The Hidden Salmon Patch: Ocular Lymphoma Mistaken as Vogt Koyanagi Disease

Ocular inflammation from various causes may have similar clinical presentation thus careful clinical evaluations are mandatory particularly when the disease appear to be resistant to treatment. This paper reports a case of ocular lymphoma which was initially treated as Vogt Koyanagi Harada disease at a different centre. A 65-year-old Sudanese man complained of gradual worsening left eye vision. Careful ocular and slit lamp examination revealed a conjunctival lesion with choroidal infiltration as well as exudative retinal detachment. Computed tomography scan (CT scan) showed left eye axial proptosis, and a homogenous enhancing mass at the posterior coat of the globe. Tissue biopsy of the conjunctival lesion revealed marginal Zone B cell (MALT type), low grade, non-Hodgkin’s lymphoma. AS the ocular signs and symptoms progressed, chemotherapy was initiated. The proptosis, exudative retinal detachment, disc hyperemia and swelling improved after chemotherapy. The post treatment CT scan showed reduction of the posterior ocular coat mass. The possibility of malignant lymphoma should be considered in patients with resistant uveitis despite administration of corticosteroid. Ocular lymphoma is an indolent tumour with good prognosis. Careful ocular examination, adequate imaging studies followed by early surgical biopsy will contribute to early diagnosis.

Successful Visual Outcome Following Vitrectomy for Complication of Ocular Tuberculosis

A 24-year-old Chinese gentleman presented with two weeks history of sudden floaters in his right temporal visual field associated with blurring of vision. This ex-smoker also reported chronic, bloody cough for two years and recent pleuritic chest pain. Examination revealed a thin patient with right eye visual acuity of 6/18 associated with optic nerve dysfunction, optic disc swelling and macula star, retinal vasculitis and retinitis. Despite anti-tuberculosis medication and corticosteroids, he developed neovascularisation. Subsequent vitreous haemorrhage necessitated trans pars plana vitrectomy, membrane peeling, endolaser and silicone oil injection. Final visual acuity was 6/9 with quiescent retinopathy.

Correlation of serum insulin like growth factor-I with retinopathy in Malaysian pregnant diabetics.

AIM: To study the association of serum insulin-like growth factor-I (IGF-I) with diabetic retinopathy. METHODS: Serum IGF-1 levels were measured in 25 pregnant diabetic patients and 25 pregnant non-diabetic patients who were matched for age, ethnicity, parity and period of gestation. Fundus examination was performed in both groups at 28, 32 and 36 weeks of gestation. RESULTS: The serum IGF-I level was significantly elevated in pregnant diabetics compared to pregnant non-diabetics (366±199µg/L vs 184±89µg/L, (P=0.0001) at 24 weeks, 535±251µg/L vs 356±89µg/L, (P=0.007) at 32 weeks and 404±166µg/L vs 264±113µg/L, (P=0.003) at 36 weeks of gestation). The pregnant diabetics with established diabetes had significantly higher IGF-1 level than gestational diabetes at 28, 32 and 36 weeks of gestation. The serum IGF-I level in pregnant diabetics with retinopathy was significantly higher than that in those without retinopathy at all periods of gestation. CONCLUSION: Increased serum IGF-1 in pregnancy may increase the risks for retinopathy.

Irreversible visual loss and optic nerve dysfunction associated with central retinal vein occlusion in Waldenstrom Macroglobulinemia.

Irreversible optic nerve dysfunction associated with central retinal vein occlusion (CRVO) is an unusual but important complication of Waldenstrom Macroglobulinemia (WM). Acute visual loss in CRVO is mainly due the severe macular oedema. However, ischaemic optic neuropathy needs to be considered in patients with CRVO when, (i) there is a relative afferent papillary defect and central scotoma, (ii) the visual acuity is not consistent with the retinal pathology, and (iii) the visual defects persisted despite resolution of macular oedema following treatment of the hyperviscosity state. The ischaemic type of CRVO is associated with a poor visual prognosis and the presenting visual acuity has a prognostic role. We report the first description of irreversible unilateral optic nerve damage associated with CRVO in a patient with WM.

A rare case of subretinal cysticercosis.

This is a case of a 25 year old lady whose eye had been infected by cysticercosis. This case highlighted that the inflammation was due to host immune response. She was treated with oral corticosteroid and the lesions regressed.

Accelerated diabetic retinopathy in pregnancy--a real and present danger.

A 36 year-old Malay lady with diabetes mellitus in pregnancy and poorly controlled hypertension developed rapid progression of diabetic retinopathy from no retinopathy to florid proliferative retinopathy over three months in her right eye. She had subsequent loss of vision due to vitreous haemorrhage in the peri-partum period. She had good final visual acuity with quiescent retinopathy following pars planar vitrectomy. A similar course was avoided in the left eye by timely pan retinal photocoagulation.

