Case Reports

Incomplete Reiter’s syndrome with focal involvement of the posterior segment

Robert M Conway, MB BS* Stuart L Graham, MB BS, FFL4CO-t Marissa Lassere, MB BS, FRACPt

Abstract

Purpose: To describe an unusual variant of Yersinia-induced, HLA-B27 associated incomplete Reiter’s syndrome with focal involvement of the posterior segment.

Methods: Review of case records of a patient presenting with incomplete Reiter’s syndrome which included a reactive arthritis with keratocon- junctivitis and anterior uveitis.

Results: The uveitis progressed to involve the posterior segment with a vitritis and two transient white retinal spots. After resolving, a retinal pigment epithelial (RPE) defect persisted at the site of one of the lesions.

Conclusions: While involvement of the anterior segment of the globe in Reiter’s disease is well recognised, a review of the literature reveals that focal posterior involvement is a rare feature in either Reiter’s syndrome or the reactive arthritis group.

Key words: HLA-B27, Reiter’s syndrome, retina, uveitis, Yersinia enterocolitica.

A 3 1 -year-old Indonesian man presented with a one-week history of bilateral red watery eyes. Four months earlier, he had developed swelling of the right fifth toe, with no known trauma, and the swelling was still present. He had experienced a diarrhoea1 illness two weeks before the onset of ocular symptoms, which had resolved at the time of presentation.

On initial examination he had a marked bilat- eral conjunctivitis and keratitis with extensive corneal subepithelial infiltrates. Best corrected visual acuity was 619 in the right eye and 6112 in the left. There was a mild left anterior uveitis with one plus flare and cells. Intraocular pres- sures and dilated fundal examination of both eyes were unremarkable.

A provisional diagnosis of adenoviral kerato- conjunctivitis was made and he was prescribed prednisolone 0.5% drops in both eyes and homa- tropine 5% drops three times daily in the left eye. Six days later, the vision in the left eye had dropped to 6/18. Dilated fundal examination showed a vitritis with two plus cells and vitreous haze. A conspicuous white retinal dot had now appeared inferior to the superonasal arcade. The right fundus remained normal.

A systemic work-up for uveitis began. A rheumatology consult confirmed a characteristic dactylitis [sausage digit] of the fifth toe and a

*University Department of Clinical Ophthalmology, Sydney Eye Hospital, Woolloomooloo, NS W. tDepartment of Ophthalmology, Prince of Wales Hospital, Randwick, NS W. $Department of Kheumatology, St George Hospital, Kogarah, 1z’S W. Reprints: Dr R M Conway.

Incomplete Reiter’s syndrome with focal involvement of the posterior segment 63

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Fig. 3 Photograph of the left fundus (visual acuity 6/6) Fig. photograph showing fundus with (visual taken several months after resolution of acute ocular-signs. acuity 6,18) a dense "initis. Two white retinal spots at A small patch of persistent W E atrophy is evident closely

stages of evolution are indicated, The superior corresponding to the site of the initial retinal lesion. arrow points-to the initial retinal lesion, now fading. The

inferior arrow indicates the second retinal lesion.

working diagnosis of Reiter's syndrome was advanced. The focal retinal involvement was of concern, however, since this had not previously been described as a feature of this condition.

He received 50 mg prednisone daily by mouth and topical therapy was continued. Three days later the patient felt subjective improve- ment. However, a second retinal lesion, larger than the first, had appeared while the original was fading (Figure 1).

Fig. 2 Fluorescein angiogram taken after the vitritis had subsided showing an W E window defect nasal to the optic disc corresponding to the site of the initial retinal lesion.

After one week of systemic steroid therapy the anterior uveitis was much improved and the second retinal lesion was also resolving. He continued both systemic and topical treatment, tapering over a two-month period. The vitritis cleared slowly and the vision improved to 619. A fluorescein angiogram (Figure 2) was done at this time and showed no subclinical retinal or vascular disease. A small window defect was the only abnormality found corresponding to the site of the initial lesion.

Laboratory tests revealed a mild neutrophil leucocytosis (WCC 16.6 x 10q/L) and serology for Yersinia enterocolitica group C was strongly positive at >2560 (convalescent sera six months later 1:80). Chest and sacroiliac x-rays were clear and films of the affected digit showed no abnor- mality. Bacterial and viral cultures of conjunc- tival and urethral swabs were both negative. Results of tests for connective tissue and granu- lomatuous disease revealed normal findings. Other results, including serology for HIV, syphilis, Lyme disease and toxoplasmosis were negative. HLA phenotyping was positive for the B27 antigen. A diagnosis of Yersinia-induced Reiter's syndrome or HLA-B27 associated uveitis with reactive arthritis was therefore tenable.

Some months later, he returned with dactylitis of his right fifth finger. There were no acute ocular signs and the left visual acuity was 6/6. The left fundus showed a small patch of

64 Australian and New Zealand Journal of Ophthalmology 1995; 23(1)

retinal pigment epithelial (WE) atrophy (Figure 3) which closely corresponded with the site of his initial retinal lesion and the W E defect on angiography. There were no other new lesions. He was given a course of non-steroidal medica- tion and the dactylitis resolved.

