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Year : 1978  |  Volume : 26  |  Issue : 3  |  Page : 8-11

Vogt Koyanagi Harda syndrome

Department of Ophthalmology, Postgraduate Institute of Medical Education & Research, Chandigarh, India

Correspondence Address:
I S Jain
Department of Ophthalmology, Postgraduate Institute of Medical Education & Research, Chandigarh-160012
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Source of Support: None, Conflict of Interest: None

PMID: 738781

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How to cite this article:
Jain I S, Kaul R L, Gangwar D N, Dhir S P, Jain G C. Vogt Koyanagi Harda syndrome. Indian J Ophthalmol 1978;26:8-11

How to cite this URL:
Jain I S, Kaul R L, Gangwar D N, Dhir S P, Jain G C. Vogt Koyanagi Harda syndrome. Indian J Ophthalmol [serial online] 1978 [cited 2021 Feb 25];26:8-11. Available from: https://www.ijo.in/text.asp?1978/26/3/8/31187

Table 1

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Table 1

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Vogt Koyanagi syndrome is characterised by a severe anterior and posterior uveitis associated with poliosis, vitiligo, alopecia and dysacousia. Meningeal symptoms occur occa­sionally. Haradas disease is characterised by an acute bilateral posterior uveitis associated with symptoms of meningeal irritation in a previously healthy young adult. If the disease becomes chronic the anterior uveitis may assume a greater role and warrant a diagnosis of Vogt Koyanagi Syndrome.

The combination of these two entities has been accepted as more papers have reported cases of V.K. syndrome with manifestation of Harada's disease and vice versa.[2],[4],[5],[6],[14].

The disease affects principally the more pigmented races and occurs more frequently in Japan and was found in 6.8% of all cases of uveitis.[16]

From our country there have been isolated reports of this disease and was first described in 1963[9] and later more cases have been added.[7],[10]

Present report is based on 10 cases of VKH syndrome out of 250 cases of uveitis seen in last four years in Uveitis Clinic of the Nehru Hospital attached to Postgraduate Institute of Medical Education and Research, Chandigarh. This report reflects that incidence of this disease is somewhat more in Northern parts of our country.

  Observation Top

The median age of patients at the onset of ocular symptoms was 31 years (range of 16-49 years). There was more predilection for males (7 men and 3 women). The initial complaint in all cases was gross diminuha of vision. All but three cases had history of headache, fever and vomiting, two cases had marked tinitus.

Sensory hearing loss was present in four of ten cases. Skin or hair changes or both were present in six of ten cases. One patient had Hepatosplenomegaly.

One case had history of trauma. Two had surgery one of cataract extraction, and another of detachment prior to disease process and one had inter current surgery (iridectomy). Vision was markedly reduced in all cases and overall prognosis of vision was poor with only four cases showing some improvement.

Anterior uveitis was present in all cases, each one of ten cases had aqueous flare, eight cases showed keratic precipitates and two had pigment dispersion on back of cornea. Two of the cases showed raised tension and another two had low tension. Signs of inflammation of vitreous and exudative retinal detachment were present in eight cases, one showed resolving vitreous haemorrhage and glial proliferation and in another fundus could not be visualised.

CSF examination results were available in six cases out of these one case showed leutic type of colloidal gold curve with non reactive CSF VDRL this being suggestive of association with multiple scleresis. Positive Toxoplasmosis titre (1:512) in one case and reversed albumin globulin ratio in four cases were the other laboratory abnormalities present in our series.

In one case HLA antigen typing was done. He had a negative HLA B 27 antigen and HLA 28, 11 and 15 were positive.

  Discussion Top

The arbitrary division of VKS disease into Vogt Koyanagi and Haradas disease is not practicable in clinical practice as these may eventually present with a complete picture of VKH disease and this is supported by more and more case reports of such nature. Also this division does not help in resolution of their probable common etiology.

The disease is characterised by a bilateral exudative iridocyclitis of sudden onset associated with opacification of vitreous and an exudative choroditis leading to secondary retinal detach­ment. Anterior or posterior inflammatory signs may predominate and consequently may be labelled V.K. syndrome or Haradas disease. Although initially there is tendency to hypotony, posterior synechia associated with seclusive

pupillae may lead to glaucoma.[4],[6],[14] sub In our series all ten cases had anterior uveitis two cases had hypotony and another two had raised tension.

