Elevated serum IL-8 levels are associated with disease activity in idiopathic intermediate uveitis

AIM—To find a laboratory indicator for systemic involvement in intermediate uveitis.
METHODS—Interleukin 8 (IL-8) and C reactive protein (CRP) serum levels were measured in patients with idiopathic intermediate uveitis (n=61), uveitis controls (n=143), and normal controls (n=29). The records of those with intermediate uveitis were reviewed with the emphasis on disease activity and severity as characterised by the presence of cystoid macular oedema, vitreous exudates or snowbank formation, papillitis, and periphlebitis.
RESULTS—Increased serum IL-8 (20 pg/ml) was found in 27 out of 61 patients with intermediate uveitis (p< 0.01), 12 of 27 patients with sarcoid uveitis (p<0.05), in 19 of 30 patients with HLA-B27 associated acute anterior uveitis (p<0.05), and in five of 29 healthy controls. Raised IL-8 levels in intermediate uveitis were significantly associated with active disease (p<0.001) and the presence of vitreous exudates (p<0.001), papillitis, and periphlebitis (p<0.01). Elevated CRP levels were found in 12 of the 143 uveitis controls but in none of the intermediate uveitis patients or normal controls. During follow up an associated systemic disease was more frequently noticed in patients with an elevated serum IL-8 at entry into the study.
CONCLUSIONS—Elevated IL-8 serum levels were found in patients with active intermediate uveitis of unknown origin. An elevated IL-8 level seems to predispose the patient to a later development of associated systemic disease.

Keywords: C reactive protein; interleukin 8; uveitis; multiple sclerosis; sarcoidosis     

Cat-scratch disease: ocular manifestations and visual outcome

To describe the intra-ocular manifestations of cat-scratch disease (CSD) found at two uveitis reference centers in Brazil. Retrospective case series study. Review of clinical records of patients diagnosed with CSD in the Uveitis Department of São Geraldo Hospital and the Ophthalmology Department of the Instituto de Pesquisa Clínica Evandro Chagas—FIOCRUZ, from 2001 to 2008. In the 8-year period, 24 patients with the diagnosis of CSD were identified. Twelve patients were male and 12 female. The mean age was 27.04 years (range 7–56). Sixteen patients (66.6%) presented with a history of a cat scratch and all patients reported cat exposure. Visual acuity ranged from counting fingers to 1.0 in the affected eye. Thirteen patients presented with bilateral disease. Sixteen (66.6%) patients complained of systemic symptoms, including fever, lymphadenopathy, liver and spleen enlargement and rash. All patients presented with serum antibodies (IgG) to Bartonella henselae. Thirty-seven eyes were affected. The most common findings were small areas of retinal infiltrates which occurred in 11 eyes (29.7%) and angiomatous lesions which occurred in nine eyes (24.3%). Neuroretinitis occurred in only six eyes (16.2%). The most common findings of CSD in our study were retinal infiltrates and angiomatous lesions. CSD patients may present with significant visual loss. Patients may benefit from systemic treatment with antibiotics.     

Ocular Toxoplasmosis: Clinical Characteristics in Pediatric Patients

Purpose: To describe the clinical features of Ocular Toxoplasmosis in pediatric patients. Methods: A retrospective, non-comparative series of cases was studied. We reviewed the clinical records of patients 16 year old or younger diagnosed with Ocular Toxoplasmosis. Results: Forty patients (56 eyes) were included. The mean age was 9.5 yrs old. Twenty were female. Unilateral involvement was noticed in 60% of patients. The most common symptoms were strabismus (32.1%) and reduced VA in (23.2%). An inactive retinal scar was observed in most cases (71.4%). Panuveitis was found in 8 eyes (14.2%), and posterior uveitis in 7 eyes of 7 patients (12.5%); one eye presented neuroretinitis. The most frequent location of retinochoroidal lesions was the posterior pole (72.7%). Conclusions: In children, ocular toxoplasmosis is most commonly diagnosed during the inactive stage. When inflammation is present, it can be severe and frequently associated with other complications such vasculitis and papillitis.     

