CASE REPORT An atypical case of HLA-B27-associated uveitis with hypopyon and posterior segment involvement BY THOMAS XIE, OD & ETTY BITTON, OD, MSc, FAAO Introduction cases.7-10 Furthermore, anterior and ABSTRACT intermediate uveitis cases have a veitis, the most common form lower risk of hypopyon compared to The presence of a hypopyon and of inflammatory eye disease, is posterior segment involvement U patients with only anterior uveitis.6 an important public health concern. are uncommon clinical findings in This report highlights an atypical It accounts for a significant percent- HLA-B27-associated uveitis. Further- case of HLA-B27-associated uveitis more, first-time attacks rarely occur in age (estimated at 10–15%) of preva- that presented with both a hypopyon the elderly. This report highlights an lent cases of legal blindness in the atypical uveitis case involving an older and severe intermediate uveitis in an United States.1 The most frequent patient, an evident hypopyon and elderly man. severe intermediate uveitis in one eye. subtype is anterior uveitis, represent- The 60-year-old Caucasian male was ing up to 92% of total cases in com- 2 admitted for a painful, red eye with munity-based ophthalmic practices. Case report a sudden decreased vision to hand HLA (human leukocyte antigen)- A 60-year-old Caucasian male was motion, in the affected eye. Ocular and B27 positivity, a human major his- seen in the eye clinic of a hospital systemic history were unremarkable. tocompatibility complex (MHC), is reporting a red, painful right eye An anterior chamber examination of the eye revealed extensive cells and the most common identifiable cause with decreased vision. The patient flare with a conspicuous hypopyon. An of anterior uveitis and accounts for was in fact seen three days earlier for evaluation of the posterior segment about 50% of the cases in different pain and inflammation from a right revealed significant vitreous haze, populations.3,4 HLA-B27-associated shoulder injury for which he was obstructing all view to the retina. Despite the age and the atypical ocular uveitis is characterized by recurrent put on a narcotic analgesic (oxy- findings of the patient, a diagnosis of alternating acute unilateral attacks codone 5 mg and acetaminophen HLA-B27-associated uveitis was made of intraocular inflammation of the 325 mg marketed as Percocet, one following an extensive clinical and anterior segment of the eye, and tablet every four hours as needed). laboratory evaluation. The inflammatory 5 condition was successfully managed typically affects young male adults. The onset of his ocular symptoms with a combination of intravenous, In uveitis related to HLA-B27, the coincided with the introduction of topical and oral corticosteroids tapered presence of a hypopyon – a layer Percocet so he discontinued the over the course of a few weeks, and of white blood cells in the anterior drug after one day, however, his visual acuity recovered to 20/30. chamber – and posterior segment vision continued to worsen. Ocular This case is an important reminder involvement of the eye are uncom- history was unremarkable with no that atypical signs, such as a hypopyon or intermediate uveitis, can occur mon. reports of trauma, surgery, inflam- and may be a significant sign of A hypopyon suggests severe mation or infection. Medication was HLA-B27-associated uveitis. Clinicians anterior segment intraocular inflam- limited to the recent use of Percocet should be aware of the diverse mation and is a rare occurrence in for the shoulder and the occasional manifestations of HLA-B27-associated uveitis and be careful to include a patients with uveitis, occurring in nonsteroidal anti-inflammatory drug 6 comprehensive assessment of both the less than 1% of all uveitis patients. (naproxen) for nonspecific pain in anterior and posterior segments of any Posterior segment involvement, the body with no reported allergies presenting painful, red eye. namely intermediate and/or pos- to any medication. Review of all Keywords: anterior uveitis, intermediate terior uveitis, is also infrequent and systems revealed episodes described uveitis, HLA-B27, hypopyon has been reported in up to 25% as podagra (i.e. inflammation on the of HLA-B27-associated uveitis big toe related to episodes of gout)11 C A N A D I A N J O U R N A L O F O P TO M E T R Y | R E V U E C A N A D I E N N E D ’ O P TO M É T R I E VOL 74 | NO 1 | 2012 47 d1071 new text.indd 47 12-04-18 11:26 AM within the past year (although he was Table 1: Clinical findings at initial presentation never officially diagnosed with gout) and a history of papular rashes on RIGHT EYE (OD) LEFT EYE (OS) his forehead and both his shins with mild erythema and some excoria- Visual acuity Hand motion 20/30 tion (abraded areas where the skin Pupil reactions Normal Normal is torn or worn off). Upon further Extraocular movements Normal Normal questioning, the