Ocular Complications of Intermediate Uveitis.

Intermediate uveitis (IU) in an intraocular inflammation that predominantly affects the vitreous. It typically manifests as chronic low-grade inflammation, mainly affecting children and young adults. Uncontrolled chronic intraocular inflammation carries a high risk of visual loss. IU, despite being a low-grade inflammation, is among the sight-threatening conditions owing to its intrinsically-associated vision-robbing ocular complications. It can cause early and late-onset ocular complications affecting the anterior and posterior segments of the eye. 40-60% of the patients who suffer from IU will develop at least one ocular complication during the disease course. Prompt management and long-term monitoring are vital for the early detection of ocular complications and timely intervention. Proactive screening programs are essential in children because of the occult nature of IU in the pediatric age group and the high prevalence of complications at the initial visit. This review provides a comprehensive panorama of anterior segment complications, namely, band keratopathy, corneal endotheliopathy, posterior synechiae, and cataract as well as of posterior segment complications including macular edema, retinoschisis, retinal detachment, vitreous hemorrhage, vasoproliferative tumor, optic neuritis, and papillitis, in addition to glaucoma and hypotony.

Development of Clarstatin, a Novel Drug Lead for the Therapy of Autoimmune Uveitis.

We describe the design, synthesis, and activity of a potent thiourea-bridged backbone cyclic peptidomimetic known as Clarstatin, comprising a 5-amino acid sequence (Q/D)1-(R/K)2-X3-X4-A5-(Gln/Asp)1-(Arg/Lys)2-AA3-AA4-Ala5-based on a motif called "shared epitope (SE)", specifically present in specific alleles of the HLA-DRB1 gene. This SE binds to a particular site within the proline reach domain (P-domain) of the cell surface-calreticulin (CS-CRT). CS-CRT is a multifunctional endoplasmic reticulum (ER) calcium-binding protein that is located on the cell surface of T cells and triggers innate immune signaling, leading to the development of inflammatory autoimmune diseases. The development of Clarstatin was based on the parent peptide W-G-D1-K2-S3-G4-A5- derived from the active region of the SE. Following the design based on the cycloscan method, the synthesis of Clarstatin was performed by the Fmoc solid phase peptide synthesis (SPPS) method, purified by HPLC to 96% homogeneity, and its structure was confirmed by LC-MS. Clarstatin reduced calcium levels in Jurkat lymphocyte cultures, ameliorated uveitis in vivo in the experimental autoimmune uveitis (EAU) mice model, and was safe upon acute toxicity evaluation. These findings identify Clarstatin as a promising lead compound for future drug development as a novel class of therapeutic agents in the therapy of uveitis.

The Spectrum of Papillophlebitis.

PURPOSE: Papillophlebitis is a rare condition, manifesting as CRVO in the young adults. We aim to present our experience in managing patients with papillophlebitis. METHODS: Retrospective review of patients' medical files. RESULTS: Included were seven patients with a mean presenting age of 24.86 ± 4.4 years and mean follow-up of 40.4 ± 50.5 months. No pre-existing systemic illness was reported by any patient. One patient was subsequently diagnosed to have Behçet disease and another patient was diagnosed with homozygous mutation to MTHFR C6771. On presentation, fluorescein angiograms showed diffuse vascular and optic disc leakage. Four patients presented with papillophlebitis-associated CME, for which they were treated with systemic steroids and intravitreal anti-VEGF injections. One patient showed full recovery. In 3 patients, due to the protracted course of papillophlebitis and refractory CME, adalimumab was added. All 3 patients eventually showed complete resolution of CME. Two of them eventually developed extensive peripheral capillary non-perfusion that was treated with panretinal photocoagulation. Three patients did not develop CME: In two patients, papillophlebitis resolved after a short course of prednisone while in the third patient, papillophlebitis resolved spontaneously. Mean ± SD presenting log MAR VA was 0.2 ± 0.32 and it was 0.057 ± 0.11 at the last follow-up. CONCLUSION: To the best of our knowledge, this is the first description that suggests a role for TNF-ɑ blockers in the management of patients with recalcitrant papillophlebitis and non-responsive CME. Further studies are needed in order to thoroughly investigate the molecular background of papillophlebitis and clinical outcomes associated with this class of medications.

Subretinal Fibrosis Developing 10 Years After First Presentation with Chronic Ocular Sarcoidosis in a Child.