Multifocal choroiditis: ocular TB or side-effects of anti-TB therapy?

A patient with end-stage renal failure secondary to polycystic kidney disease was seen in the Eye Clinic for a corneal abrasion. Incidental fundal examination revealed bilateral multiple, small, raised, pale yellow sub-retinal nodules. Past medical history of inadequately treated pulmonary tuberculosis was obtained. Following initiation of anti-tuberculous therapy, the choroidal lesions resolved.

Bilateral optic neuritis in pregnancy.

A 25-year old primigravida at 11-weeks period of amenorrhoea presented with bilateral optic neuritis following Varicella Zoster viral (VZV) infection. She was serologically positive for systemic lupus erythematosus but negative for virus. The exact pathogenesis of the patient's severe optic neuritis, adduction and neurological deficit was unknown. The initiation of high dose steroids for optic neuritis was a big clinical dilemma in a pregnant patient with viral infection. The patient was treated with high dose steroids after three days of commencement of antiviral treatment. At 6 months after presentation, her visual acuity in the right eye was 6/36 with perception to light in the left.

Bilateral optic neuritis in pregnancy

A 25-year old primigravida at 11-weeks period of amenorrhoea presented with bilateral optic neuritis following Varicella Zoster viral (VZV) infection. She was serologically positive for systemic lupus erythematosus but negative for virus. The exact pathogenesis of the patient's severe optic neuritis, adduction and neurological deficit was unknown. The initiation of high dose steroids for optic neuritis was a big clinical dilemma in a pregnant patient with viral infection. The patient was treated with high dose steroids after three days of commencement of antiviral treatment. At 6 months after presentation, her visual acuity in the right eye was 6/36 with perception to light in the left.

Optic neuritis and retinal vasculitis as primary manifestations of systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a common multisystem disorder. However, retinal vasculitis as a primary manifestation of SLE is uncommon, accounting for only 4% of causes of retinal vasculitis. The postulated mechanism appeared to be vaso-occlusion of the retinal arterioles by thrombosis, with resultant ischaemia. Optic neuropathy in SLE is also rare, with a prevalence of 1%. This is a case report of a young lady who presented to us with retinal vasculitis as her initial presentation of SLE. Interestingly, the pathologic mechanism appeared to be inflammatory and not vaso-occlusive.

Behçet's disease in Japan and in Great Britain: a comparative study.

Behçet's disease (BD) is an important cause of visual morbidity throughout the world, but shows striking differences in racial predilection. Despite important advances in the therapeutic management of acute intraocular inflammation, the long-term impact of these new strategies on visual outcome of BD and their efficacy in different ethnic groups is unknown. A comparative study of patient characteristics, clinical ocular features and inflammatory score, and current therapy was undertaken on all patients fulfilling the International Study Group criteria for Behçet's disease and the Behçet's Disease Research Committee of Japan, who attended the Uveitis Clinics of Moorfields Eye Hospital (n=19) and Kurume University School of Medicine (KUS) (n=35) during a continuous consecutive four-week period. Japanese patients were significantly older (43.2+/-11.8 years) than the patients seen in London (35.4+/-8. 9 years). There was a predominance of male patients in both groups. All patients seen in KUS were Japanese, while the patients in London included 12 Caucasians, five Middle Eastern, one African, and one Asian. No significant differences were seen between the two populations in the duration of systemic disease or systems affected by the disease, such as mouth ulcers, genital ulcers, skin lesions including erythema nodosum, or arthritis. The duration of ocular disease was similar in both centres: around seven years. There was, however, a significant difference in the number of eyes with active anterior uveitis (59.7% KUS vs 18.4% London (chi-square: 5.4; p=0.006)) and/or posterior uveitis (31.3% KUS vs 18.4% London (chi-square: 5.42; p<0.02)). No significant differences were found in the number of eyes with optic disc swelling or optic atrophy and in each centre the number of eyes with vision greater than 6/9 or worse than 6/60 were the same. The treatment schedules were very different between the two centres. More patients were treated with topical steroids in Japan (68.7% KUS vs 10.5% London (chi-square: 30.5; p=0.001), but a similar number used concomitant intraocular pressure-lowering agents. More patients received systemic steroids in London (84.2% London vs 17% KUS (chi-square: 20.25; p<0.001)). Three patients received systemic steroids alone, five had prednisolone and cyclosporin, four had prednisolone and azathioprine, and four had triple therapy with prednisolone, cyclosporin, and azathioprine. Only one patient used colchicine. Cyclosporin use was similar in London and KUS (47.4% and 42.8%, respectively). In Japan, three patients used prednisolone alone and three tacrolimus (FK506). In addition, two patients, who were on steroids alone, took colchicine as well. More patients in Japan had undergone surgery for cataract and glaucoma (chi-square: 4.0; p=0.045). In KUS, seven of 67 eyes had cataract surgery. A further three eyes had visually significant cataract and two eyes had undergone glaucoma surgery. In contrast, no patients in London had undergone any surgery up to and including this period.