Tumor necrosis factor alpha blockade with infliximab for refractory uveitis and scleritis

OBJECTIVE: To assess the efficacy and safety of the anti-tumor necrosis factor alpha agent infliximab in treatment-resistant uveitis and scleritis. DESIGN: Retrospective, noncomparative interventional case series. PARTICIPANTS: Seven patients with noninfectious ocular inflammatory disease that was refractory to alternative immunosuppression. These included one patient with idiopathic retinal vasculitis and panuveitis, one patient with intermediate uveitis, one patient with chronic juvenile anterior uveitis, three patients with scleritis, and one patient with scleritis and peripheral ulcerative keratitis. Four patients had an underlying systemic disease that was in remission in three cases. INTERVENTION: Infusions of infliximab, 200 mg, were given at 4-week to 8-week intervals, depending on the clinical response. MAIN OUTCOME MEASURES: Clinical response, including symptoms, visual acuity, degree of scleral vascular engorgement, corneal thinning, anterior chamber activity, and posterior segment inflammation, reduction in concomitant immunosuppression, and adverse effects. RESULTS: The mean patient age was 47 years (range, 24-78), and four patients were female. The mean number of infliximab infusions was seven (range, 2-19), and the mean follow-up period was 12 months (range, 4-22 months). Six patients experienced a clinical improvement, with five achieving remission and significant reduction in immunosuppression. One patient showed an initial response but developed a delayed hypersensitivity response that precluded further treatment. No other adverse effects occurred. CONCLUSIONS: Infliximab seems to be an effective and safe treatment for noninfectious uveitis and scleritis and may be indicated as rescue therapy for relapses of ocular inflammation or as maintenance therapy when conventional immunosuppression has failed. Further investigation of infliximab for treatment-resistant scleritis and uveitis is warranted.     

Clinical course of ocular sarcoidosis in patients with histologically proven systemic sarcoidosis]

PURPOSE: We reviewed the clinical features, natural history and visual prognosis of 9 patients with histologically confirmed ocular and systemic sarcoidosis. PATIENTS: Nine patients underwent a follow-up study between 1993 and 1998. The diagnosis of sarcoidosis was supported by histological evidence of non caseating epithelioid-cell granuloma in tissue biopsy. RESULTS: The mean age was 40.912 years. The mean follow-up was 22.7 months (range 6 - 54 months). The ophthalmic involvement was bilateral in 8 patients. Before treatment, 3 eyes (17.6%) had a visual acuity less than 1/10. Two eyes (11.7%) had a visual acuity less than 3/10 and 10 eyes (58.8%) more than 6/10. The anterior uveitis was granulomatous in 2 eyes (11.7%) and non granulomatous in 8 eyes (47%). Five eyes (29.4%) had a pars planitis. The posterior segment manifestations of sarcoidosis were retinal vasculitis in 7 eyes (41.1%), papillitis in 10 eyes (58.8%) and choroidal granulomas in 2 eyes (11.7%). Seven patients were treated with systemic corticosteroids. One patient was treated with steroid eye-drops. After treatment, one eye (5.8%) had a visual acuity less than 1/10 and 13 eyes more than 6/10. After treatment, the choroidal granulomas disappeared and the intra-ocular inflammation was controlled in all cases. CONCLUSION: Although the number of patients in our series is small, the prognosis for ocular sarcoidosis appears to be good.     

Submacular exudates with serous retinal detachment caused by cat scratch disease

Purpose: To present submacular exudates as a manifestation of cat scratch disease. Methods: Report of two cases. Results: The first patient, a 34-year-old man, developed submacular exudates with serous retinal detachment ten days after having axillary lymphadenopathy and fever. The second patient, a 30-year-old woman, developed submacular exudates with serous retinal detachment mimicking central serous chorioretinopathy. Fluorescein angiography revealed late staining of the subretinal lesions in both cases.The lesion resolved spontaneously in the first patient, while sulfamethoxazole and trimethoprim was required for the second patient. Both patients had a positive IgG titer for Bartonella henselae. Conclusions: Submacular exudates with serous retinal detachment can occur in cat scratch disease. Cat scratch disease should be included in the differential diagnosis of submacular exudates with central serous chorioretinopathy.     

