Case report

A 14-year-old Japanese boy noted a blurred vision for 3 weeks in the left eye and was referred to our clinic on 1 February 2001. His best-corrected visual acuity (BCVA) was 6/6 in the right eye and 6/300 in the left eye. Intraocular pressure was normal in both eyes. Heavy ciliary injection, fine keratic precipitates, 2+ cells, 2+ flare, peripheral anterior synechia (PAS) in the anterior chamber, as well as 2+ vitreous cells were noted in the left eye. Both ocular angles were wide open, except for the PAS, with no hypopyon. Fundoscopic examination disclosed ghost vessels in both eyes. Severe retinal vasculitis, pre-retinal haemorrhage, vitreous opacity and scattered exudate were noted in the left eye (Figure 1). Fluorescein angiography (FAG) revealed perivascular staining and leakage from the veins in the left eye. Erythrocyte sedimentation rate (ESR) was 20 mm/h. HLA typing revealed B27 haplotype. Purified-protein derivative skin test was barely positive, but not strong enough to indicate Eales disease. Results of other laboratory tests were normal. Rigorous examination in internal medicine, neurology and dermatology did not detect any symptoms or signs of Behcet's disease, sarcoidosis, systemic lupus erythematosus, and other autoimmune diseases. HLA-B27-associated uveitis was suspected. Both eyes were treated with topical 0.1% betamethasone. For the left eye, additional dexamethasone (6 mg) was subconjunctivally injected daily.

Figure 1.

Panfundoscopic view of the left eye at the initial examination.

Full figure and legend (119K)

On 15 February, BCVA in the left eye had increased to 6/30. Retinal vasculitis remained severe and optic disc neovascularization was recognized. He started a daily dosage of 40 mg of prednisolone. On 1 March, retinal vasculitis subsided slightly. However, proliferative tissues along optic disc neovascularization remained unchanged. On 22 March, in the left eye, FAG revealed massive optic disc neovascularization and wide nonperfusion area became evident (Figure 2). Consequently, panretinal photocoagulation was immediately performed. However, on 16 April, traction detachment developed in the left eye. He received the first vitrectomy with silicone oil tamponade. Systemic prednisolone was tapered down to. However, on 6 June, PVR had relapsed and BCVA in the left eye was 6/60. There were no remarkable changes in the right eye. The second vitrectomy, reinjection of silicone oil, buckling and encircling were performed. Additionally, systemic prednisolone was increased to 40 mg. Since this operation, there has been no relapse and prednisolone had been tapered off over a period of 13 weeks. However, BCVA in the left eye has decreased and remained count finger.

Figure 2.

Fluorescein angiogram of the left eye on 22 March. Hyperfluorescence of the optic head and wide avascular area were seen.

Full figure and legend (110K)

Comment

The patient reported here could be one of the most fulminant manifestations developed in HLA-B27-associated uveitis. Systemic corticosteroid therapy could not terminate or decrease the development of neovascularization, resulting in PVR. Laser photocoagulation to the nonperfusion area did not yield any effects on the clinical outcome.

Differential diagnosis included Behcet's disease, sarcoidosis, systemic lupus erythematosus, viral retinitis, and Eales disease. He did not have any systemic signs and symptoms such as oral aphtha, genital lesions, and skin lesions. The absence of these systemic symptoms and laboratory results indicative of autoimmune diseases excluded many of the aforementioned diseases, especially Behcet's disease. Viral retinitis will yield more severe exudates than preretinal or vitreous haemorrhage. It is uncommon in Eales disease that anterior uveitis and dense vitritis exist as seen in our case.⁴

This patient and his father have HLA-B27, which is possessed only in 0.8% of the Japanese population.¹ His father had a past history of unknown uveitis. It is quite possible that his disease was also related to the haplotype of HLA-B27. Furthermore, HLA-B27 AAU occurs in B27-positive members of the patients' families (approximately 10%) more frequently than in B27-positive healthy controls (1–2%).⁵ Posterior segment involvement in HLA-B27-associated uveitis occurs somewhere between 4 and 17.4% in Caucasians.^2,6 Benitez del Castillo reported that the blood–aqueous barrier had been broken when HLA-B27-positive acute anterior uveitis was in active stage.⁷ However, these cases usually have good responses to corticosteroid or immunosuppressive therapy. HLA-B27-associated uveitis in Japanese have also good visual prognoses.⁸ However, there was no report of PVR despite intensive therapy. Our case, thus, verifies the fact that severe and refractory inflammation can happen in HLA-B27-associated uveitis that could lead to the development of PVR in a short period.

References

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