|Year : 1964 | Volume
| Issue : 4 | Page : 147-153
Viral uveitis- a review of 11 cases
SD Paul, OP Ahuja, BR Shukla
Muslim University Institute of Ophthalmology and The Gandhi Eye Hospital, Aligarh, India
|Date of Web Publication||13-Feb-2008|
S D Paul
Muslim University Institute of Ophthalmology and The Gandhi Eye Hospital, Aligarh
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Paul S D, Ahuja O P, Shukla B R. Viral uveitis- a review of 11 cases. Indian J Ophthalmol 1964;12:147-53
Interest in the subject of viral uveitis has been renewed since the attempts in recent years to correlate Vogt-Koyanagi disease, Harada's disease, Behcets' disease and sympathetic ophthalmia.
In a review of 140 cases of endogenous uveitis carried out in our department in the last four years, uveitis was attributed to a probable viral source in 11 cases. An analysis of the clinical aspects of these cases is undertaken here to study their resemblances and differences in correlating these diseases. Their typical clinical features stand them out distinctly from other causative factors of endogenous uveitis. No virus studies have been carried out in these cases and only clinical and certain laboratory findings have been depended upon.
In the present series were noted four cases of Vogt-Koyanagi-Harda's syndrome, three cases of Behcet's disease and four of sympathetic ophthalmia.
A detailed history and comprehensive examination was made and recorded in each case. The main laboratory investigations carried out were total and differential white blood cell count, erythrocyte sedimentation rate, V.D.R.L. and Kahn's tests, Mantaux's test and examination of cerebrospinal fluid.
The cases were put on treatment and their progress was regularly observed and recorded. The cases have beer followed up for a period of 2-4 years.
Vogt-Koya Nagi-Harada's Syndrome
Under this head there were four cases, 2 males and 2 females of ages between 12-49 years.
The disease process was clinically manifested with meningeal symptoms and poliosis in all the four cases while vitiligo was present in 3 cases, dysacousia in 2 and alopecia in one case only. In none of these cases the fundus condition could be ascertained due to hazy media.
It may be of interest to note that in one case, vitiligo and poliosis and in a second one vitiligo were present before the onset of ocular disease, whereas deafness in the other two cases developed after the onset of ocular disease and was only temporary. It disappeared after a couple of days.
Amongst the laboratory investigations a pleocytosis in cerebro-spinal fluid was seen in 3 cases while a raised erythrocyte sedimentation rate was present in 2 cases.
All the cases were given prolonged, systemic corticosteroid therapy and considerable improvement of the clinical picture and the visual function was seen in 3 out of 4 cases. The improvement was as much as from 2/60 to 6/24 in one case, whereas in the left eye of case No. 3 where there was no light perception, no improvement was possible.
Out of the 4 cases of sympathetic ophthalmia, three were males and l female, with ages varying from 11-40 years.
The period which elapsed between the dates of injury and development of the disease in the uninjured eye varied from 12 days to 2 months.
In two cases the sympathetic disease started as anterior uveitis while in the other two cases the process started as neuro-retinitis.
All the cases were treated mainly with prolonged systemic corticosteroids and locally with mydriatics and corticosteroid preparations. In 3 cases a satisfactory improvement was noted which has been maintained up to the end of the follow-up period, i.e. 2-3 years whereas in I case the eye did not improve and became atrophic.
The three cases of Behect's disease were 2 males and 1 female, the ages varying from 24-32.
Hypopyon iritis and aphthous ulcers in the mouth were seen in all the three cases while polyarthritis was seen in two cases and ulcers on genitalia in one case. In one case the onset of the ocular disease was accompanied by fever. The same case also suffered from pyodermia.
In another case, optic nerve involvement was observed. First, a marked bilateral constriction of retinal blood vessels was seen. This was followed by a complete bilateral optic atrophy.