Presumed ocular bartonellosis

BACKGROUND: The spectrum of diseases caused by Bartonella henselae continues to expand and ocular involvement during this infection is being diagnosed with increasing frequency. METHODS: The clinical features and visual prognosis for 13 patients with intraocular inflammatory disease and laboratory evidence of bartonellosis were investigated. There were nine patients with neuroretinitis and four with panuveitis with positive antibody titres against B henselae determined by an enzyme immunoassay (IgG exceeding 1:900 and/or IgM exceeding 1:250). RESULTS: Positive IgG levels were found for eight patients and positive IgM levels for five. Despite animal exposure of 10 patients, only two (IgG positive) cases had systemic symptoms consistent with the diagnosis of cat scratch disease. Pathological fluorescein leakage of the optic disc was observed in all affected eyes. At 6 months' follow up, 3/18 (17%) affected eyes had a visual acuity of less than 20/100, owing to optic disc atrophy and cystoid macular oedema. 12 patients (17 eyes) were treated with antibiotics; visual acuity improved two or more Snellen lines for 9/17 (53%) eyes. CONCLUSIONS: The possibility of B henselae infection should be considered in patients with neuroretinitis and panuveitis (especially in cases with associated optic nerve involvement) even in the absence of systemic symptoms typical for cat scratch disease.     

102例少年儿童葡萄膜炎临床分析

目的 观察分析少年儿童葡萄膜炎的病程、病因及并发症.方法 回顾分析102例少年儿童葡萄膜炎患者的临床资料.患儿年龄2.5～16.0岁,平均年龄11.9岁.均进行常规视力、裂隙灯显微镜、检眼镜检查以及相关实验室检查.根据国际葡萄膜炎研究组制定的根据解削位置分类的标准对疾病进行诊断、分类.回顾分析时,对患者发病眼别、复发情况、炎症部位、病因、并发症等临床资料进行归纳比较.结果 102例葡萄膜炎患儿共170只眼受累,双眼受累68例,占66.6%.前葡萄膜炎38例,占37.3%;中间葡萄膜炎19例,占18.6%;后葡萄膜炎10例,占9.8%;全葡萄膜炎35例,占34.3%.发病至就诊时间为5 d～2.4年,平均发病时间3.6个月,治疗及随访时间为2周～10余年.病因前3位为幼年慢性关节炎、Vogt-Koyanagi Harada综合征、Behcet病.36例患儿发生并发症,包括并发性白内障19例,继发性青光眼7例,角膜带状变性5例,虹膜前后粘连12例,继发性视网膜脱离1例,眼球萎缩2例.因幼年慢性关节炎全身和局部使用糖皮质激素出现双侧股骨头坏死而行双髋关节置换1例.有10例患儿出现2种以上并发症.结论 少年儿童葡萄膜炎病因复杂多样,症状多不典型,疾病过程迁延慢长,易出现并发症导致盲目产生.     

Clinical Manifestations and Outcomes of Syphilis-associated Uveitis in Northern Spain

Purpose: To describe the clinical characteristics and to assess visual prognosis of patients with syphilis-associated uveitis in northern Spain.

Methods: Retrospective review of clinical records in eight general uveitis referral centers.

Results: Since the year 2000, 50 patients have been diagnosed: 31 men and 19 women; median age was 41 (19–76) years. A total of 34% were co-infected with HIV and 24% presented systemic manifestations of syphilis. Median initial visual acuity and vision at last visit in 93 affected eyes was 20/50 (20/20–20/2000) and 20/22 (20/20–20/2000), respectively (p?p?=?0.009).

Conclusions: In our series, patients with syphilitic uveitis were more usually middle-aged men and were frequently co-infected with HIV. Although most patients showed posterior segment involvement, visual prognosis was good.     

