dual fluorescein and ICG angiographic scoring system for uveitis). Int Ophthalmol. 2010 Oct;30(5):539-52. doi: 10.1007/s10792-008-9263-x. Epub 2008 Sep 16.

  PMID: 18795232 BACKGROUND

• Nakayama M, Keino H, Watanabe T, Okada AA. Clinical features and visual outcomes of 111 patients with new-onset acute Vogt-Koyanagi-Harada disease treated with pulse intravenous corticosteroids. Br J Ophthalmol. 2019 Feb;103(2):274-278. doi: 10.1136/bjophthalmol-2017-311691. Epub 2018 Apr 17.

  PMID: 29666121 BACKGROUND

• Kawaguchi T, Horie S, Bouchenaki N, Ohno-Matsui K, Mochizuki M, Herbort CP. Suboptimal therapy controls clinically apparent disease but not subclinical progression of Vogt-Koyanagi-Harada disease. Int Ophthalmol. 2010 Feb;30(1):41-50. doi: 10.1007/s10792-008-9288-1. Epub 2009 Jan 17.

  PMID: 19151926 BACKGROUND

• Herbort CP Jr, Abu El Asrar AM, Yamamoto JH, Pavesio CE, Gupta V, Khairallah M, Tugal-Tutkun I, Soheilian M, Takeuchi M, Papadia M. Reappraisal of the management of Vogt-Koyanagi-Harada disease: sunset glow fundus is no more a fatality. Int Ophthalmol. 2017 Dec;37(6):1383-1395. doi: 10.1007/s10792-016-0395-0. Epub 2016 Nov 14.

  PMID: 27844182 BACKGROUND

• Abu El-Asrar AM, Dosari M, Hemachandran S, Gikandi PW, Al-Muammar A. Mycophenolate mofetil combined with systemic corticosteroids prevents progression to chronic recurrent inflammation and development of 'sunset glow fundus' in initial-onset acute uveitis associated with Vogt-Koyanagi-Harada disease. Acta Ophthalmol. 2017 Feb;95(1):85-90. doi: 10.1111/aos.13189. Epub 2016 Aug 18.

  PMID: 27535102 BACKGROUND

MeSH Terms

Conditions

Uveomeningoencephalitic Syndrome; Inflammation; Choroid Diseases; Vision Disorders

Condition Hierarchy (Ancestors)

Autoimmune Diseases of the Nervous System; Nervous System Diseases; Uveitis; Uveal Diseases; Eye Diseases; Autoimmune Diseases; Immune System Diseases; Pathologic Processes; Pathological Conditions, Signs and Symptoms; Sensation Disorders; Neurologic Manifestations; Signs and Symptoms

Results Point of Contact

• Title: Dra Joyce Hisae Yamamoto
• Organization: Hospital das clínicas- Faculdade de Medicina da Universidade São Paulo

vmsakata@yahoo.com.br

41988263757

Study Officials

• Joyce H Yamamoto, PhD

  Universidade São Paulo

  PRINCIPAL INVESTIGATOR

Publication Agreements

• PI is Sponsor Employee: No
• Restrictive Agreement: No

Study Design

• Study Type: interventional
• Phase: not applicable
• Allocation: NA
• Masking: NONE
• Purpose: OTHER
• Intervention Model: SINGLE GROUP
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Clinical Professor, person-in-charge of Uveitis Service

Model Details: All patients were treated with a standard high-dose corticosteroid

Study Record Dates

• First Submitted: January 10, 2019
• First Posted: January 22, 2019
• Study Start: June 1, 2011
• Primary Completion: January 31, 2017
• Study Completion: January 31, 2017
• Last Updated: April 17, 2025
• Results First Posted: November 22, 2024

Record last verified: 2025-04

Locations

BR (1)