Intermediate Uveitis and Alopecia Areata:Is There a Relationship? Report of 3 Pediatric Cases

Three previously healthy children, aged 5, 8, and 15 years, with idiopathic intermediate uveitis (IU) and alopecia areata (AA) are described. These are the first 3 cases of which we are aware with this coexistence. The results of extensive diagnostic evaluations were negative in all 3 cases. AA preceded the diagnosis of bilateral IU in 1 child and followed within several months after IU diagnosis in 2 children. The severity of uveitis ranged from mild to sight-threatening, and hair loss ranged from local lesions in 2 cases to total alopecia in 1 case. Pathogenesis of both diseases is discussed. Theoretically, the coexistence of IU and AA might be based on the similarities in their complex pathogenesis. However, more research is needed to evaluate if the coexistence is based on an association between 2 autoimmune disorders or is a coincidence. Pediatrics 2011;128:e1013-e101

Infiltration of Plasma Cells in the Iris of Children With ANA-Positive Anterior Uveitis

PURPOSE: We investigated inflammatory cell infiltrates in iris biopsies in uveitis associated with juvenile idiopathic arthritis (JIA) in comparison with other pediatric uveitis entities and noninflammatory pediatric controls. METHODS: Iridectomy specimens were obtained during elective trabeculectomy from 31 eyes of 25 patients: 12 eyes with JIA-associated uveitis, 13 eyes with other uveitis entities, and 6 eyes with open angle nonuveitic juvenile glaucoma. Histopathologic and immunohistochemical analyses were performed. A semiquantitative scoring system was used with a scale ranging from 0 to 4 depending on the number of stained cells. RESULTS: An inflammatory infiltrate was present in 8/12 (67%) specimens with JIA-associated uveitis. The cellular infiltrate in JIA specimens was characterized by the presence of CD138+ plasma cells and CD68+ macrophages, while the presence of CD20+, CD4+, and CD8+ cells was variable. Presence of plasma cells in the inflammatory infiltrates in anterior uveitis correlated with antinuclear autoantibody (ANA) positivity regardless of the diagnosis of JIA. CD4+ and CD8+ T cells were not always detectable in the iris biopsies of all childhood uveitis patients, although a slight predominance of CD4+ cells was noted. CONCLUSIONS: Children with ANA-positive anterior uveitis often show an infiltrate of plasma cells, regardless of the diagnosis of JIA. The iris of JIA-associated uveitis patients is additionally characterized by the presence of various numbers of macrophages

Uveitis Associated with Juvenile Idiopathic Arthritis

Juvenile idiopathic arthritis (JIA) is the most common cause of uveitis in children. While symptoms are usually mild, persistent eye inflammation could lead to severe complications and impaired vision. It is essential that JIA patients at risk are diagnosed with uveitis early, receive adequate treatment, and avoid developing complications, such as cataract, glaucoma, and amblyopia. The purpose of this mini-review is to summarize the screening strategies and clinical management for JIA-associated uveitis (JIA-U) as well as the current state of molecular markers linked to this condition. Because glaucoma is one of the most common causes of visual loss in JIA-U, special focus will be put on this serious complication. We conclude by describing the current evidence regarding the long-standing question of whether chronic anterior uveitis without arthritis may be the same disease entity as JIA-U.</p

The clinical course of juvenile idiopathic arthritis-associated uveitis in childhood and puberty

Aim The long-term course of juvenile idiopathic arthritis (JIA)-associated uveitis is not known yet. This study investigates the course and activity of JIA-associated uveitis in childhood and puberty. Design Retrospective study of the clinical data of 62 JIA patients with uveitis. The main outcome measurements consisted of uveitis activity measured as mean cell grade in the anterior chamber, topical and systemic medication and ocular complications related to disease activity. All data were scored and evaluated per year of age. Results Uveitis activity took a biphasic course with a quiet phase around the age of 9 years and showed increased activity during early teenage years. The biphasic course was significantly related to age (p=0.048) but not to uveitis duration. More patients were treated with systemic immunosuppressive medication in estimated puberty years (63% in boys, 53% in girls) compared with prepuberty years (46% and 28%, respectively), although the difference was only significant in girls (p Conclusions JIA-associated uveitis appears to take a biphasic course with the second phase of activity during early teenage years and more treatment with systemic immunosuppressive medication occurred during estimated puberty compared with prepuberty years