The expanding clinical spectrum of ocular lyme borreliosis

OBJECTIVE: To delineate the clinical manifestations of ocular Lyme borreliosis, while concentrating on new symptoms and findings and the phase of appearance of ophthalmologic disorders. DESIGN: Observational case series. PARTICIPANTS: Ten patients with Lyme borreliosis-associated ophthalmologic findings previously reported from the Helsinki University Central Hospital in addition to 10 new cases that have since been diagnosed. INTERVENTION/TESTING: The patients underwent medical and ophthalmologic evaluation. The diagnosis of Lyme borreliosis was based on medical history, clinical ocular and systemic findings, determinations of antibodies to Borrelia burgdorferi by enzyme-linked immunosorbent assay and immunoblot analysis, the detection of DNA of B. burgdorferi by polymerase chain reaction, and exclusion of other infectious and inflammatory causes. MAIN OUTCOME MEASURES: Ocular complaints, presenting ophthalmologic findings, and the stage of Lyme borreliosis were recorded. RESULTS: Four patients presented with a neuro-ophthalmologic disorder, five had external ocular inflammation, 10 patients had uveitis, and one had branch retinal vein occlusion. One patient developed episcleritis and one patient developed abducens palsy within 2 months of the infection incident. In the remaining 14 patients in whom the time of infection was traced, the ocular manifestations appeared in the late stage of Lyme borreliosis. Two patients with a neuro-ophthalmologic disorder and one with external ocular inflammation experienced severe photophobia, whereas the main reported symptom of the patients with uveitis was decreased visual acuity. Four patients with external ocular disease and one with a neuro-ophthalmologic disorder experienced severe periodic ocular or facial pain. Retinal vasculitis developed in seven patients with uveitis. CONCLUSIONS: Lyme borreliosis can cause a variety of ocular manifestations, which develop mainly in the late stage of the disease. Photophobia and severe periodic ocular pain can be characteristic symptoms of Lyme borreliosis. In the differential diagnosis of retinal vasculitis, Lyme borreliosis should be taken into account, especially in endemic areas.     

Soluble ICAM-1 serum levels in patients with intermediate uveitis

AIM: To investigate whether serum levels of soluble intercellular adhesion molecule 1 (sICAM-1) can serve as a marker of the presence of systemic disease in intermediate uveitis. METHODS: In a multicentre study sICAM-1 serum levels were measured in 61 patients with idiopathic intermediate uveitis, controls included 56 uveitis patients with a systemic disease (26 sarcoid associated uveitis and 30 HLA-B27 positive acute anterior uveitis), 58 uveitis patients without systemic disease (30 toxoplasma chorioretinitis and 28 Fuchs' hetrochromic cyclitis), and 21 normal controls. The clinical records of the patients with intermediate uveitis were analysed for disease characteristics at the time of blood sampling and for a relation with the development of a systemic disease after a mean follow up of 4.5 years. RESULTS: Increased serum levels of sICAM-1 were found in 34 out of 61 patients with intermediate uveitis and were significantly different when compared with toxoplasmosis, Fuchs' cyclitis, and healthy controls (p<0.001). Elevated sICAM-1 levels were also found in 18 out of 26 patients with sarcoid uveitis and in 11 out of 30 patients with HLA-B27 associated anterior uveitis. Raised sICAM-1 levels in the intermediate uveitis group were significantly associated with active ocular disease (p<0.01) and the presence of vitreous exudates (p<0.05). Increased levels of sICAM-1 correlated with interleukin 8 levels (IL-8) (tested in a previous study in the same group of intermediate uveitis patients) in patients with active systemic involvement. Follow up of the patients showed that an established or suspected systemic disease was found more often in the 21 intermediate uveitis patients with increased sICAM-1 and IL-8 levels compared with the other 40 patients with intermediate uveitis (p<0.01). CONCLUSIONS: The measurement of both sICAM-1 and IL-8 can be used as a marker for ocular disease activity and for a predisposition of developing an associated systemic disease in intermediate uveitis patients.     

