Anterior Uveitis and Hypopyon*
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ANTERIOR UVEITIS AND HYPOPYON* BY Leonardo P. DAlessandro, MD (BY INVITATION), DavidJ. Forster, MD (BY INVITATION), AND Narsing A. Rao, MD INTRODUCTION ANTERIOR UVEITIS IS THE MOST COMMON FORM OF INTRAOCULAR INFLAM- mation seen by the ophthalmologist. When severe, anterior uveitis can result in the formation of hypopyon within the anterior chamber. Hypo- pyon in endogenous anterior uveitis has been related classically to Beh- get's syndrome.1-3 A few investigators have also reported the occasional observation of hypopyon in association with herpetic keratouveitis,4'5 Reiter's syndrome, and ankylosing spondylitis.6-8 We undertook a study to determine the incidence, as well as the most common causes, of hypopyon in patients with acute endogenous anterior uveitis. MATERIALS AND METHODS Medical records of all patients with endogenous anterior uveitis referred to our institution from 1984 to 1990 were reviewed. For each patient, information was obtained regarding the presence or absence ofhypopyon as determined by slit lamp examination, as well as medical history, review of systems, and results of laboratory investigations performed. Laboratory investigations had been performed using a tailored ap- proach and included any or all of the following: tests for antinuclear antibodies, angiotensin-converting enzyme, rheumatoid factor, and HLA- B27 and HLA-B5; syphilis serologic studies, tuberculin skin testing; chest radiography; and radiologic examination of the lumbosacral spine. Patients with concurrent posterior uveitis (retinitis or choroiditis), as well those who had recently undergone ocular surgery or sustained pene- trating trauma to the eye, were excluded. *From the A. Ray Irvine Jr, MD, Ophthalmic Pathology Laboratory of the Doheny Eye Institute, and the Department of Ophthalmology, University of Southern California, Los Angeles. This study was supported in part by core grant EY03040 from the National Eye Institute, Bethesda, and by an unrestricted grant from Research to Prevent Blindness, Inc, New York. TR. AM. OPHTH. Soc. vol. LXXXIX, 1991 304 Rao et al RESULTS A total of216 cases ofanterior uveitis were identified. Ofthese, 155 were classified as acute, endogenous anterior uveitis (AAU) in that the symp- toms had an acute onset and the inflammation persisted for less than 3 months. These patients also met the other criteria for inclusion in this study. Hypopyon developed in 11 (7.1%) of the 155 patients. The hypo- pyon occupied from 5% to 15% ofthe anterior chamber: in five patients it filled about 5%, in another five it filled 10%, and in the remaining patient it filled about 15% of the anterior chamber. The characteristics of the 11 patients who had hypopyon secondary to anterior uveitis are summarized in Table I. The age ofthe patients ranged from 10 to 63 years (mean, 37 years), and there was no apparent predilec- tion for either sex. Overall, a slight majority (55%) ofpatients were white, but hypopyon was seen in other races as well (three Orientals, one black, and one Hispanic). In all cases the hypopyon was unilateral, and in none were there "mutton fat" keratic precipitates. The inflammation was acute in all cases and recurrent in eight. The hypopyon occurred in the first episode of acute uveitis in only four cases; in the remaining seven, the hypopyon was seen with recurrent episodes of AAU. No patient had a recurrent hypo- pyon. Four ofthe 11 patients with hypopyon also had fibrinous membrane formation in the anterior chamber. In two cases there was a pronounced spillover of inflammatory cells into the anterior vitreous. Visual acuity at the time of the hypopyon was 20/200 or less in six patients and 20/100 to 20/50 in the other five. Ten of the patients were treated with topical corticosteroids, topical mydriatic agents, and systemic (oral) corticoster- oids or deposteroid injection(s) in sub-Tenon's space. The remaining patient received topical steroids every 2 hours and mydriatics only. Visual acuity in all patients improved by an average of 5 Snellen lines. After 2 weeks of treatment, visual acuity was 20/40 or better in ten patients and 20/70 in one. In all cases the hypopyon disappeared within the first 10 days of treatment. In terms of HLA association, 9 (82%) of the 11 patients with AAU in whom hypopyon developed were positive for HLA-B27. Two of these HLA-B27-positive patients had Reiter's syndrome and one had ankylosing spondylitis; all three of these patients were male. The remaining six patients positive for HLA-B27 had no documented systemic illness at the time oflast examination; four ofthese patients did complain oflower back pain, but radiologic examinations of the lumbosacral region were normal. Of the patients negative for HLA-B27 who had hypopyon, one patient, aged 63, had mixed connective tissue disease (MCTD) and another, aged Anterior Uveitis 305 Z cZ : 6 0 >~~~~~~~~~~ 0<~~~~~~~~~~ cn;808808cq C c cqc8808 z XzCCCCNiiGG t*0 0 Z Q u ZH QQQQQ Q QQQr" 0~~~~~~~ 0 A" rzS Hoo oooooooo A H*0 z 0 0~~~~~~~~~~ zr&4~~ ~ ~ 0 0 0 48H z. 0 + + + + + + + +"0 bO ,;tR 04I -<0 0 bC) u mo~~~ F0 O~~~~~~J~ ~ ~ 00 -4 0 C# IVIn t "- 0 0( 4- + G 306 Rao et al 40, had idiopathic uveitis; both patients were white women. In both cases hypopyon occurred during recurrent episodes of uveitis. At the time of the hypopyon, visual acuity was 20/200 in the patient with MCTD and 20/70 in the patient with idiopathic uveitis. Both patients were treated with topical steroids, mydriatic agents, and systemic (oral) corticosteroid; after 2 weeks of treatment, visual acuity improved to 20/70 in the patient with MCTD (this patient also had a history of amblyopia) and to 20/40 in the patient with idiopathic uveitis. Complicatidns were relatively uncommon in this group ofpatients, but follow-up was not long enought to make a definitive statement regarding long-term sequelae. Posterior synechiae developed in three patients. Bilateral posterior subcapsular cataracts and early band keratopathy de- veloped in a 10-year-old Vietnameses boy (case 3) who had a 4-year history of recurrent bilateral iridocyclitis. This boy was diagnosed as having ankylosing spondylitis, and he had a brother who also had ankylosing spondylitis. This patient underwent bilateral pars plana lensectomy and vitrectomy, and at last examination visual acuity was 20/60 in each eye. Of the 155 cases of AAU, 62 (40%) were positive for HLA-B27 and 93 were negative for HLA-B27; of the 11 patients with hypopyon, 9 were positive for HLA-B27 and only 2 were negative. Thus, the incidence of hypopyon was 14.5% among AAU patients who were positive for HLA- B27, but only 2.2% among AAU patients who were negative for HLA- B27. Chi-square analysis of these two groups revealed significantly in- creased occurrence of hypopyon in the HLA-B27 group (P < 0.003). Two patients with Behcet's syndrome and anterior uveitis were included in the group of 155 patients, but hypopyon did not develop in these patients. DISCUSSION In this series of patients with anterior uveitis, the most common cause of hypopyon was HLA-B27-related iridocyclitis, accounting for 82% (9 of 11) of the cases. However, the incidence of hypopyon among HLA-B27- positive patients with AAU was 14.5%. All nine patients had characteristic features of HLA-B27-related anterior uveitis: they were young to middle- aged and had acute, unilateral or alternating unilateral, severe episodes of anterior inflammation. Four patients also had associated fibrinous mem- brane formation in the anterior chamber, but none of the patients had mutton fat keratic precipitates. These findings (including the absence of keratic precipitates) have been described by several investigators7-16 and are typical ofHLA-B27-related AAU; however, none ofthese investigators found as high an incidence of hypopyon in association with the anterior uveitis as was noted in our study. Anterior Uveitis 307 Typically, our patients had been treated initially with intensive (every 1 to 2 hours) topical corticosteroids for their acute anterior uveitis; how- ever, in spite of this treatment, hypopyon developed, and the majority of the patients required supplemental systemic corticosteroids (generally 60 to 80 mg of prednisone daily) to bring the inflammation under control. The exception was one patient who responded well to intensive topical treatment. In all cases, the hypopyon resolved after several days of systemic corticosteroid treatment. None of our patients, including the two with Behcet's syndrome, had recurrent hypopyon, although this is the classic pattern seen in patients with this syndrome.1 Two other patients, excluded from this series because they also had evidence of retinal vasculitis, had Behcet's syndrome with the characteristic recurrent hypopyon. Overall, there did not appear to be a sex predilection among the patients in whom hypopyon developed. However, among patients who were positive for HLA-B27, hypopyon was seen more frequently in males than in females (ratio, 2:1). Furthermore, all three patients who had hypopyon associated with an HLA-B27-related systemic disease were male. Of interest in our patients was the wide distribution of cases with respect to race or ethnic origin. HLA-B27-related iridocyclitis is typically described as occurring in whites. However, ofour 11 cases ofhypopyon in HLA-B27-related AAU, only 4 patients (44%) were white; 3 patients (33%) were Oriental, 1 (11%) was black, and 1 was Hispanic. In addition, among the 53 HLA-B27-positive patients who did not have hypopyon, 37 (70%) were white, 8 (15%) were Hispanic, 5 (9%) were Oriental, 2 (4%) were black, and 1 (2%) was Asian Indian. To our knowledge, this racial distribu- tion has not been reported in previous series.8 13,16 Our study did not include long-term follow-up, so no conclusions can be drawn regarding the long-term prognosis of these patients.