The disease occurs in third decade predo­minantly in orientals[6],[13]. Alopacia and poliosis which occur in 90% of cases may occur within three weeks to six years from the onset of disease and may resolve from months to years later. Vitiligo and sensory hearing loss occur in half of cases and may accompany initial decrease in vision. Meningeal irritation occurs occasionally[1],[4],[6],[11],(17).

In our series also skin or hair changes or both were present in six and sensory hearing loss in four of the cases. All but two of our cases had history of headache, fever and vomit­ing and two had marked tinitus.

Bilateral retinal detachment starts in central region. They are initially small, bullous and located inferiorily and quickly become total. Edema of disc is common in this disease. In present series signs of vitreous inflammation and exudative retinal detachment are manifested in eight of ten cases. One had a resolving vitreous haemorrhage and in one case fundus could not be visualised.

Sympathetic ophthalmia which at times presents with all the signs of VKH syndrome may also be etiologically linked to this entity[3]. We had three such cases, in one there was history of trauma and another two had under­gone surgery, Cataract extraction in one and retinal detachment surgery in another.

VKH syndrome may also be associated with diseases as multiple sclerosis[12] and Toxopla­smosis.[15] We also had one case having associa­tion with multiple sclerosis and another with toxoplasmosis. Investigations may also reveal presence of globulin in CSF and reversal of globulin ratio as was seen in our series.

  Summary Top

The overlapping of clinical manifestations between the Vogt Koyanagi and Harada's disease justifies considering them as part of a single disease entity (Vogt Koyanagi Harada's syndrome). Ten cases of VKH are presented. Mostly loss of vision was preceded by fever, headache and vomiting. All cases showed signs of anterior uveitis. Most of the cases showed edema of disc and exudative retinal detach­ment. Some cases also showed skin and hair changes and sensory hearing loss. Similarity of etiopathogensis between sympathetic ophth­almia and VKH is suggestive by three cases.

Association with multiple sclerosis and toxoplasmosis is suggested by CSF and blood investigations. Among other laboratory inves­figations presence of globulin in CSF and reversal of albumin globulin ratio were present. Pro­gnosis after treatment is not satisfactory.

  References Top

Bennet, F.K., 1962, Brit. J. opththal., 46, 563.  Back to cited text no. 1
Bruno, M.G. and Mcpherson, S.D., 1949, Amer. Jour. ophthal., 32, 513.  Back to cited text no. 2
Cordes, F.C., 1955, Amer. Jain, Ophthal., 39, 499.  Back to cited text no. 3
Cowper. A.R., 1958), Arch. Oph., 45, 367.  Back to cited text no. 4
Davies, W.S., 1935), Arch. Oph., 14, 239.  Back to cited text no. 5
Duke Elder, S and Perkins, E.S., 1966, Diseases of uveal tract. In Duke Elder. S (ed) System of Ophthalmology. 9, London Henry Kimpton, 373.  Back to cited text no. 6
Jain, I.S., 1964, Orient Arch. Ophth., 2/1, 36.  Back to cited text no. 7
Jain, I.S., Gangwar, D.N.; Kaul, R.L. and Jain, G.D., Sympathetic ophthalmia simulating VKH after retinal detachment surgery (In press).  Back to cited text no. 8
Mitra, R.R., Dhanda, R.P. 1963, Jour. All India Oph. Soc., 11, 68  Back to cited text no. 9
Nirankari, M.S., Khanna, K.K., Chawla, G.K. and Mathur, R.P.D. 1970: Jour. All Ind. Oph., 24, 439.  Back to cited text no. 10
Pattison, W.R., 1940, Arch. Ophth. 24, 439.  Back to cited text no. 11
Pattison, E.M., 1965, Arch. Neurel. 12, 197.  Back to cited text no. 12
Reed, W, Lindsay, A, Spealemen, J. Manektoni, G.; and Can. Med. Assoc. J. 79, 451.  Back to cited text no. 13
Rosen, E, 1945, Arch. Oph., 33, 2k 1.  Back to cited text no. 14
Sasaki, K; Kamiysma, T. Kikuchi, T. et al, 1971, Jap. J. Clinical Ophthal. 77/3, 281.  Back to cited text no. 15
Woods, A.C., 1958, Endogenous Uveitis Balti­more, Williams and Williams, 967.  Back to cited text no. 16


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