All the cases were treated with systemic broad spectrum antibiotics, corticosteroids, salicylates and irgapyrin. No relief was seen in any of the eyes and all the eyes were ultimately lost.
| Discussion|| |
Vogt (1906) described elaborately a form of uveitis associated with poliosis. Koyanagi (1929) amplified these observations and added, in the clinical picture, the accompaniment of dysacousia, vitiligo and alopecia in addition to poliosis as described by Vogt, and the syndrome began to be known as Vogt-Koyanagi syndrome.
Harada (1926) described a condition in young adults accompanied by marked accumulation of subretinal fluid leading to retinal detachment. Pleocytosis in cerebrospinal fluid was a constant finding.
Bruno and McPherson (1939) correlated the two conditions and found many a feature which were common to both Vogt-Koyanagi syndrome and Harda's disease. This has been agreed to by a number of other workers and now the condition is generally termed as Vogt-Koyanagi-Harada's syndrome. (Woods 1961).
From time to time certain more unusual features have been added to the classical description of Vogt-Koyanagi Harada's syndrome, e.g. bilateral papilloedema-Gregory (1959) and bilateral scleritis Cowper (1951).
A probable hereditary factor is introduced by Benedict and Benedict (1951) who reported Vogt-Koyanagi Harada's syndrome in siblings, and Flyn (1952) described a case of the same syndrome, whose paternal relations exhibited the same picture.
Recent work on isolation of the virus has confirmed the viral nature of Vogt-Koyanagi Harada's disease.
Sugiera and his associates (1953) reported the actual isolation of a virus from the eyes of patients suffering from this syndrome. After four to eight blind passages, the authors isolated a specific virus strain. They further reported that the blood serum of another patient, suffering from the same disease, gave a positive neutralisation test against this virus with an index of 100.
Erbaken (1962) also has been able to isolate a virus from the spinal fluid of a patient suffering from Vogt-Koyanagi-Harada's syndrome. The type of virus, however, remained undetermined.
In all cases of Vogt-Koyanagi-Harada's syndrome the presence of meningeal symptoms was a noticeable feature.
Cowper (1951) in his review of the cases of Vogt-Koyanagi-Harada's syndrome reported the incidence of meningeal symptoms of one kind or the other in 90%, of his cases. The severity of these symptoms varied from case to case. Cordes (1955) also, reported meningeal symptoms in the initial stages of the disease in his case.
It appears, therefore, that some kind of intra-cranial affection leading to the signs of meningeal irritation forms a definite component of the disease process. The precise mechanism of this phenomomenon is obscure. However, keeping in mind the widespread manifestations of the disease, it may be presumed that the virus of Vogt-Koyanagi-Harada's syndrome probably affects the ocular and intracranial structures simultaneously causing a sort of meningitis or meningo-encephalitis.
In this context, Hague (1944) also, suggested that the disease process starts as an encephalitis and the clinical manifestations of the syndrome are the results of involvement of the hypothalamus.
Although all the cases in our series of Vogt-Koyanagi-Harada syndrome had meningeal symptoms, all the other classical systemic signs, viz.-alopecia, poliosis, vitiligo and dysacousia were not present together in every case. They need not be so. Similar varying incidence of these signs have been noted by Parker (1940) and Rosen (1945).
Further, it was observed that the ocular and extra-ocular signs of the syndrome did not develop simultaneously, either group of signs preceding the other in different cases.
The clinical diagnosis of Vogt-Koyanagi-Harada's syndrome becomes evident in a case where the ocular and extra-ocular signs are present together. On the other hand, if the extra ocular signs do not develop until late in the course of the ocular disease, it becomes difficult to make the diagnosis of Vogt-Koyanagi-Harada's syndrome in its early stages.
For an early diagnosis of such cases, where the extra-ocular signs appear late, it is suggested that a cerebro-spinal fluid examination be carried out in all cases of granulomatous uveitis where other etiologic factors have been ruled out. This statement is borne out by the observation that a pleocytosis in cerbro-spinal fluid can be found in most cases of Vogt-Koyanagi-Harada's syndrome. This was observed in 3 out of our 4 cases.
Therapeutic response to the systemic administration of corticosteroids was fairly encouraging in our cases of Vogt-Koyanagi-Harada's syndrome. A considerable clinical and functional improvement was seen in 3 out of 4 cases. Similar results of systemic corticosteroid therapy have been reported by Lamberto (1955) and Bronstein (1957).
