Search

Systemic vasculitides are a large group of heterogeneous diseases characterized by inflammatory destruction of blood vessels targeting diverse organs and tissues including the eye. As the most vascularized layer of the eye, the choroid is expected to be affected in multiple systemic rheumatologic diseases with vascular involvement. While there are plenty of studies investigating retinal vascular involvement, choroidal vascular involvement in systemic vasculitides has not been investigated in isolation. However, choroidal manifestations including thickness changes, choroidal vasculitis and ischemia may be the earliest diagnostic features of systemic vasculitic diseases. Thus, multimodal imaging of the choroid may help early detection of choroidal involvement which may also have prognostic implications in these life-threatening diseases. This article aimed to review involvement of the choroid in systemic vasculitic diseases. [ABSTRACT FROM AUTHOR]

Several proteins are investigated as candidate auto-antigens in the pathogenesis of Behçet’s disease (BD). This study aimed to screen the cellular responses to auto-antigen (αB-crystallin, αBC), and microorganism ( Streptococcus sangius KTH-1 BES-1 protein) derived peptides as well as to isopentenyl pyrophosphate (IPP). Peripheral blood mononuclear cells (PBMC) from 22 BD patients and 22 healthy controls (HC) were stimulated in vitro with αBC, BES-1 peptides, IPP and PPD and induced proliferation as well as the secreted interleukin (IL)-12 and interferon (IFN)-γ production levels were measured. We did not observe any significant difference in cellular proliferation between BD patients and HC. Induction of IL-12 secretion with αBC was stronger in BD patients ( P = 0.04) and in the disease subgroup with uveitis ( P = 0.027) compared the HC. When responses to PPD were compared, proliferation of PMBC was lower ( P = 0.03), whereas IL-12 secretion was higher in BD ( P = 0.04) as well as in patients under colchicum treatment ( P = 0.04) and with vascular involvement ( P = 0.006) compared to HC. BES-1373–385 peptide induced also higher IL-12 productions by PBMC of BD patients ( P = 0.017) and of patients with uveitis ( P = 0.013). Finally, IPP stimulated higher IL-12 secretions from PBMC in BD patients ( P = 0.035) and in patients with ( P = 0.02) or without ( P = 0.017) uveitis or arthritis ( P = 0.04), under colchicum treatment ( P = 0.01) or not receiving any immunosuppressive treatment ( P = 0.007) compared to HC. These results suggest a more prominent pro-inflammatory cytokine secretion from PBMC in BD patients compared to HC in response to various antigens including αBC protein, BES-1373–385, IPP and PPD. [ABSTRACT FROM AUTHOR]

Background: Multiple evanescent white dot syndrome (MEWDS) is a rare inflammatory eye condition affecting the outer retina as a consequence of choriocapillaris non perfusion. The pathophysiology of MEWDS will be discussed based clinical appraisal and on multimodal imaging appraisal. Methods: Narrative review and perspective opinion. Results: Literature review results helped us to put forward (1) the specific symptomatology (decreased/blurred vision, photopsia, subjective scotomas), (2) the ill-asserted character of clinical findings (foveal granularity, white dots in fundoscopy), (3) and the crucial importance of multimodal imaging with the diagnostic triad of ICGA hypofluorescent areas, BL-FAF hyperautofluorescent areas and loss/damage of IS/OS-ellipsoid zone on SD-OCT that characterise the disease and can practically help the clinician to diagnose MEWDS. A comprehensive alternative perspective of the disease was formulated. Conclusions: The bulk of evidence that we are presenting in this review, thanks to new performing non-invasive and invasive imaging modalities, is sufficiently compelling to consider MEWDS as a primary choriocapillaritis/inflammatory choriocapillaropathy. Multimodal imaging allows the clinician to diagnose MEWDS with a high level of certainty and ensures a precise follow-up. [ABSTRACT FROM AUTHOR]

Introduction: The EyeCOPE study characterized noninfectious intermediate posterior, or panuveitis (NIIPPU) before biologic agents were widely available. Methods: This retrospective, observational study included adults with NIIPPU attending a routine ophthalmological visit. Data were collected from the study visit and medical records. Results: Of 565 patients, 58.8% were female, and the mean age was 41.3 years; 33.8% had idiopathic uveitis and 45.8% had panuveitis. The median time from symptom onset to diagnosis and treatment was 27.0 and 30.5 days, respectively. Patients received immunosuppressants and systemic/local corticosteroids. Most patients experienced substantial decline in ocular function (mean best corrected visual acuity, 0.4 logMAR). Mean total work productivity impairment among employed patients was 31.0%. Most patients reported ocular complications (70.8%) such as vision loss and cataracts. Conclusions: Despite treatment, most patients with NIIPPU experienced a decline in ocular function and ocular complications. There is an unmet need for additional NIIPPU treatment, such as targeted monoclonal antibodies. [ABSTRACT FROM AUTHOR]