Bilateral acute depigmentation of the iris

Purpose To report on five cases of unusual bilateral stromal depigmentation of the iris and pigment dispersion masquerading as uveitis.Methods We describe the clinical features of five consecutive patients who presented with acute depigmentation of the iris stroma between June and October 2003.Results Four patients were female, one was male. Age at presentation ranged from 15 to 25 years. Presenting symptoms were sudden-onset ocular discomfort and red eye in four patients and change of eye color in one patient. All patients had bilateral involvement, with a symmetrical diffuse depigmentation of the iris stroma in three cases and patchy areas of iris depigmentation in two. Other common features were mild ciliary injection (seven eyes), Krukenberg spindle (seven eyes), circulating pigment in the anterior chamber (eight eyes), and heavy pigment deposition in the angle (ten eyes). No eyes had iris transillumination defects, inflammatory keratic precipitates or inflammatory cells in the anterior chamber. Systemic laboratory work-up was unrewarding in all cases, and PCR analysis of the aqueous humor for HSV1 and 2 was negative in one patient. Four patients were treated with a short course of topical corticosteroids and three with oral acyclovir. One patient was lost to follow-up. The remaining four patients were followed up for 6–19 months with a stable clinical picture.Conclusion In contrast to pigment dispersion syndrome, pigment seemed to be released from iris stroma in the five cases described here. Although patchy depigmentation of the iris resembled the lesions seen in herpetic iridocyclitis in two of the patients, symmetrical bilateral involvement and lack of intraocular inflammation were the differentiating features. The patients described here could represent a new entity or an unusual presentation of herpetic eye disease.Presented at the American Uveitis Society Meeting, Ft. Lauderdale, Florida, 24 April 2004.     

Changing patterns of intraocular inflammatory disease in Japan

Purpose: We investigated the frequencies and clinical characteristics of Japanese patients with uveitis. Methods: Records of 189 patients referred from April 1999 to March 2001 were retrospectively reviewed. Results: Fifty-six patients (29.6%) had anterior uveitis, 13 (6.9%) intermediate uveitis, 59 (31.2%) posterior uveitis, 58 (30.7%) panuveitis, and three (1.6%) papillitis. The most common diagnoses were Vogt-Koyanagi-Harada (VKH) disease (10.1%), biopsy-proven or presumed sarcoidosis (9.5%), acute anterior uveitis (7.9%), tuberculosis (6.9%), and Behçet’s disease (5.8%). Seventy-three patients (38.6%) were treated with local therapy alone, and 95 patients (50.3%) required systemic therapy. Ocular complications developed in 19.6% of patients, and systemic complications in 2.1%. Conclusions: These results confirm a continued high frequency of VKH disease and sarcoidosis, but suggest a decreased frequency of Behçet’s disease and an increased frequency of tuberculosis. Roughly one-half of the patients required systemic treatment in addition to local therapy, and ocular and/or systemic complications developed in one-fifth of the patients.     

Clinical and angiographic characteristics of retinal manifestations in cat scratch disease

PURPOSE: To elucidate clinical and angiographic features of retinal manifestations in cat scratch disease. METHODS: Clinical characteristics as well as fluorescein and indocyanine green (ICG) angiographic features were reviewed in 4 consecutive patients with retinal manifestations caused by serologically confirmed cat scratch disease. RESULTS: A subretinal to intraretinal granuloma at the upper margin of the optic disc was found in 3 patients, while 1 patient developed subretinal to intraretinal granuloma in the midperiphery with serous retinal detachment. Fluorescein angiography revealed the abnormal vascular network of the peripapillary granuloma in the early phase followed by its dye leakage toward the late phase. Indocyanine green angiography demonstrated more clearly the abnormal vascular network with its minimal dye leakage than did fluorescein angiography. In contrast, only the late dye leakage was noted from granuloma of the midperipheral fundus by fluorescein angiography in one patient. Indocyanine green angiography detected no choroidal lesions other than the retinal lesions delineated by fluorescein angiography. The granulomas disappeared in response to a 4-week course of sulfamethoxazole-trimethoprim combined with steroids. CONCLUSIONS: Granuloma with abnormal vascular network as revealed by fluorescein and ICG angiography is characteristic of retinal manifestations in cat scratch disease.     