Discussion Keiter’s syndrome is characterised by the classic triad of non-bacterial urethritis, inflammatory involvement of the anterior segment of the globe and arthritis. In addition, a high proportion of these patients will be positive for the genetic marker HLA-B27.’-’ The presentation, however, is frequently varied and an incomplete form of the disease, such as HLA-B27 associated anterior uveitis with reactive arthritis, is recognised.’

The aetiology of Reiter’s syndrome remains controversial. Recently, Yersinia species infec- tion, along with other microbial pathogens, has received much attention as a potential triggering agent in this condition.’-4 Genetic susceptibility also appears to be involved, with approximately 60% to 70% of patients being HLA-B27 p~si t ive.~ However, the precise role of genetic factors and microbial antigens in the patho- genesis of this disease remains to be elucidated. Our patient was a genetically susceptible host (HLA-€327 positive) who had serological evidence of recent Yersinia infection, suggesting a likely aetiological role for this microbe, a t least in association with the acute ocular signs and prob- ably also the dactylitis involving his right fifth finger. The HLA-B27 positive individual appears to be susceptible to an overlapping spectrum of disease, including acute anterior uveitis, Reiter’s syndrome and various forms of seronegative arthropathy, which often displays a prolonged clinical course of exaccerbations and remissions.’-3 It might be argued that the presence of the dactylitis involving the right fifth toe four months prior to the onset of the acute ocular signs and the high Yersinia serology, may well reflect this frequent association of the HLA-B27 haplotype with various forms of reactive arthritis following systemic infections caused by a wide variety of microorganisms.

Ocular involvement in Reiter’s syndrome or HLA-B27 associated reactive arthritis is frequently described.’ The manifestations over- whelmingly present as pathology of the anterior globe and include, most commonly, conjunc-

tivitis and iridocyclitis. Keratitis is a less frequently seen but well described entity which may mimic adenovirus infection, as highlighted by this case.5

Posterior segment presentations of Reiter’s syndrome have not been commonly reported. Vitritis with blurring of fie disc margins or diffuse retinal or macular oedema complicat&g cases O f severe or prolonged iridocyclitis is well recognised.’*-* Other manifestations include pars plana exudates and occasionally retinal vasculitis.* A recent large survey by Rodriguez et d.* suggests that these posterior segment mani- festations, which may be severe and sight threatening, are an under-recognised feature of HLA-B27 positive uveitis and associated syndromes. However, in the case reported here we describe the rare occurrence of focal posterior segment lesions. A review of the English litera- ture revealed only one other detailed report of focal retinal involvement in this disease. This case featured not only episodes of diffuse retinal oedema, but also the appearance of ‘yellowish- white point (lesions)’ between the fovea and papilla, which occurred after an initial irido- cyclitis had subsided.6

Regarding the site of the retinal lesions, one might speculate that the appearance of a new area of RPE atrophy and an accompanying window defect on fluorescein angiography, closely corresponding to the initial area of retinal involvement, point towards a lesion at the level of the W E or inner choroid.

However, no definite pathological process can be ascribed to these transient white retinal spots. The resolution of the lesions was too rapid for cottonwool spots, which usually take about six weeks to resolve, and we did not see any other signs of vasculitis.’ It may represent a choroiditis, but the course would be atypical, leaving only minimal pigmentary changes. In multiple evanes- cent white dot syndrome the spots fade completely, but usually after several weeks.’O

These lesions do, however, constitute only the second detailed report of focal retinal involve- ment in Reiter’s syndrome, and their evolution and response to steroids implies an inflammatory aetiology. A systemic Yersinia infection may have played some role in initiating this inflammatory process.

Incomplete Reiter’s syndrome with focal involvement of the posterior segment 65

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2. Brewerton DA. HLA-BZ7 and the inheritance of suscep- tibility to rheumatic disease. Arthritis Rheum 1976;19:656-68.

3. Wakefield D, Montanaro A, McCluskey P. Acute anterior uveitis and HLA-B27. Surv Ophthalmol 1991;36:223-32.

4. Solem JH, Lassen J. Reiter’s disease following Yersiniu enterocoliticu infection. Scand J Infect Dis 197 1;3:83-5.

5. Rowson NJ, Dart JKG. Keratitis in Reiter’s syndrome [letter]. Br J Ophthalmol 1992;76:126.

HAPTIC SHELLS CONFORMERS r I

6. Mattson R Recurrent retinitis in Reiter’s disease. Acta Ophthalmol. 1955;33:403-8.

7. Haarr M. Reiter’s disease. Acta Ophthalmol 1945;23:143-9.

8. Rodriguez A, &ova YA, Pedroza-Seres M, Foster CS. Posterior segment ocular manifestations in patients with HLA-BZ7-associated uveitis. Ophthalmology, 1994; 101:

9. Brown GC, Brown MM, Hiller T, Fischer D, Bcns,on WE, Magargal LE. Cotton wool spots. Retina 1985;5:206-14.

10. Jampol LM, Sieving PA, Pugh D, Fishman GA, Gilbert H . Multiple evanescent white dot syndrome. 1. Clinical findings. Arch Ophthalmol 1984;102:67 1-4.

1267-74.

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66 Australian and New Zealand Journal of Ophthalmology 1995; 23(1)