Increased CD4+ expression and decreased IL-10 in the anterior chamber in idiopathic uveitis.

PURPOSE: To compare cell types and cytokines in aqueous humor from patients with uveitis either occurring in association with a systemic disease or apparently isolated and not associated with a systemic disease. METHODS: Cells were collected by centrifugation of fresh aqueous humor from uveitis and controls, and immunofluorescence techniques were performed with markers for T cells, B cells, and monocytes. Cytokines were measured in the aqueous supernatants, and serum samples were assayed for soluble interleukin-2 receptors. RESULTS: When aqueous samples from idiopathic uveitis were compared with those from uveitis associated with a systemic disease, there were increases in CD3+, CD4+ (p = 0.001), and activated CD4+ T cells (p = 0.02) and a decrease in B cells (p = 0.0013). This was not reflected in the peripheral blood where there were no differences in the cell types or in soluble interleukin-2 receptor levels. No cells were obtainable from control aqueous. Interleukins-10 and -12, interferon-gamma, and transforming growth factor-beta2 were detected in aqueous supernatants. Interleukin-10 was reduced (p = 0.024) in uveitis in comparison with controls. CONCLUSIONS: The results suggest a selective recruitment of CD4+ T cells within aqueous humor but only in idiopathic uveitis. In both disease groups there was a decrease in the immunoregulatory cytokine interleukin-10, which might enable an immune response to occur in an otherwise highly immunosuppressive microenvironment. Increases in activated CD4+ T cells combined with depressed interleukin-10 levels could partially explain why, for example, in acute anterior uveitis, the inflammatory disease is often more severe.

Characterization of phenotype and cytokine profiles of T cell lines derived from vitreous humour in ocular inflammation in man.

Intermediate uveitis (IU) and Fuchs' heterochromic cyclitis (FHC) are two chronic ocular inflammatory disorders. They differ considerably in ocular morbidity, which is higher in IU. T cell lines were derived from the vitreous humour (VH) and peripheral blood (PB) of 10 patients with IU and four patients with FHC. There was a predominance of CD8+ in all the lines. However, there was a significantly higher percentage of CD4+ T cells in the T cell lines derived from VH of IU (32.0 +/- 8.6%) compared with FHC patients (19. 2 +/- 8.9%) (P = 0.04). The VH-derived T cell lines (VDTC) produced significantly higher levels of IL-2, interferon-gamma (IFN-gamma) and IL-10, but not IL-4, compared with PB-derived T cell lines (PBDTC) in both entities. There was significantly higher IL-2 production by VDTC from IU when compared with FHC patients (1810 +/- 220 pg/ml versus 518 +/- 94 pg/ml; P = 0.009), which could account for the more aggressive clinical features of this condition. In contrast IL-10 production was significantly higher by the VDTC from FHC compared with IU patients. The high IL-10 production by T cells infiltrating VH of FHC patients could down-regulate the inflammatory responses, thereby contributing to the benign clinical course seen in these patients. The accumulation of T cells with differing cytokine profiles in the VH suggests an important role for these cytokines in the pathogenesis of these chronic uveitides.

Characterization of T cells and cytokines in the aqueous humour (AH) in patients with Fuchs' heterochromic cyclitis (FHC) and idiopathic anterior uveitis (IAU).

FHC and IAU are two forms of anterior uveitis which are localized to the eyes with no evidence of systemic involvement. However, FHC has distinct clinical features and differs from IAU in that the inflammation is low grade, steroid non-responsive, and has a less aggressive clinical course. To try to dissect the mechanism for this difference the phenotypes of the cells in the AH and blood (PB) and the cytokines present in the AH in patients with FHC and IAU were compared. Three-colour flow cytometry was performed on the cells isolated from the AH and PB. Percentage of cells bearing the following markers were determined: CD3, CD4, CD8, CD4/CD25, CD8/CD25, CD19 and CD14. The cytokines IL-4, IL-10, IL-12 and interferon-gamma (IFN-gamma) were assayed by ELISA. In both groups T cell numbers were higher in the AH than PB, although the distribution of T cell subsets in PB was similar. In the AH, CD8+ T cell numbers were higher in FHC than in IAU (P = 0.003), whilst CD4+ numbers were higher in IAU than FHC (P = 0.01). AH cytokine profiles were different in the two groups: IFN-gamma levels were higher and IL-12 levels lower in the FHC group than IAU (P = 0.02), whilst IL-10 levels tended to be higher in the FHC group (P = 0.5). We suggest that different local mechanisms governing the balance of T cell/cytokine-mediated inflammation in the anterior segment may underlie clinical differences such as chronicity and response to steroids in these disorders.