PURPOSE: To provide a comprehensive overview of the diagnostic and therapeutic journey of a pediatric patient with persistent sarcoid-associated panuveitis over a 10-year period, who ultimately developed bilateral macular subretinal fibrosis and visual loss. METHODS: Retrospective case report. RESULTS: The patient was diagnosed with sarcoidosis after undergoing a transbronchial biopsy. She was followed up because of granulomatous panuveitis, multifocal choroiditis, and papillitis bilaterally. She maintained a stable condition, and visual acuity was 0.3 RE and 0.5 LE. Immunomodulatory therapy included prednisone, methotrexate, and adalimumab. The patient was lost to follow-up for 20 months because of the COVID-19 pandemic. She was represented with active uveitis and was not responding to TNF-ɑ inhibitors (adalimumab and infliximab). Ultimately, the patient's intraocular inflammation was successfully controlled by using intravitreal steroids (Triamcinolone and Fluocinolone acetonide implant). However, the visual outcome was guarded because of bilateral subretinal fibrosis. CONCLUSION: 10% of patients with sarcoidosis-associated uveitis risk blindness in one eye. The index case progressed to sight-robbing bilateral subretinal fibrosis, a rare complication of ocular sarcoidosis despite a combination of conventional and biologic anti-inflammatory therapies. There is a pressing need to develop new treatment agents for refractory non-infectious uveitis.

Aqueous humor perturbations in chronic smokers: a proteomic study.

The detrimental effects of smoking are multisystemic and its effects on the eye health are significant. Smoking is a strong risk factor for age-related nuclear cataract, age-related macular degeneration, glaucoma, delayed corneal epithelial healing and increased risk of cystoid macular edema in patients with intermediate uveitis among others. We aimed to characterize the aqueous humor (AH) proteome in chronic smokers to gain insight into its perturbations and to identify potential biomarkers for smoking-associated ocular pathologies. Compared to the control group, chronic smokers displayed 67 (37 upregulated, 30 downregulated) differentially expressed proteins (DEPs). Analysis of DEPs from the biological point of view revealed that they were proteins involved in complement activation, lymphocyte mediated immunity, innate immune response, cellular oxidant detoxification, bicarbonate transport and platelet degranulation. From the molecular function point of view, DEPs were involved in oxygen binding, oxygen carrier activity, hemoglobin binding, peptidase/endopeptidase/cysteine-type endopeptidase inhibitory activity. Several of the upregulated proteins were acute phase reactant proteins such as clusterin, alpha-2-HS-glycoprotein, fibrinogen, alpha-1-antitrypsin, C4b-binding protein and serum amyloid A-2. Further research should confirm if these proteins might serve as biomarkers or therapeutic target for smoking-associated ocular diseases.

Vasoproliferative Tumor Secondary to Sarcoidosis-Associated Intermediate Uveitis.

We report the visual and clinical outcomes of a middle-aged woman who presented with exudative retinal detachment (ERD) secondary to a vasoproliferative tumor (VPT) in an eye with sarcoidosis-associated intermediate uveitis. A 55-year-old woman previously diagnosed with sarcoidosis presented with decreased vision in the left eye (LE). Visual acuity in the LE was counting fingers. She had active vitritis, and a peripheral retinal vascular mass was noted in the superotemporal periphery. The mass was associated with ERD involving the posterior pole. The patient was managed with systemic and intravitreal steroids, and cyclosporine was subsequently added as a steroid-sparing agent. Because of recurrence of ERD, the patient underwent pars plana vitrectomy, and cryotherapy and laser photocoagulation were applied to the VPT. Two months postoperatively, visual acuity in the LE improved to 6/10. There was marked regression of the VPT and total resolution of the ERD. In conclusion, we report a favorable visual and clinical outcome in a patient with VPT-associated ERD who responded to a combination of medical therapy and surgical intervention. VPT may lead to different remote complications, so timely diagnosis of these tumors and proper management of their complications is warranted.

The Great Imitator Masquerading as Autoimmune Hepatitis and Demystifying as Fulminant Syphilitic Retinitis.

