Behcet syndrome (BS) is a chronic systemic inflammatory disorder involving vessels of all sizes, characterized by relapsing episodes of oral and/or genital ulcers, as well as skin lesions. Ocular, vascular, gastrointestinal, neurological system involvement can cause significant morbidity and mortality. Glucocorticoids and immunosuppressants are the cornerstones for the management of BS. Biologic agents has been recommended for severe and/or refractory BS. Interferon-α (IFN-α) had multiple biological effects, such as antiviral and antiproliferative, that could regulate both innate and adaptive immunity in BS. Growing evidence showed the efficacy of IFN-α in severe and/or refractory BS. Many studies have demonstrated that IFN-α has comparable effectiveness and tolerance profiles as anti-tumor necrosis factor (TNF) agents for Behcet's uveitis with a much lower cost and steroid-and immunosuppressant-sparing effects. IFN-α has been recommended as second-line therapy for ocular involvement of BS in EULAR (The European League Against Rheumatism) 2018. IFN-α also improves mucocutaneous lesions in BS with the dosage from 3 to 9-12 million IU three times per week. A few cases indicated the therapeutic potential of IFN-α in intestinal BS. As a new trial of IFN-α in vascular BS (VBS), a recent study revealed the lower relapse rate and higher recanalization rate with IFN-α in lower extremity deep vein thrombosis (DVT). Another two case reports presented the efficacy of IFN-α in pulmonary artery involvement in BS. Also, case reports have shown successful treatment in refractory neurological involvement. There are two subtypes of IFN-α commonly used in autoimmune diseases, named IFN-α2a and IFN-α2b. IFN-α2a seemed more effective than IFN-α2b, especially in ocular and mucocutaneous involvement of BS. Side effects of IFN-α are dose-dependent and not severe. The most frequent side effects are flu-like syndrome, mild leukopenia and alopecia. Considering the potential risk of tuberculosis (TB) and hepatitis B virus (HBV) reactivation of TNF-α inhibitors, IFN-α is safe due to its anti-HBV effect and protective effect on TB. In conclusion, IFN-α is a promising choice for severe and/or refractory BS patients, especially for those who are intolerant or contraindicant to other biological agents, such as TNF inhibitors. Further prospective controlled studies are warranted to confirm the efficacy and safety of IFN-α in BS.
Behcet Syndrome/drug therapy* ; Glucocorticoids ; Humans ; Immunosuppressive Agents ; Uveitis ; Venous Thrombosis

PURPOSE: To report a case of multicentric Castleman disease that presented with scleritis. CASE SUMMARY: A 42-year-old male presented with decreased visual acuity in the left eye. Castleman disease had been diagnosed 21 months before and treated with systemic steroids and combined chemotherapy. Best-corrected visual acuity (BCVA) of the left eye was 0.02 and the intraocular pressure was 42 mmHg. Scleral edema and corneal edema were noted using a slit lamp examination. The anterior chamber cell was 2+ according to Standardization of Uveitis Nomenclature criteria. The fundus was invisible due to the anterior segment lesion. After one month, scleritis developed in the right eye and the patient complained of ocular pain. Topical steroids and non-steroidal anti-inflammatory drugs were prescribed. Due to recurrent scleritis and anterior uveitis, cataract extraction and laser iridectomy were performed on the left eye, and systemic steroids and the antimetabolite methotrexate were started. After 9 years of follow-up, all medications were stopped and there was no recurrence of inflammation, with a BCVA of 1.0 in both eyes. CONCLUSIONS: Treatment of a patient with scleritis accompanied with Castleman disease using systemic steroids and methotrexate resulted in a good prognosis.
Adult ; Anterior Chamber ; Cataract Extraction ; Corneal Edema ; Drug Therapy ; Edema ; Follow-Up Studies ; Giant Lymph Node Hyperplasia ; Humans ; Inflammation ; Intraocular Pressure ; Iridectomy ; Male ; Methotrexate ; Prognosis ; Recurrence ; Scleritis ; Slit Lamp ; Steroids ; Uveitis ; Uveitis, Anterior ; Visual Acuity