patient reported Tonometry (Tonopen) 21 mmHg 22 mmHg no history of ulcers, sores, irritable Bulbar conjunctiva 2+ injection bowel disease, bloody stool, urina- tion difficulties or shortness of Cornea Fine keratic precipitates widely distributed breath. The patient’s family history Anterior chamber 4+ cells and flare, fibrin production with a 1.5 mm high hypopyon, grade IV Van Herick angle was unremarkable. The patient also Unremarkable denied excessive nicotine or alcohol Crystalline lens 1+ nuclear sclerotic cataract without posterior use, and had not engaged in any synechiae sexual activity recently. The patient Posterior segment Severe vitreous haze (grade 4+ vitreous cell) with was oriented to time, place and per- extensive vitreous debris son, and was lucid at the time of the examination. nongranulomatous anterior and Upon ocular examination, visual intermediate hypopyon uveitis, with acuity (VA) was hand motion OD a tentative HLA-B27 association. (without improvement with pinhole) The patient received 125 mg of and 20/30 OS. Slit lamp examina- intravenous anti-inflammatory tion of the right eye revealed 2+ glucocorticoid (methylprednisolone injection of the bulbar conjunctiva. sodium succinate) coupled with a Fine keratic precipitates (KPs) were topical anti-inflammatory corticoste- widely distributed throughout the roid (1% prednisolone acetate qh, cornea, although no corneal thin- with a loading dose before bedtime ning was noted. Anterior chamber and upon awakening), a cycloplegic/ Figure 1 – A hypopyon (height of (AC) examination of the right eye mydriatic agent (atropine 1% tid), 1.5mm) seen at the lower quadrant of revealed 4+ cells and flare (without and an oral anti-inflammatory the anterior chamber convection current), fibrin produc- corticosteroid (prednisone 80 mg tion with a 1.5 mm hypopyon in the daily). The patient was then sent to B27 positivity. The patient did not lower quadrant as seen in Figure 1. the laboratory to have his blood have a primary care provider, so a Dilated funduscopic examination drawn for further analysis. consultation with a rheumatologist (DFE) revealed severe vitreous haze A subsequent review of his was recommended. (grade 4+ vitreous cells), obstructing laboratory examination revealed an The patient responded well to all view to the retina. B-scan ultra- elevation of ESR, CRP and white therapy. Ten days after treatment sound revealed extensive vitreous blood cells. Laboratory results were was initiated, VA of the right eye debris. The left eye revealed only an positive for the following markers: improved to 20/60, IOP was early nuclear sclerotic cataract with HLA-B27, HSV IgG and HSV IgM. 16 mmHg, anterior segment re- no evidence of active or past inflam- (SeeTable 2) The remainder of the vealed few fine KPs inferiorly, 1+ mation. A summary of the ocular work-up, including ACE, Toxoplas- cells and flare in the AC and a findings is shown in Table 1. ma, FTA-ABS, RPR and VZV titre, <0.5 mm hypopyon. A DFE dem- Given the B-scan, the patient was was negative. The etiology of the onstrated 1+ anterior vitreous cells, diagnosed with acute unilateral uveitis was thus confirmed as HLA- snowbanks and snowballs resting 48 VOL 74 | NO 1 | 2012 C A N A D I A N J O U R N A L O F O P TO M E T R Y | R E V U E C A N A D I E N N E D ’ O P TO M É T R I E d1071 new text.indd 48 12-04-18 11:26 AM Table 2: Clinical laboratory results PURPOSE NORMAL VALUES RESULT Erythrocyte Inflammation ≤ 30 mm/hr Elevated (110 mm/hr) Sedimentation Rate (ESR) C-Reactive Protein (CRP) Inflammation <6 mg/L Elevated (19.35 mg/L) White blood cells Inflammation 4 × 109 to Mild leukocytosis 1.1 × 1010/L (13.1 x 109/L) Human Leukocyte Specific protein Negative Positive Antigen B27 (HLA-B27) strongly associated with spondyloarthropathies Figure 2: Anterior segment photo illustrating the resolved hypopyon and a Herpes Simplex Virus Herpes simplex Negative Positive (HSV) IgG virus-specific antibody clear anterior chamber Herpes Simplex Virus Herpes simplex Negative Positive (HSV) IgM virus-specific antibody Angiotensin-Converting Sarcoidosis Negative Negative Enzyme (ACE) The patient was initially diagnosed Toxoplasma Toxoplasmosis Negative Negative with an acute nongranulomatous Treponema Pallidum Syphilis Negative Negative uveitis with a tentative HLA-B27 Antibody (FTA-ABS) association, however, the hypopyon Rapid Plasma Regain Syphilis Negative Negative and intermediate uveitis were atypi- (RPR) cal. Ramsay and Lightman (2001) Varicella-Zoster Virus Varicella zoster virus Negative Negative classified the causes of hypopyon (VZV) titer antibodies into non-infectious causes, infec- tious agents, neoplasms, and corneal inferiorly. The macula, optic disc and anterior segment was unremarkable, disorders.26 Table 4 shows the most the rest of the retina were unremark- and a posterior segment examination common differential diagnosis for able.