Pattern Visual Evoked Potentials in Eyes with Disc Swelling due to Cat Scratch Disease-associated Neuroretinitis

Purpose: To evaluate optic nerve function by pattern visual evoked potentials (VEPs) in eyes with optic disc swelling due to neuroretinitis associated with cat scratch disease (CSD). Methods: Four eyes of four patients with marked optic disc swelling resembling optic neuritis but diagnosed serologically as CSD received systemic steroid treatment. VEPs elicited by black and white checkerboard stimuli created on a TV monitor were recorded before the treatment. Results: The visual acuity (VA) in the affected eyes was decreased to 20/50 in two eyes and finger counting in two eyes at their initial visits. Ophthalmoscopic examination revealed neuroretinitis characterized by severe optic disc swelling, chorioretinal exudates, and macular edema in all eyes. Anti-Bartonella henselae serum antibody was markedly elevated in all patients confirming the diagnosis of CSD. The P100 of the transient VEPs was only mildly reduced without a delay in the implicit times in three eyes and only slightly delayed in the other eye. The steady-state VEPs were mildly reduced in two eyes and phase-reversed in other two eyes. The VA fully recovered after systemic steroid treatment in all patients. Conclusions: Although all examined patients showed marked swelling of the optic disc and visual decrease, the pattern VEPs were not affected as severely as in idiopathic optic neuritis. However, the degree of change of the pattern VEPs varied among patients.     

Clinical features and visual outcome of intermediate uveitis in children

PURPOSE: To determine the presentation, course, treatment and outcome of idiopathic intermediate uveitis in children. METHODS: The records of all patients under the age of 16 presenting with idiopathic intermediate uveitis under the care of one consultant (SL) between 1990 and 2001 were reviewed. The case notes were analysed with respect to presenting symptoms, associated conditions, ocular signs, change in visual acuity, treatment and complications. All patients with systemic disease were specifically excluded from the study. RESULTS: Twenty-six patients (45 eyes) were identified with intermediate uveitis from 114 cases of childhood uveitis seen. The mean age at presentation was 10 years (range 7-13 years) and the average follow-up time was 3 years (range 0-7 years). All patients had varying degrees of vitritis, 28 eyes had snowbanks and 5 had cystoid macular oedema at presentation. Six patients required no treatment, five received topical treatment only, five were managed with orbital floor steroids alone and a further two were managed with orbital floor steroids and oral prednisolone. Systemic steroids were required in eight patients, four of whom also required cyclosporin. Six of these children had successful disease control and maintained a visual acuity of 6/9 in at least one affected eye. CONCLUSION: Idiopathic intermediate uveitis is a relatively uncommon condition in children but can have severe effects on vision. With careful management, vision can be preserved in at least one eye. This study describes the course of the disease in a relatively large series. The variation in disease severity prompts the use of individually tailored immunosuppressive regimens.     

Pars plana vitrectomy as anti-inflammatory therapy for intermediate uveitis in children

PurposeIntermediate uveitis represents between 2 and 26% of uveitis in children. The spectrum of the disease is highly variable, ranging between mild cases that resolve spontaneously and chronic, severe forms that develop multiple episodes and complications. The purpose of this study is to evaluate the efficacy of vitrectomy to control inflammation in children with recurrent intermediate uveitis.MethodsRetrospective evaluation of patients with at least six months of follow-up. All patients under 16 who had undergone vitrectomy for intermediate uveitis were included. Vitrectomy was performed after at least two episodes of intermediate uveitis in children that had had no previous prophylactic systemic immunosuppressant treatment. Data recorded were visual acuity (VA), recurrences and surgical complications.ResultsSeven eyes of five children with intermediate uveitis who underwent vitrectomy were included. After a mean follow-up of 34 months, VA improved in all eyes after surgery. Four eyes developed mild subcapsular posterior cataracts. Post-surgical recurrences were anterior and responded to topical treatment, except for an episode of intermediate uveitis that required a periocular injection of triamcinolone. Only one patient is being treated with systemic immunosuppressants, due to the presence of repeated episodes of uveitis in the non-vitrectomised eye and since his parents were unwilling to have him undergo new surgery.ConclusionsVitrectomy with inferior cryotheraphy controls inflammation in intermediate uveitis in children with good mid-term results avoiding the secondary side-effects of systemic immunosuppressants.     