BACKGROUND: The present study describes our 10-year experience with uveoretinal adverse events that manifest because of chemotherapy. METHODS: A retrospective chart review was performed for all patients who presented to the ophthalmologic department while undergoing systemic chemotherapy between July 2005 and June 2015. RESULTS: A total of 55 patients (mean age, 51.2 years, 38 women [69.1%]) suspected of having uveoretinal disease owing to the use of chemotherapeutic agents alone were enrolled. Breast cancer was the predominant disease (36.4%); noninfectious anterior uveitis (21.8%) was the most common condition. Bilateral involvement was observed in 16 patients (29.1%). Although cisplatin (21.8%) was the most commonly used drug, daunorubicin, cytarabine, tamoxifen, toremifene, and imatinib were also frequently used. The median duration until ophthalmologic diagnosis was 208.5 days (range, 19–5,945 days). The proportion of patients with final visual acuity (VA) < 20/40 Snellen VA (0.5 decimal VA) was 32.7%. However, no relationship was observed between final VA < 20/40 and age, sex, therapeutic agents, and metastasis. CONCLUSION: Uveoretinal complications were mostly mild to moderate and exhibited a favorable response to conservative therapy. A considerable number of patients exhibited significant irreversible loss of vision after cessation of the causative chemotherapeutic agent. Ophthalmological monitoring is required during chemotherapy.
Antineoplastic Agents ; Breast Neoplasms ; Cisplatin ; Cytarabine ; Daunorubicin ; Diagnosis ; Drug Therapy ; Drug-Related Side Effects and Adverse Reactions ; Female ; Humans ; Imatinib Mesylate ; Molecular Targeted Therapy ; Neoplasm Metastasis ; Retrospective Studies ; Tamoxifen ; Toremifene ; Uveitis ; Uveitis, Anterior ; Visual Acuity

PURPOSE: To report the clinical characteristics of retinoblastoma patients whose diagnosis was difficult due to atypical ocular manifestations. METHODS: Among retinoblastoma patients who were diagnosed and treated from January 1999 to December 2014 at Seoul National University Children's Hospital, 6 patients whose diagnosis was difficult were retrospectively reviewed. Factors including age, sex, family history, initial findings, time to final diagnosis, histopathologic examination, additional treatment, and survival rate were evaluated. RESULTS: Among 6 patients, 5 were male, and the mean age at the initial visit was 32.9 ± 19.1 months. None of the patients had family history, and all presented with unilateral lesion at the initial visit. The initial diagnoses were Coats' disease and uveitis in 2 patients, respectively, and persistent hyperplastic primary vitreous and traumatic hyphema in 1 patient, respectively. During an intensive short-term follow-up of 8.3 ± 5.3 weeks, 2 patients showed malignant cells after external subretinal fluid drainage procedure, and 4 patients demonstrated increasing ocular size or calcification in imaging. These patients received enucleation under suspicion of malignancy and were finally diagnosed with retinoblastoma after histopathologic examination. There were 2 patients with optic nerve involvement, and 3 patients underwent additional systemic chemotherapy. Five patients were followed-up for 7.6 ± 6.3 years after enucleation, and the mean age at final follow-up was 10.6 ± 7.4 years. CONCLUSIONS: Retinoblastoma is one of the diseases in which early diagnosis and treatment are important. However, some cases are difficult to diagnose, even for experienced clinicians. If there are no typical manifestations such as mass or calcification and early findings show retinal detachment, glaucoma, pseudohypopyon, or hyphema, intensive short-term follow-up to exclude retinoblastoma is needed.
Diagnosis* ; Drainage ; Drug Therapy ; Early Diagnosis ; Follow-Up Studies ; Glaucoma ; Humans ; Hyphema ; Male ; Optic Nerve ; Persistent Hyperplastic Primary Vitreous ; Retinal Detachment ; Retinoblastoma* ; Retrospective Studies ; Seoul ; Subretinal Fluid ; Survival Rate ; Uveitis

OBJECTIVETo study the influence of Yanyankang powder on Th1/Th2 in rats with experimental autoimmune uveitis (EAU).

METHODSThe EAU models were induced in Lewis rats by immunization with interphotoreceptor retinoid binding protein (IRBP) 1177-1191 in complete Freund's adjuvant. The rats were randomly divided into 3 groups: a model control group, a Yanyankang group, and a prednisone group, 9 rats in each group. The model control group was intervened with saline solution by gavage. The Yanyankang group was intervened with Yanyankang powder 4 g/(kg day) by gavage. The prednisone group were intervened with prednisone acetate tablets 5 mg/(kg d) by gavage. All groups were intervened after immunization once every 2 days for 18 days and monitored by slit-lamp biomicroscopy daily until day 18. The levels of gamma interferon (INF-γ) and interleukin-10 (IL-10) in the supernatants of T cells were determined by enzyme-linked immunosorbent assay. Polymerase chain reaction (PCR) technology was used for measuring Th1 and Th2 related cytokine mRNA expressions.