Ocular complications in children within 1 year after hematopoietic stem cell transplantation

Importance: It is essential to have insights into the risk of ocular involvement after hematopoietic stem cell transplantation (HSCT) in the pediatric population because young and severely ill children are unaware of their ocular problems. Objective: To study the development of ocular complications in children within 1 year after HSCT. Design and Setting: This prospective study includes all consecutive patients who had undergone an HSCT at the Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, the Netherlands, in 2009 and 2010. Participants: Forty-nine consecutive patients underwent systematic ophthalmologic evaluations before HSCT, before leaving the HSCT unit after HSCT, and 3, 6, and 12 months after HSCT. Additional examinations were performed during systemic viral reactivations. Main Outcome Measure: Development of ocular complications, including uveitis, hemorrhagic complications, optic disc edema, and dry eye syndrome. Results: Thirteen patients (27%) developed an ocular complication after HSCT. These complications included DES (n=7 [14%]), (sub)retinal hemorrhage (n=6 [12%]), optic disc edema (n=3 [6%]), chorioretinal lesions (n=2 [4%]), vitritis (n=1 [2%]), and increased intraocular pressure (n=1 [2%]). Median time to the development of dry eye syndrome was 5 months after HSCT, whereas all other ocular complications were detected within the first 3months after HSCT. In most cases, the symptoms were mild and self-limiting. Children with malignant disease had a higher risk of the development of ocular complications compared with children with nonmalignant disease. Conclusions and Relevance: Ocular complications in pediatric HSCT patients are common, although mostly mild. The risk of viral uveitis development during systemic viral reactivations is low; however, the potential risk of vision-threatening complications in this population cannot be ruled out. Copyrigh

Pathogenesis of juvenile idiopathic arthritis associated uveitis: the known and unknown

Juvenile idiopathic arthritis (JIA) is the most common childhood rheumatic disease and the most prevalent systemic disorder in children with uveitis. The current prevailing opinion is that JIA is a multifactorial, genetically predisposed autoimmune disorder that can be influenced by environmental factors and infections; the specific pathogenesis of JIA-associated uveitis is not understood, however, nor has the relationship between the eye and joint inflammation been established. Nevertheless, subtypes of JIA that are associated with uveitis, oligoarthritis, polyarticular rheumatoid factor negative, and psoriatic arthritis appear to have common pathogenicity. We summarize our current knowledge regarding the pathogenesis of JIA-associated uveitis and discuss the possible role of immune responses and cytokine involvement, genetic associations, and the influence of external triggers in this disease-an association that is supported by data obtained from arthritis research and experimental uveitis models. (C) 2014 Elsevier Inc. All rights reserved

Current practice in the management of ocular toxoplasmosis

Background Ocular toxoplasmosis is common across all regions of the world. Understanding of the epidemiology and approach to diagnosis and treatment have evolved recently. In November 2020, an international group of uveitis-specialised ophthalmologists formed the International Ocular Toxoplasmosis Study Group to define current practice. Methods 192 Study Group members from 48 countries completed a 36-item survey on clinical features, use of investigations, indications for treatment, systemic and intravitreal treatment with antiparasitic drugs and corticosteroids, and approach to follow-up and preventive therapy. Results For 77.1% of members, unilateral retinochoroiditis adjacent to a pigmented scar accounted for over 60% of presentations, but diverse atypical presentations were also reported. Common complications included persistent vitreous opacities, epiretinal membrane, cataract, and ocular hypertension or glaucoma. Most members used clinical examination with (56.8%) or without (35.9%) serology to diagnose typical disease but relied on intraocular fluid testing-usually PCR-in atypical cases (68.8%). 66.1% of members treated all non-pregnant patients, while 33.9% treated selected patients. Oral trimethoprim-sulfamethoxazole was first-line therapy for 66.7% of members, and 60.9% had experience using intravitreal clindamycin. Corticosteroid drugs were administered systemically by 97.4%; 24.7% also injected corticosteroid intravitreally, almost always in combination with an antimicrobial drug (72.3%). The majority of members followed up all (60.4%) or selected (35.9%) patients after resolution of acute disease, and prophylaxis against recurrence with trimethoprim-sulfamethoxazole was prescribed to selected patients by 69.8%. Conclusion Our report presents a current management approach for ocular toxoplasmosis, as practised by a large international group of uveitis-specialised ophthalmologists