Demographic and Clinical Features of Pediatric Uveitis in Israel

ABSTRACT

Purpose: To report the epidemiology, etiology, ocular characteristics, treatment and visual outcome of pediatric uveitis in Israel.

Methods: Retrospective study from two tertiary uveitis centers.

Results: Included were 107 patients (182 eyes), 55% females. Mean age at diagnosis 8.8 years. Uveitis was predominantly anterior, idiopathic, bilateral, and chronic. Systemic associations were seen in 36% of patients of which the most common disease was juvenile idiopathic arthritis. Infectious uveitis accounted for 37% of posterior uveitis cases of which toxoplasmosis was the most common cause. Anterior segment complications were commonly observed at presentation (41%); the most predominant were posterior synechiae, cataract, and band keratopathy. The most common posterior segment complications were papillitis, epiretinal membrane, and macular atrophy/scar. Ninety-three percent of eyes had visual acuity >20/40 at last follow-up.

Conclusion: The pattern of pediatric uveitis in Israel is similar to that in the western world. Visual outcome was good in most eyes.     

Uveitis in childhood sarcoidosis

BACKGROUND: Sarcoidosis is a rare cause of uveitis in childhood. Differentiation from other rheumatic diseases may be difficult. We report on five children with uveitis in childhood sarcoidosis and review the previous literature. RESULTS: Children were aged between seven month and 13 years at the time of diagnosis of uveitis. Four of the five patients had panuveitis, the fifth one had intermediate uveitis. In addition, three children had systemic disease with skin, joint and central nervous system involvement. The two children younger than 5 years showed the clinical triad of eye, joint and skin disease. Uveitic complications included corneal band keratopathy, cataract, posterior synechiae, macular scars and choroidal neovascularisation. Vision-limiting complications were found in six of nine eyes at the first visit. CONCLUSION: In childhood sarcoidosis two clinical courses can be differentiated. Younger children aged up to five years express a clinical triad of arthritis, skin lesions and uveitis. In the older group of patients, the clinical course is more similar to that of adults. Rapid diagnosis and quiescence of disease are of particular importance to improve the long-term visual prognosis.     

Long-term treatment of refractory posterior uveitis with anti-TNFalpha (infliximab)

PURPOSE: To evaluate the long-term efficacy and safety of infliximab as treatment for noninfectious posterior uveitis. METHODS: An open-label clinical trial including seven patients (12 eyes) with posterior uveitis refractory to conventional treatment regimens with corticosteroids and at least one immunosuppressive agent. Three intravenous doses of 5 mg/kg of infliximab were administered at weeks 0, 2, and 6. Infliximab infusion was repeated in patients undergoing a relapse of uveitis after initial remission. Improvement was defined as amelioration of visual acuity or disappearance of retinal exudates and/or haemorrhages, decreased macular oedema and/or vitreous opacities. All patients were followed up for at least 36 months. RESULTS: Six of the seven patients (five diagnosed with Beh?et's disease and one diagnosed with sarcoidosis) showed a significant improvement after the first infliximab dose. Only in one patient diagnosed with chronic idiopathic multifocal choroiditis did the drug have no effect, and this patient was withdrawn from the study. At the end of follow-up, one eye had lost one line of vision and three eyes showed improved vision. All eyes had improved in terms of signs of inflammation. No adverse effects of treatment were observed. CONCLUSION: Infliximab is efficient and safe for the long-term management of refractory posterior uveitis, especially in patients with predominant retinal vasculitis and vitritis.