RESULTSSlighter intraocular inflammation was found in the Yanyankang group and the prednisone group than the control group. The levels of the IFN-γ and IL-10 in the supernatants of the spleen lymph node cells were 382.33±6.30, 155.87±4.46 μg/L in the Yanyankang group and 270.93±7.76, 265.32±11.88 μg/L in the prednisone group. Both had significant differences compared with the control group (941.53±8.59, 20.67±4.65 μg/L; =0.01). The PCR results showed the same tendency.

CONCLUSIONYanyankang powder showed favorable effects in the rats with EAU by influencing the function of Th1 and Th2 cells.

Animals ; Drugs, Chinese Herbal ; pharmacology ; therapeutic use ; Eye ; pathology ; Female ; Immunization ; Inflammation ; pathology ; Interferon-gamma ; genetics ; metabolism ; Interleukin-10 ; genetics ; metabolism ; Lymph Nodes ; drug effects ; metabolism ; Powders ; RNA, Messenger ; genetics ; metabolism ; Rats, Inbred Lew ; Spleen ; metabolism ; Th1 Cells ; drug effects ; immunology ; Th2 Cells ; drug effects ; immunology ; Uveitis ; drug therapy ; genetics ; immunology

Behcet's disease (BD) involves multisystem vasculitis of unknown origin. Ocular manifestations of BD mostly include bilateral panuveitis and retinal vasculitis, which are very challenging to treat. Interferon alfa-2a (IFN) has been recently introduced for treating refractory Behcet uveitis, mainly in Germany and Turkey. Nonetheless, there is so far no consensus about the ideal treatment regimen of IFN for Behcet uveitis. We report our experience of IFN treatment in five Korean BD patients with refractory uveitis. All patients complained of oral ulcers; one patient had a positive pathergy test and 2 showed the presence of HLA-B51. Immunosuppressive agents used prior to IFN treatment included cyclosporine and methotrexate. The IFN treatment was commenced with a dose of 6-9 MIU/day for 7 days, adjusted according to individual ocular manifestations, tapered down to 3 MIU three times in a week, and then discontinued. All patients showed positive response to IFN treatment; 50% of them showed complete response without additional major ocular inflammation during the follow-up period. Other BD symptoms also improved after IFN treatment in most cases. After treatment, the relapse rate and the required dose of oral corticosteroid were decreased in most cases, showing a significant steroid-sparing effect. However, the visual acuity was not improved in most cases due to irreversible macular sequelae. Despite the small sample size of this study, we suggest that, in Korean patients, IFN is an effective treatment modality for BD uveitis as was observed in German and Turkish patients.
Adult ; Behcet Syndrome/complications/diagnosis/*drug therapy ; Chronic Disease ; Cyclosporine/therapeutic use ; Female ; Humans ; Immunosuppressive Agents/administration & dosage ; Interferon-alpha/*therapeutic use ; Male ; Recombinant Proteins/therapeutic use ; Recurrence ; Remission Induction ; Treatment Outcome ; Turkey ; Uveitis/diagnosis/*drug therapy/etiology ; Visual Acuity

Behcet's disease is an inflammatory disorder characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesions. A few cases of hematologic disease in patients with Behcet's disease have been reported in the literature. However, acute precursor T cell lymphoblastic leukemia has never been described in association with Behcet's disease. We recently encountered a case of acute precursor T cell lymphoblastic leukemia in a 62-year-old man with a prior diagnosis of Behcet's disease. The patient presented with febrile neutropenia and his bone marrow biopsy revealed acute precursor T cell lymphoblastic leukemia. He was scheduled to undergo therapeutic chemotherapy, but unfortunately he died from pneumonia prior to treatment.
Biopsy ; Bone Marrow ; Diagnosis ; Drug Therapy ; Febrile Neutropenia ; Hematologic Diseases ; Humans ; Middle Aged ; Pneumonia ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma* ; Skin ; Stomatitis, Aphthous ; Ulcer ; Uveitis

