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Visual Outcome of Patients with Idiopathic Ischaemic and Non-Ischaemic Retinal Vasculitis

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Visual Outcome of Patients with Idiopathic Ischaemic and Non-Ischaemic Retinal Vasculitis VISUAL OUTCOME OF PATIENTS WITH IDIOPATHIC ISCHAEMIC AND NON-ISCHAEMIC RETINAL VASCULITIS H. E. PALMER, M. R. STANFORD, M. D. SANDERS and E. M. GRAHAM London SUMMARY Idiopathic RV can be classified into ischaemic and This study investigated whether patients with ischaemic non-ischaemic forms; idiopathic ischaemic RV is like retinal vasculitis have a worse visual outcome than 'Eales disease', named after Henry Eales who first 1880? those with non-ischaemic disease. A retrospective study reported the disease in To date, there is no was made of 53 patients with idiopathic retinal evidence that patients with ischaemic RV have a vasculitis (RV), with minimum 5 year follow-up. worse visual prognosis than those with non-ischaemic disease. Indeed, ischaemic disease has been reported Patients were categorised into ischaemic and 4 non-ischaemic groups by fluorescein angiography. by Elliot to be associated with a good prognosis. In a Visual outcome was determined by visual acuity at pros�ective study between 1970 and 1991, Atmaca their last attendance. Twenty patients (38 eyes) had et al: also found that the visual outcome for Eales ischaemic RV; 33 patients (63 eyes) had non-ischaemic patients was favourable. Other papers on Eales RV. At presentation there was no significant difference disease, whilst giving detailed descriptions of the between the groups in the proportion of eyes with poor disease manifestations, have not focused upon visual 6 7 vision (6/60 or less). Ischaemic RV patients had a worse outcome. Patients with pars planitis have also been s visual outcome than those with non-ischaemic RV: 13 found to have a good visual outcome. It was our of 38 (34%) eyes in the ischaemic group had a final clinical impression that patients with ischaemic RV poor vision compared with 4 of 63 (6%) eyes in the fared less well. The main aim of this study was, non-ischaemic group (Fisher's exact test, p = 0.0005). therefore, to determine whether patients with ischaemic RV have a worse visual outcome than Retinal vasculitis (RV) is an inflammatorydisease of those with non-ischaemic disease and to assess the the retina, uveal tract and vitreous, which causes of visual failure in each group. predominantly affects young people and can lead to blindness. It is a disease which is characterised by METHODS intraocular inflammation with involvement of the A retrospective study was undertaken of RV patients retinal blood vessels. When the inflammation is who had been attending the Medical Eye Unit at predominantly at the pars plana, the disease is St Thomas' Hospital for a minimum follow-up period termed intermediate uveitis. RV can occur in of 5 years, for whom there was accurate documenta­ isolation (idiopathic RV), or in association with tion of ophthalmic features at least every 6 months. systemic inflammatory conditionssuch as sarcoidosis The age at presentation and sex of each patient were 1 and Beh<;et's disease, infections or neoplasia. The recorded, as were details of their smoking habits and two main causes of visual loss in R V are cystoid duration of RV before referral. At presentation, all macular oedema and vitreous haemorrhage, from the patients had had a full ophthalmological and new vessel formation consequent upon retinal general medical history taken, and a thorough ischaemia, which can occasionally lead to traction examination which included Snellen and reading retinal detachment? acuities, colour vision testing, slit lamp and fundal examination and grading of intraocular activity. From: Medical Eye Unit, St Thomas' Hospital, London, UK. Particular note was made of the cause of poor vision Correspondence to: Miss H. E. Palmer, Department of Ocular (6/60 Immunology, Rayne Institute, St Thomas' Hospital, London SEI or less) in an affected eye. The eyes were 7EH, UK. categorised as having ischaemic or non-ischaemic Eye (1996) 10, 343-348 © 1996 Royal College of Ophthalmologists 344 H. E. PALMER ET AL. disease on the basis of fluorescein angiography: patients with ischaemic RV smoked compared with ischaemic RV was identified by any angiographic 18 of 33 (54.5%) with non-ischaemic RV. evidence of capillary non-perfusion. In addition, the One patient with ischaemic RV had orally fluorescein angiograms previously performed on all controlled diabetes mellitus but no diabetic retino­ patients were retrospectively reviewed for the pres­ pathy. There were no patients with hypertension or a ence of macular ischaemia which may not have been haemoglobinopathy. identified at the time of the angiogram. Macular ischaemia was defined as closure of perifoveal Ophthalmic Findings at Presentation capillaries identified by an enlarged