Some patches of vitiligo of several years standing, were seen in the mother of case No. 1 of Vogt-Koyanagi-Harada's syndrome. These patches were, however, not associated with any ocular signs of the syndrome. Therefore, no conclusion could be drawn regarding the hereditary character of the disease as reported by Benedict and Benedict (1951) and Flyn (1952).
Woods (1961) describes the commencement of the sympathetic reaction in the posterior segment in some cases of sympathetic ophthalmia. He states that the process is usually present in the anterior choroid. No mention has been made about the development of neuro-retinitis.
In the present series, however, 2 out of 4 cases of sympathetic ophthalmia exhibited a significant feature of neuroretinitis as the reaction of sympathising with the injured eye. In the other two cases the sympathetic reaction conformed to the classical description of a plastic iridocyclitis.
Levatin (1958) reported the development of neuro-retinitis during the course of disease in a case of sympathetic ophthalmia.
The review of recent literature of sympathetic ophthalmia thus reflects a great paucity of reports about this rare manifestation of the disease process. The authors, in the present report, contribute two such cases where the disease process in the sympathising eye started as a neuro-retinitis.
The condition of sympathetic ophthalmia has always presented many problems regarding the determination of its etiology. Many factors notably allergy (Elschnig 1911) saprophytic infection (Redslob 1956), and others have been put forward as to the causative factor of sympathetic ophthalmia.
Recently many workers have tried to establish a correlation between autoimmune diseases and sympathetic ophthalmia. Aronson and co-workers (1963) tried to sensitise the animals to complete adjuvant and uveal tissue but all they could find was a uveitis of a shorter duration which was histologically dissimilar to that in sympathetic ophthalmia. Perkins and Wood (1964) tried to establish a correlation of synpathetic ophthalmia with auto-immune disease. They were able to demonstrate staining of fluorescein in the choroid. The over all results were however not specific.
In this context, it is interesting to note various similarities between sympathetic ophthalmia and Vogt-Koyanagi-Harada's syndrome as reported from time to time. Hutchinson (1892-1893) in a report of historical interest described an incomplete case of Vogt-Koyanagi-Harada's syndrome as "non-traumatic sympathetic ophthalmia".
Laje-Weskamp (1932) has pointed out that the sporadic association of symptoms like dysacousia, alopecia, vitiligo and poliosis in both these conditions have engaged the attention of many clinicians Soriano (1929), Takahashi (1930), whereas demonstration of uveal pigment sensitivity-Rones (1932) and histological similarity-Hager (1957), in both these conditions have been instrumental in forcing an etiological identity between the two.
This has eventually led Ikui and Associates (1958) to the view that probably sympathetic ophthalmia and Vogt-Koyanagi-Harada's syndrome are merely stages of a local manifestation of a systemic involvement. The etiologic factor probably acts specifically on the pigment-bearing structures of the body and uvea, the factor possibly being a choromato-tropic virus.
It is another important condition which has been attributed to be caused by a virus.
Behcet (1937) described it as a condition of recurrent uveitis with hypopyon and aphthous ulcers on the mucous membrane of the mouth, tongue and genitalia. Occasionally other manifestations, notably acute inflammatory poly-arthritis, various skin lesions, orchitis, phlebitis with a febrile onset have been described (Woods 1961).
Martin (1954) reported a case of Behcet's disease in which an eye was enucleated. Histological examination revealed a round-cell perivascular infiltration which he believed was the fundamental lesion in the disease.
Several workers have been able to isolate a virus from patients suffering from Behcet's disease.
Sezer (1953, 1956) reported the isolation of a filter passing agent from the ocular fluids of three patients suffering from Behcet's disease. He cultured this agent on chick embryos and produced a typical encephalitis in mice by intracerebral inoculation of this material. Later he has been able to isolate the virus from the blood of 20 additional cases and from the urine of some of them. Similarly Evans and associates (1957) have isolated a filter passing agent from the aqueous and Nakagowa and Shingu (1959) from vitreous and aphthous ulcers in the mouth and genitalia of patients suffering from Behcet's disease.