PURPOSE: Syphilis is a "Great Masquerader" because of its versatile clinical manifestations. We aim to report a patient whose first presentation was with presumed autoimmune hepatitis. Fulminant retinitis subsequently ensued, thus enabling correct diagnosis and treatment. METHODS: Case description. RESULTS: A 62-year-old male presented with bilateral drop in vision. One month earlier, right eye (RE) arteritic ischemic optic neuropathy was suspected because of severe headache, sudden drop in vision, relative afferent pupillary defect, and elevated inflammatory markers. Systemic steroids were instituted. Brain imaging and temporal artery biopsy were unyielding. Four months earlier, liver biopsy performed because of elevated cholestatic liver enzymes, revealed granulomatous hepatitis. After ruling out viral hepatitis, autoimmune etiology was presumed, and prednisone was started. On presentation, visual acuity (VA) was counting fingers in RE and 6/20 in the left eye (LE). Bilateral panuveitis with punctate inner retinitis, placoid chorioretinitis was diagnosed. Serological tests were strongly positive for syphilis. Lumbar puncture confirmed the existence of neurosyphilis. Systemic penicillin was initiated. One month later, VA improved to RE 6/10, LE 6/7.5, with marked resolution of posterior uveitis. No recurrence was observed over 27-month-period. CONCLUSION: Acquired syphilitic hepatitis is rarely reported. Administering steroids potentially aggravated the infection. The characteristic features of placoid chorioretinitis and punctate inner retinitis connected the pieces of the puzzle together to the diagnosis of ocular and neurosyphilis. Intrahepatic cholestasis with negative serological panel of hepatotropic pathogens should raise the suspicion of non-hepatotropic pathogens especially syphilis.

Long-term clinical outcomes of patients with sympathetic ophthalmia.

PURPOSE: To present the long-term clinical outcomes of patients with sympathetic ophthalmia (SO). METHODS: Retrospective review of patients' medical files between 2002 and 2022. RESULTS: Included were seven patients (four males). The mean ± SD age at presentation was 37.9 ± 22.5 years. Four patients had co-morbidities: three had diabetes mellitus type 2 and one had Turner Syndrome. Trauma was the inciting event in six patients and postoperative endophthalmitis in one patient. Decreased visual acuity (VA) was the leading symptom in the sympathizing eye and all of the patients presented with panuveitis. The mean ± SD interval between the triggering incident and the onset of SO in six cases was 4.3 ± 4.2 months. One case presented 30 years following the eye injury. Five patients underwent enucleation/evisceration of the exciting eye. The mean ± SD presenting LogMAR BCVA in the sympathizing eye was 0.57 ± 0.82, and the final LogMAR BCVA was 0.61 ± 0.95. Inflammation was completely controlled in 5 patients at a mean ± SD of 8.55 ± 9.21 months following the institution of immunomodulatory therapy, and it was partially controlled in 2 patients. VA deteriorated in all 3 diabetic patients and improved or remained stable in the 4 young and healthy patients. The mean ± SD follow-up period after achieving drug-free remission was 28 ± 22.8 months. The mean ± SD follow-up time was 6.8 ± 5.6 years. CONCLUSIONS: SO is one of the most sight-threatening conditions, affecting the healthy eye. In this cohort, the favorable visual outcome was especially seen in young and healthy individuals. Visual prognosis is directly related to prompt diagnosis and treatment.

Long-term follow-up of a healthy man with endogenous Streptococcus anginosus endophthalmitis.

We report the long-term follow-up of an immunocompetent patient who presented with slowly progressive endogenous endophthalmitis secondary to Streptococcus anginosus. A 46-year-old healthy man presented with a two-month history of right eye iritis. On examination, visual acuity was 20/60 with intraocular pressure of 6 mm Hg. There was a small layer of hypopyon with non-granulomatous anterior uveitis and vitritis. On funduscopy, fluffy white peripheral retinal and pre-retinal lesions were noted in superonasal periphery. The patient denied any present or past illness. Diagnostic pars plana vitrectomy was performed. Culture and polymerase chain reaction of the vitreous sample were positive for Streptococcus anginosus. Intravitreal vancomycin and ceftazidime and systemic ceftriaxone were administered. Work-up which included blood and urine cultures, chest x-ray, echocardiography and abdominal ultrasound was unyielding. Subsequently and because of persistent post-infectious inflammatory reaction, intravitreal and oral steroids were administered in addition to oral azathioprine later on. After one year of follow-up, visual acuity was 20/20 with near vision of Jaeger 3 + and no signs of active uveitis were seen. Therefore, Streptococcus anginosus should be considered in the differential diagnosis of a slowly progressive endophthalmitis also in immunocompetent individuals.

The Humira in Ocular Inflammations Taper (HOT) Study.

