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

Tubulointerstitial nephritis and uveitis (TINU) syndrome is a rare disease entity usually occurring in children. In the present study a case of TINU syndrome in an elderly patient is described and relevant literature is reviewed. A 61-year-old man presented with bilateral flank pain, urinary frequency, and foamy urine. A kidney ultrasonography revealed an increase in kidney parenchyma echogenicity. Following a kidney biopsy, the patient was diagnosed with acute tubulointerstitial nephritis. An ophthalmology examination initially performed for floater symptoms, revealed anterior uveitis in both eyes. Acute tubulointerstitial nephritis and anterior uveitis in both eyes responded to treatment with oral prednisolone, furosemide, carvedilol, and a topical steroid. TINU syndrome can occur in the elderly and should be part of the differential diagnosis when seeing a patient who has uveitis in association with renal disease; any therapy should be managed by both an internist and an ophthalmologist.
Biopsy ; Diagnosis, Differential ; Glucocorticoids/therapeutic use ; Humans ; Male ; Middle Aged ; Nephritis, Interstitial/*diagnosis/drug therapy ; Uveitis/*diagnosis/drug therapy

Adult ; Behcet Syndrome ; complications ; drug therapy ; Drugs, Chinese Herbal ; therapeutic use ; Humans ; Male ; Uveitis ; complications ; drug therapy

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

Suprofen eye drop was instilled into one eye of 10 pigmented rabbits and then anterior uveitis was induced by intraperitoneal injection of endotoxin of Shigella flexneri serotype 1A to evaluate the effects of non-steroidal anti-inflammatory drug on endotoxin induced uveitis. The pupillary diameters were measured, and aqueous cell and flare gradings were recorded in 20 eyes of 10 rabbits for one week at an interval of 12 hours for the first 24 hours and then every 24 hours for a week. A difference between the treated and control groups were investigated. All the above parameters showed greatest changes at 12 or 24 hours after injection and became normal by one week. The two groups demonstrated statistically significant difference at 12 hours, day 1 and day 2 as for pupillary diameter, at day 1 and day 2 as for cell and at 12 hours and day 1 as for flare. Thus, it can be concluded that prostaglandins play a role in miosis, in the appearance of inflammatory cells and flare in endotoxin induced uveitis and the topical administration of non-steroidal anti-inflammatory drug can alleviate signs of anterior uveitis. Specific relationship between leukotriene B4 and aqueous cell was not demonstrated.
Administration, Topical ; Animals ; Cell Count ; Endotoxins ; Rabbits ; Shigella flexneri ; Suprofen/*therapeutic use ; Uveitis, Anterior/*drug therapy/pathology

We report a case of post-streptococcal uveitis mainly presenting with bilateral recurrent retinal vasculitis in Korea. A 14-year-old Asian female presented with decreased visual acuity of 20 / 30 in the right eye and 20 / 25 in the left eye. The patient had a history of glomerulonephritis nine months before onset of uveitis. The manifestation of uveitis was predominantly retinal vasculitis. We presumed post-streptococcal uveitis because probable streptococcal infection was confirmed by anti-streptolysin O titer elevation. With topical and oral steroid treatments, the patient experienced complete vision recovery. Post-streptococcal uveitis occurs rarely and mostly involves young patients in the form of non-granulomatous anterior uveitis. However, as this case shows, it may primarily involve the posterior uvea without anterior inflammation and may recur.
Adolescent ; Diagnosis, Differential ; Female ; Humans ; Recurrence ; Retinal Vasculitis/drug therapy/*microbiology ; Steroids/therapeutic use ; Streptococcal Infections/*diagnosis ; Uveitis/drug therapy/*microbiology ; Visual Acuity

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

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

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

PURPOSE: To report one case of anterior uveitis following influenza vaccination in immunecompromised patient. METHODS: A 63-year-old woman with anterior uveitis following influenza vaccination 9 days after ovarian cancer chemotherapy was examined systematically and treated with 1% prednisolone eyedrop and dexamethasone subconjunctival injection. RESULTS: At the first visit, the corrected vision and intraocular pressure were 0.3 and 23 mmHg in both eyes. And, corneal edema and inflammatory cells in anterior chamber and vitreous were found, but there was no abnormal finding in fundus photography, fluorescein angiography and systemic evaluation. Although the response to steroid treatment was slow, she got better ophthalmologically and no problem in follow checkup 6 months later. CONCLUSIONS: We report a case of anterior uveitis following influenza vaccination in an immunocompromised patient after chemotheraphy and recommend to be cautious in influenza vaccination in the suspicious case of immunecompromized state.
Anterior Chamber ; Corneal Edema ; Dexamethasone ; Drug Therapy ; Female ; Fluorescein Angiography ; Humans ; Immunocompromised Host ; Influenza, Human* ; Intraocular Pressure ; Middle Aged ; Ovarian Neoplasms ; Photography ; Prednisolone ; Uveitis, Anterior* ; Vaccination*