In this disease the extra-ocular signs, are again different and distinctive. As regards the intraocular signs, the uveitis is usually so severe that hypopyon is a regular feature.
In our Behcet series the optic nerve involvement in one case perhaps lends support to the suggestion of Sezer (1956) and Zeavin et al (1956) that the disease probably begins in the optic nerve, spreads to the retina and secondarily involves the uveal tract.
An experimental evaluation in animals has recently been carried out by Mortada and Imam (1964). The authors have conclusively proved the viral etiology of Behcet's disease by isolating the virus from hypopyon and also from ulcers of the membrane. They have shown that the virus primarily involves the neuro-epithelium of the iris, the ciliary body and the retina, causing secondary vasculitis of the uvea and the retina.
As regards therapeusis the response to cortico-steroids is discouraging, in contrast to that in Vogt-Koyanagi-Harada and sympathetic ophthalmia. In this series all the eyes were ultimately lost in spite of rigorous treatment.
Comparing the clinical manifestations of all the three, [Table - 1] they all had ocular and extra-ocular manifestations. True it is that in sympathetic ophthalmia no extra-ocular manifestations have been recorded. In this condition the ocular manifestation always comes first in contrast with the other two conditions, where extra-ocular manifestations may precede the ocular ones. In our reported series, favourable response to cortico-steroids commented upon, did not allow the later extra-ocular manifestations to develop in sympathetic ophhalmia. However, one can always recall to memory cases of sympathetic ophthalmia with deafness and vitiligo often found in precortison days. (See Table on p. 148.)
Thus, as regards extraocular manifestations, Vogt-Koyanagi-Harada and sympathetic ophthalmia are intimately related and probably may be having an identical viral etiology (Chromotropic virus) whereas it may be quite different in the case of Rehcet's, where exposed mucousmembranes are involved and no disturbance of the pigment-bearing structures have been reported.
Comparing the ocular manifestations, again one is impressed by the similarity of the granulomatous type of iritis in Volt-Koyanagi-Harada and sympathetic ophthalmic whereas that of Behcet's is quite different - hypopyon iritis, where there is a strong cyclitic element.
Comparison of the fundus condition offers a faint degree of similarity, a neuro-retinitis being found even in Behcet's disease. though only in 1 case.
As examination of the cerebro-spinal fluid was done only in the case of Vogt-Koyanagi-Harada, and so does not offer itself for comparison.
Thus, the clinical and virological studies offer support to the similarity of the nature of the lesion in sympathetic ophthalmia and Vogt-Koyanagi-Harada, but does not offer the same degree of support to Behect's as a related condition, although the latter can also be classed among VIRAL UVEITIS.
| Summary|| |
Out of 140 cases of endogenous uveitis, eleven cases with a probable viral etiology are described and compared. Of these 11, 4 were cases of Vogt-Koyanagi syndrome, 4 of sympathetic ophthalmia and 3 of Behcet's disease.
Clinical comparison and study of literature on the subjects suggest a close relationship between Vogt-Koyanagi-Harada disease and sympathetic ophthalmia but not so with Behcet's, although all the three can be grouped under viral uveitis.
Of special interest are two cases of sympathetic ophthalmia, which are recorded, in which the sympathising reaction began as an optic neuritis in the sympathising eye.
| References|| |
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Behcet. H. (1937): Derm, Wschr., 105: 1152. As quoted in 36 p. 263.
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Hague, E. B. (1944): Arch. Ophthal. 31:520.
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(1956): Amer. J. Ophth. 41: 41.
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Sugeirra, S., Fukunda, M., and Eda, K. (1953): Acta. Soc. Ophthal. Jap. 57: 117. As quoted in 36 p. 268.
Takahashi, M. (1930) Acta Soc. Ophthal. Jap. 34: 506 as quoted in 36. p. 268.
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Zeavin, B. H.. King, M. J. and Gohd. R. S. (1956) Amer. J. Ophthal. 41: 55.
[Table - 1]