andlor irregular Disease Duration Before Referral foveal avascular zoneY The majority of patients were initially under the care Disease course was documented in terms of the of their local ophthalmic unit and then referred to number of vitreous haemorrhages and relapse rate. St Thomas' Medical Eye Unit for further manage­ Relapse was defined as an increase in vitreous ment. The mean duration of RV prior to referral was cellularity, which was usually associated with signs 35 months for ischaemic and 32 months for non­ of retinal inflammation such as cystoid macular ischaemic RV patients. oedema.lO Details of systemic treatment and photo­ coagulation were noted. Visual Acuity and Causes of Grade IV Vision At the patient's last visit Snellen visual acuity was The distribution of visual acuity grades at recorded and, again, any cause of visual loss was presentation is as shown in Fig. 1. At presentation, recorded. As in our previous papers, outcome was overall 16 eyes of 15 patients had grade IV visual graded as follows: grade I ('excellent'), 6/6 or better; acuity, the causes of which are listed in Table II. In grade II ('good'), 6/9 or 6112; grade III ("fair'), over one-third of these eyes (6/16) this was due 6/18-6/36; and grade IV ('poor'), 6/60 or worse. A to cystoid macular oedema (CMO). Vitreous successful visual outcome was defined as either (a) haemorrhage was present in 3 of 16 eyes at maintenance of visual acuity of 6/12 or better, or (b) presentation, of which 2 occurred in ischaemic RV improvement by one or more grades, by the last patients. Although a greater proportion of the eyes clinic attendance. Patients who did not fulfileither of with ischaemic RV had grade IV acuities at this stage these criteria were defined as having a poor visual (9 of 38 (23.6%) with ischaemic RV compared with outcome.](),ll 7 of 63 (11.1 %) with non-ischaemic RV), this difference was not statistically significant (Fisher's RESULTS exact test, p = 0.08). Patients and Characteristics at Presentation The patients and their characteristics at presentation Disease Course 5 are outlined in Table 1. The case files of 53 patients Follow-up ranged from to 20.2 years and was not with idiopathic RV were studied: 20 had ischaemic significantly different between the two groups RV and 33 had non-ischaemic RV. Forty-eight of (median of 6.2 years for ischaemic and 5.9 for these patients had bilateral disease and in 5 patients non-ischaemic patients: Mann-Whitney V-test, 7 the disease was unilateral; there was thus a total of p = 0.8 ). 101 affected eyes. There were no significantdifferences in either age Disease Relapses (Mann-Whitney V-test, p = 0.46) or sex distribution The number of relapses over the follow-up period for (Fisher's exact text, p = 0.22) between the two the ischaemic RV patients ranged from nil to 14 groups. A greater proportion of the patients with (mean of 2.26) and for the non-ischaemic RV ischaemic RV were smokers compared with their patients ranged from nil to 8 (mean of 2.71). When non-ischaemic counterparts: 14 of the 20 (70%) the follow-up period for each patient was taken into Table I. Patient characteristics at presentation lschaemic RV Non-ischacmic RV Total RV No. of patients 20 33 53 No. of affected eyes 33 68 101 Age (years) Range 6-50 11-56 6-56 Median 25 26 26 Sex Male 9 10 19 Female 11 23 23 Smokers Yes 14 (70%) 18 (54.5%) 32 No 6 (30%) 15 (45.5%) 21 VISUAL OUTCOME IN RETINAL VASCULITIS 345 statistically significant (Mann-Whitney V-test, p = 0.33). Vitreous Haemorrhage A significantly higher number of ischaemic RV patients had at least one episode of vitreous haemorrhage compared with the non-ischaemic RV patients (9 and 1 respectively; Fisher's exact test, p = 0.0003). In the case of the non-ischaemic RV patients this vitreous haemorrhage occurred in one eye and was secondary to neovascularisation due to inflammation rather than ischaemia. The vitreous haemorrhage resolved and the new vessels regressed on systemic treatment leading to a successful visual outcome. Six of the 9 ischaemic RV patients (75%) were smokers. Macular Ischaemia On review of all the angiograms performed, 4 patients (7.5%) had macular ischaemia. The visual acuities in the affected eyes ranged from 6/12 to 6/36, and resulted in a poor visual outcome in 3 of these eyes. Two of these patients (3 eyes) had been documented as having non-ischaemic RV; the remaining 2 patients had ischaemic RV. None of II 1/1 IV the affected eyes had grade IV vision at the end of 3 Visual Acuity Grade the study although eyes fulfilled the criteria for a poor visual outcome. Fig. 1. The distribution of visual acuity grades at the Treatment beginning of the study of eyes ofischaemic RV (above) and non-ischaemic RV patients (below). Grade I, 6/6 or better; Photocoagulation grade II, 6/9 or 6//2; grade III, 6/18-6/36; grade IV, 6/60 or None of the non-ischaemic RV patients had laser worse.
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