AbstractThe uveitides are a heterogeneous group of diseases characterized by inflammation inside the eye. The uveitides are classified as infectious or non-infectious. The non-infectious uveitides, ...which are presumed to be immune mediated, can be further divided into those that are associated with a known systemic disease and those that are eye limited,—ie, not associated with a systemic disease. The ophthalmologist identifies the specific uveitic entity by medical history, clinical examination, and ocular imaging, as well as supplemental laboratory testing, if indicated. Treatment of the infectious uveitides is tailored to the particular infectious organism and may include regional and/or systemic medication. First line treatment for non-infectious uveitides is corticosteroids that can be administered topically, as regional injections or surgical implants, or systemically. Systemic immunosuppressive therapy is used in patients with severe disease who cannot tolerate corticosteroids, require chronic corticosteroids at >7.5 mg/day prednisone, or in whom the disease is known to respond better to immunosuppression. Management of many of these diseases is optimized by coordination between the ophthalmologist and rheumatologist or internist.
Vogt−Koyanagi−Harada disease Burkholder, Bryn M
Current opinion in ophthalmology,
2015-November, Letnik:
26, Številka:
6
Journal Article
PURPOSE OF REVIEWThe purpose of this article is to review the current literature on Vogt−Koyanagi−Harada (VKH) disease, including current treatment options and new research directions.
RECENT ...FINDINGSRecent publications on VKH disease show an increased focus on the immunogenetics and immune pathways associated with the development of VKH disease. There have also been advances in imaging modalities and techniques that may help to better elucidate the disease process in eyes with VKH disease.
SUMMARYVKH disease is an autoimmune, multisystem inflammatory disorder, the cause of which is still incompletely understood. Continued research may elucidate the causes and triggers of immune dysregulation in this disease, and in doing so, identify novel therapeutic targets.
Acquired hemophilia A (AHA) is a rare condition that may be drug-induced. In this case report, we describe a patient who presented with extensive subcutaneous bleeding three years after beginning ...treatment with adalimumab for necrotizing scleritis. His workup was compatible with drug-induced AHA. He was treated with high-dose corticosteroids, cyclophosphamide, and rituximab. Adalimumab was discontinued. We present this case as an example of a rare, but potentially life-threatening, complication of adalimumab.
To report a case of relapsing thrombotic thrombocytopenic purpura (TTP) in a patient treated with infliximab for chronic uveitis.
A 57-year-old African American woman with chronic anterior and ...intermediate uveitis, treated with infliximab for more than 1 year, presented with fatigue, dark colored urine, and ecchymosis on her extremities. She was diagnosed with thrombotic thrombocytopenic purpura (TTP) and recovered after treatment. After a remission period of 8 months, she was treated again with infliximab for recurrent intraocular inflammation. She developed a relapse of TTP 4 weeks after reintroducing infliximab.
Relapsing thrombotic thrombocytopenic purpura can be a rare complication associated with infliximab. To our knowledge, it has not been reported in the literature to date.
•Eighty-three percent of eyes with anterior scleritis treated with difluprednate achieved disease resolution.•Twenty-six percent of eyes treated with difluprednate had elevated intraocular pressure.
...To describe the effectiveness and side effect profile of difluprednate therapy in a series of patients with anterior scleritis.
Retrospective, interventional case series.
Data collected from all patients with anterior scleritis who used difluprednate as a single treatment agent from January 1, 2018, to January 1, 2020, including demographics, scleritis type, presence of nodules or necrosis, changes in scleritis activity, intraocular pressure (IOP), number of difluprednate drops used, best-corrected visual acuity (BCVA), and lens status. The primary outcome was clinical resolution of scleritis. Secondary outcomes included BCVA loss ≥2 lines, change in lens status or cataract surgery, and IOP ≥24 mm Hg.
Twenty-five patients (35 eyes) were analyzed. The median age was 60 years (range 13-78); 60% were female; 64% were White. Forty percent had bilateral disease, and 44% of patients had an associated systemic disease. The majority of eyes (66%) had diffuse anterior scleritis. Eighty-three percent of eyes achieved resolution of scleritis, with a median time of resolution of 6 weeks. Eyes treated with an initial dose of ≥4 times daily were more likely to achieve disease resolution (hazard ratio HR = 3.43, 95% confidence interval CI 1.19, 9.88, P = .02). Nine eyes had IOP elevation. Four eyes lost ≥2 lines of BCVA, and 1 due to cataract progression. One eye underwent cataract surgery.
Difluprednate alone may effectively treat non-infectious anterior scleritis with a tolerable side effect profile.
Autoimmunity and deficiency of the transcription factor autoimmune regulator protein (AIRE) are known associations with Down syndrome (DS). Lack of AIRE abrogates thymic tolerance. The autoimmune eye ...disease associated with DS has not been characterized. We identified a series of subjects with DS (n = 8) and uveitis. In three consecutive subjects, we tested the hypothesis that autoimmunity to retinal antigens might be a contributing factor.