Purpose: The purpose of this study was to investigate the safety and effectiveness of an intravitreally injected dexamethasone-containing implant (Ozurdex) in the treatment of uveitis in children. Methods: The study group included ten patients (14 eyes) aged 6.5-15 years (mean age 12 years) with intermediate or posterior uveitis who were treated with the Ozurdex implant at two tertiary medical centers between 2009 and 2014, following an insufficient response to standard uveitis therapy. All were followed for at least 6 months (mean 12.2 ± 4.9 months). Clinical data before and after treatment were collected retrospectively from the medical files. Outcome measures were best corrected visual acuity, vitreous haze, and macular thickness. Ocular complications were documented. Results: Visual acuity improved in 12 eyes (86 %) and intraocular inflammation decreased in 13 eyes (93 %) from 1 week to 3 months after the first injection. Macular edema decreased in all eyes from 1 month to 3 months after the first injection. Five patients underwent repeated injections because of an increase in macular thickness at 3-6 month follow-up. Complications included cataract progression in one patient after one injection and cataract formation in two patients after two injections, and an elevation in intraocular pressure in two patients who responded well to topical treatment. Conclusions: Both single and repeated injections of a dexamethasone-containing implant are safe and effective for the treatment of noninfectious intermediate and posterior uveitis in children. The duration of the beneficial effect may be limited. More data are required to establish the safety profile of the implant in the pediatric age group. [ABSTRACT FROM AUTHOR]

Background: Recombinant human (rh) interferon alpha2a (IFN-α2a) therapy is successfully used for the treatment of Behçet's disease (BD) uveitis refractory to conventional immunosuppressive treatment. Purpose: Our aim in this study was to investigate the frequency and clinical significance of anti-IFN-α antibodies and autoantibodies during recombinant human rhIFN-α2a therapy in patients with BD uveitis. Methods: This comparative, cross-sectional, serological screening study included 30 BD patients treated with rhIFN-α2a (Group 1), 29 BD patients treated with conventional immunosuppressive agents (Group 2), 29 BD patients who received only colchicine (Group 3), and 30 healthy subjects (Group 4). Anti-IFN-α-binding antibodies and autoantibodies, including anti-nuclear antibody, anti-thyroid peroxidase antibody, and anti-cardiolipin antibody, were measured in serum samples. Antibody seropositivity was compared between study groups. Retrospective clinical data were compared between antibody-positive and antibody-negative patients. Results: A significantly higher proportion of patients in Group 1 had anti-interferon-α (26.6 %) and autoantibody (30 %) seropositivity compared to the other groups. No correlation was found between seropositivity for anti-interferon-α and other autoantibodies. No significant difference was found in cumulative dose of IFN-α, duration of IFN-α therapy, time to first uveitis attack, or attack rate between anti-interferon-α antibody-positive and antibody-negative patients in Group 1. Uveitis attacks were observed in 22 % of autoantibody-positive and 71 % of autoantibody-negative patients in Group 1 ( p = 0.018). Conclusions: Patients with BD uveitis develop anti-IFN-α-binding antibodies and autoantibodies during treatment with rhIFN-α2a. While the clinical relevance of anti-IFN-α-binding antibodies remains unclear in this study, induction of autoimmunity was found to be associated with a tendency for better therapeutic response. [ABSTRACT FROM AUTHOR]

Individuals with Behçet's disease suffer from episodic inflammation often affecting the orogenital mucosa, skin and eyes. To discover new susceptibility loci for Behçet's disease, we performed a genome-wide association study (GWAS) of 779,465 SNPs with imputed genotypes in 1,209 Turkish individuals with Behçet's disease and 1,278 controls. We identified new associations at CCR1, STAT4 and KLRC4. Additionally, two SNPs in ERAP1, encoding ERAP1 p.Asp575Asn and p.Arg725Gln alterations, recessively conferred disease risk. These findings were replicated in 1,468 independent Turkish and/or 1,352 Japanese samples (combined meta-analysis P < 2 × 10?9). We also found evidence for interaction between HLA-B*51 and ERAP1 (P = 9 × 10?4). The CCR1 and STAT4 variants were associated with gene expression differences. Three risk loci shared with ankylosing spondylitis and psoriasis (the MHC class I region, ERAP1 and IL23R and the MHC class I-ERAP1 interaction), as well as two loci shared with inflammatory bowel disease (IL23R and IL10) implicate shared pathogenic pathways in the spondyloarthritides and Behçet's disease. [ABSTRACT FROM AUTHOR]

Behçet's disease is a genetically complex disease of unknown etiology characterized by recurrent inflammatory attacks affecting the orogenital mucosa, eyes and skin. We performed a genome-wide association study with 311,459 SNPs in 1,215 individuals with Behçet's disease (cases) and 1,278 healthy controls from Turkey. We confirmed the known association of Behçet's disease with HLA-B*51 and identified a second, independent association within the MHC Class I region. We also identified an association at IL10 (rs1518111, P = 1.88 × 10−8). Using a meta-analysis with an additional five cohorts from Turkey, the Middle East, Europe and Asia, comprising a total of 2,430 cases and 2,660 controls, we identified associations at IL10 (rs1518111, P = 3.54 × 10−18, odds ratio = 1.45, 95% CI 1.34–1.58) and the IL23R-IL12RB2 locus (rs924080, P = 6.69 × 10−9, OR = 1.28, 95% CI 1.18–1.39). The disease-associated IL10 variant (the rs1518111 A allele) was associated with diminished mRNA expression and low protein production. [ABSTRACT FROM AUTHOR]

Uveitis is an important component of many rheumatic diseases. The main causes of recurrent uveitis are seronegative spondylarthropathies and Behçet’s disease. We describe a rare case of Henoch Schönlein vasculitis (HSV) along with multiple recurrences of acute anterior uveitis. In cases of skin rash and recurrent anterior uveitis, HSV should be considered in the differential diagnosis. [ABSTRACT FROM AUTHOR]