The authors wish to report a case of bilateral acute anterior uveitis and optic disc edema following a hemotoxic snake bite, in order to highlight the concomitant occurrence of these conditions and the potential adverse effects of anti-snake venom (ASV). A 35-year-old male was bitten by a viper at seventeen thirty hours, and was started on ASV. Two days following treatment he experienced sudden onset redness and painful diminution of vision in both eyes (OU). On examination, the patient's visual acuity (VA) in OU was 20/200. Examination revealed fresh keratic precipitates, cells, and flare in the anterior chamber (AC), posterior synechiae, sluggish and ill-sustained pupillary reaction, and hyperemic, edematous disc with blurred margins in OU. He was started on topical steroids, cycloplegics and intravenous methylprednisolone. Following treatment, the patient showed improvement and was continued on topical medications and oral prednisolone tapered over 3 weeks, after which VA OU improved, the AC showed no cells and flare and disc edema resolved. Uveitis and optic disc edema in snake bite can either be due to the direct toxic effects of the venom or the effect of ASV. Steroids have a beneficial role in the management of these symptoms.
Acute Disease ; Adult ; Antivenins/*adverse effects ; Glucocorticoids/administration & dosage ; Humans ; Hyperemia/drug therapy/etiology/pathology ; Male ; Methylprednisolone/administration & dosage ; Papilledema/drug therapy/*etiology/pathology ; Snake Bites/*complications/*therapy ; Uveitis, Anterior/drug therapy/*etiology/pathology

A 32-year-old male who had infective endocarditis complained of photophobia and blurred vision in both eyes. Biomicroscopic examination and fundus examination revealed anterior chamber reaction, vitritis, optic disc swelling, and Roth spots. He was diagnosed with bilateral nongranulomatous uveitis and treated with topical steroid eye drops and posterior sub-Tenon injection of triamcinolone. His visual symptoms were resolved within 1 week, and inflammation resolved within 4 weeks after treatment.
Adult ; Diagnosis, Differential ; Echocardiography, Transesophageal ; Endocarditis, Bacterial/*complications/diagnosis ; Fluorescein Angiography ; Fundus Oculi ; Glucocorticoids/administration & dosage ; Humans ; Intraocular Pressure ; Intravitreal Injections ; Male ; Triamcinolone Acetonide/administration & dosage ; Uveitis/*complications/diagnosis/drug therapy ; Visual Acuity

The clinical features of HIV/AIDS-related ocular manifestations in Korean patients were investigated in this study. Data on 200 consecutive Korean patients diagnosed with AIDS who visited the Seoul National University Hospital from January 2003 to June 2008 were reviewed. Fifty-seven patients (28.5%) had ocular manifestations, and they showed significantly lower CD4+ T cell count than patients without ocular manifestations. Among them, 23 (40.3%) patients showed retinal microvasculopathy, and 22 (38.5%) patients showed cytomegalovirus (CMV) retinitis. Other manifestations included retinal vein occlusion (n = 4), herpes zoster ophthalmicus (n = 4), syphilitic uveitis (n = 2), acute retinal necrosis (n = 1), and progressive outer retinal necrosis (n = 1). The mean CD4+ lymphocyte counts of the patients with retinal microvasculopathy and cytomegalovirus retinitis were 108.5 cells/microL and 69.4 cells/microL, respectively. In conclusion, ocular manifestations including CMV retinitis are common complications in Korean patients with AIDS even in the era of highly active anti-retroviral therapy. Compared to previous reports in western countries, prevalence of CMV retinitis is relatively low and CD4+ lymphocytes count at the time of diagnosis is relatively high.
AIDS-Related Opportunistic Infections/*etiology ; Adult ; Aged ; Anti-HIV Agents/therapeutic use ; CD4 Lymphocyte Count ; CD4-Positive T-Lymphocytes/cytology ; Cytomegalovirus Retinitis/epidemiology/etiology ; Eye Diseases/etiology ; Eye Infections, Viral/etiology ; Female ; HIV Infections/*complications/drug therapy/metabolism ; Humans ; Male ; Middle Aged ; Necrosis/etiology ; Prevalence ; Republic of Korea/epidemiology ; Retinitis/etiology ; Uveitis/etiology ; Young Adult