This was a multicentred, retrospective case series. Deidentified clinical data of subjects with both DS and uveitis were collected via questionnaire by uveitis-trained ophthalmologists. Anti-retinal autoantibodies (AAbs) were detected using an Autoimmune Retinopathy Panel tested in the OHSU Ocular Immunology Laboratory.
We characterized eight subjects (mean age 29 range, 19-37 years). The mean age of detected uveitis onset was 23.5 range, 11-33 years. All eight subjects had bilateral uveitis (p < 0.001 based on comparison to published university referral patterns), with anterior and intermediate uveitis found in six and five subjects respectively. Each of three subjects tested for anti-retinal AAbs was positive. Detected AAbs included anti-carbonic anhydrase II, anti-enolase, anti-arrestin, and anti-aldolase.
A partial deficiency in the AIRE on chromosome 21 has been described in DS. The similarities in the uveitis presentations within this patient group, the known autoimmune disease predisposition in DS, the recognized association of DS and AIRE deficiency, the reported detection of anti-retinal antibodies in patients with DS in general, and the presence of anti-retinal AAbs in three subjects in our series supports a causal association between DS and autoimmune eye disease.
Purpose: To report the outcomes of the escalation of adalimumab (ADA) dose for refractory ocular inflammatory diseases.
Methods: A retrospective case series of 15 patients (29 eyes) diagnosed with ...ocular inflammatory disease, including uveitis and scleritis, which was not adequately controlled with standard, every other week ADA dosing, leading to an escalation to weekly dosing.
Results: Ten of fifteen patients escalated to weekly ADA achieved control of their inflammation; neither of the two patients increased for control of cystoid macular edema (CME) had resolution and required regional corticosteroids. One patient discontinued weekly ADA due to serious infection. The median length of follow up was 12 months.
Conclusion: Our series suggests that the escalation of ADA can be a useful strategy for treating recalcitrant ocular inflammation, but may not be adequate to treat refractory CME.
Purpose To describe the clinical outcomes of ocular syphilis. Design Retrospective chart review. Methods The charts of patients with ocular syphilis (regardless of human immunodeficiency virus HIV ...status) seen in a uveitis referral center between 1984 and 2014 were reviewed. Results The study included 35 patients (61 eyes). Panuveitis was the most common type of ocular inflammation (28 eyes), independent of HIV status. Thirty-three of 35 patients received systemic antibiotics with 24 patients treated with intravenous (IV) penicillin only. When compared to the HIV-positive patients, HIV-negative patients with ocular syphilis were older ( P < .001), were more likely to be female ( P = .004), and had poorer visual acuity at presentation ( P = .01). During follow-up, the incidence rates of visual impairment were 0.29 per eye-year (EY; 95% confidence interval CI: 0.06/EY-0.86/EY) and 0.12/EY (95% CI: 0.01/EY-0.42/EY) among the HIV-negative and the HIV-positive patients, respectively. The incidence of blindness was 0.07/EY (95% CI: 0.009/EY-0.27/EY) and 0.06/EY (95% CI: 0.002/EY-0.35/EY) among the HIV-negative and the HIV-positive patients, respectively. Longer duration of uveitis prior to diagnosis and chorioretinitis in the macula at presentation were associated with ≥2 Snellen lines of visual loss ( P < .01) and visual acuity loss to 20/50 or worse ( P = .03) in HIV-negative patients, respectively. Conclusions Syphilis is an uncommon cause of ocular inflammation in both HIV-negative and HIV-positive patients. Visual loss and ocular complications were common among HIV-negative patients even with systemic antibiotic treatment. Delay of diagnosis and chorioretinitis in the macula were associated with visual loss in these patients.
Abstract Purpose To identify determinants of adverse outcomes in acute retinal necrosis (ARN), presenting characteristics and incidence rates of vision loss and ocular complications in a cohort of ...polymerase chain reaction (PCR)-positive eyes were analyzed. Design Retrospective, observational cohort study. Methods Forty-one eyes of 36 patients with clinically-diagnosed ARN, PCR-positive for herpes simplex virus or varicella zoster virus and evaluated between January 2002 and June 2013, were included. Main outcome measures included incidence rates of vision loss and retinal detachment (RD). Results Presenting visual acuity was generally poor (20/50 to >20/200 in 27%; 20/200 or worse in 56%). The incidence rate of ≤20/200 was 0.66/eye-year (EY), (95% CI, 0.32/EY – 1.22/EY); the rate of light perception or no light perception vision was 0.07/EY (95% CI, 0.02/EY – 0.16/EY). During follow-up, 59% of eyes developed at least one RD (rate = 0.40/EY, 95% CI, 0.19/EY – 0.58/EY). Eyes with retinitis involving ≥25% of the retina at presentation detached at nearly 12 times the rate, as compared to those with <25% retinal involvement (0.70/EY vs. 0.06/EY; p=0.001). Development of an RD was the greatest determinant of adverse visual outcomes, with 4% of eyes, that had experienced at least one RD, achieving a best-corrected visual acuity of ≥20/40 compared to 53% of eyes that never detached (p=0.0003). Conclusions Poor outcomes in ARN were common in this cohort. RD confers the greatest risk of incident vision loss, and once 25% or more of the retina is involved the risk of RD and visual loss increases significantly.
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