PURPOSE: To assess factors that impact the risk of relapse in patients with noninfectious uveitis (NIU) who undergo adalimumab tapering after achieving remission. DESIGN: Retrospective study. METHODS: In this multicenter study, patients with NIU were treated with adalimumab and subsequently tapered. Patient demographics, type of NIU, onset and duration of disease, the period of inactivity before tapering adalimumab, and the tapering schedule were collected. The primary outcome measures were independent predictors of the rate of uveitis recurrence after adalimumab tapering. RESULTS: Three hundred twenty-eight patients were included (54.6% female) with a mean age of 34.3 years. The mean time between disease onset and initiation of adalimumab therapy was 35.2 ± 70.1 weeks. Adalimumab tapering was commenced after a mean of 100.8 ± 69.7 weeks of inactivity. Recurrence was observed in 39.6% of patients at a mean of 44.7 ± 61.7 weeks. Patients who experienced recurrence were significantly younger than those without recurrence (mean 29.4 years vs 37.5 years, P = .0005), and the rate of recurrence was significantly higher in younger subjects (hazard ratio [HR] = 0.88 per decade of increasing age, P = .01). The lowest rate of recurrence was among Asian subjects. A faster adalimumab taper was associated with an increased recurrence rate (HR = 1.23 per unit increase in speed, P < .0005). Conversely, a more extended period of remission before tapering was associated with a lower rate of recurrence (HR = 0.97 per 10-weeks longer period of inactivity, P = .04). CONCLUSIONS: When tapering adalimumab, factors that should be considered include patient age, race, and duration of disease remission on adalimumab. A slow tapering schedule is advisable.

Increased Risk of Celiac Disease in Patients with Uveitis.

PURPOSE: To examine whether patients with non-infectious uveitis (NIU) are at increased risk for celiac disease (CeD). METHODS: Celiac antibody testing was completed in 112 patients. The control group included patients who had undergone upper endoscopy for suspicion of CeD. RESULTS: 2/112 (1.79%) of patients with NIU had positive anti-tTG serology and CeD was confirmed in both patients. When compared to the expected risk of CeD in the general Israeli population of 0.31%, this corresponded to an odds ratio of 5.77 (95% CI 1.4118 to 23.4737, P = 0.049). Three additional patients had positive serology for CeD but the diagnosis was not confirmed. CONCLUSIONS: An increased risk of CeD was detected in patients with NIU. We therefore recommend screening for CeD in NIU patients. Larger prospective studies are required to further validate these results.

Uveitis in Children: The Role of Biological Agents in Its Management.

We aimed to determine medium and long-term effects of TNF-α inhibitors in patients with pediatric uveitis. This was a retrospective review of medical charts. Included were 50 patients (84 eyes). Mean age at diagnosis was 7.22 ± 4.04 years. At baseline (time of initiation of biologic therapy), all patients had active uveitis. Complete control of uveitis was achieved in 84.52% (n = 71) of eyes, after a median of 3 months (IQR 2 months). Mean LogMAR BCVA at baseline was 0.23 ± 0.44; it remained stable at 12 and 24 months. At baseline, 64% of patients were treated with oral corticosteroids, this decreased to 29.5% at 12 months (p = 0.001) and to 21.9% at 24 months (p < 0.001). Mean time to prednisone dose of ≤0.2 mg/kg/day was 8.1 ± 2.02 months after baseline. A total of 40.5% of eyes were treated with topical steroids at baseline and this significantly decreased to 5.8% at 12 months. Multiple linear regression model was calculated to predict moderate and severe visual loss; only presenting visual acuity accounted for a unique variance in the model. In conclusion, TNF-α inhibitors achieved rapid disease control while enabling a remarkable steroid-sparing effect in children suffering from chronic uveitis. Presenting visual acuity was the sole predictor of moderate to severe visual loss.

Total exudative retinal detachment in a child with pars planitis- a challenging case with optimistic results.

We describe a case report of pediatric pars planitis complicated with massive exudative retinal detachment (ERD). A 7-year-old presented with visual acuity (VA) in the right eye (RE) of 6/9 and in the left eye (LE) 6/15. Fundoscopy revealed BE inferior retinoschisis, vitritis and snowballs. He was treated with systemic immunosuppressants. RE retinoschisis resolved within 2 months. Three years later presented with LE VA 6/60 and total ERD. Systemic and intravitreal steroids were administered. Due to refractoriness, he underwent 360° scleral buckle and drainage of subretinal fluid. No retinal breaks or traction were detected. Five months postoperatively LE VA was 6/7.5. Long-term stable outcome was maintained. We report a challenging total ERD as a complication of pars planitis. Although extensive and non-responsive to medical therapy, complete resolution and improvement in vision was achieved with surgical intervention and systemic immunosuppression. We speculate that uncontrolled chronic vasculitic process culminated in diffuse ERD.

The effect of pregnancy on the course of uveitis in single and multiple pregnancies.

PURPOSE: To explore the effect of pregnancy on the clinical course, outcome, and treatment in multiparous women with non-infectious uveitis. METHODS: Retrospective study of women with a history of non-infectious uveitis and pregnancies prior to and during disease course. Disease activity and severity 1-year prior pregnancy, during pregnancy, and 1-year postpartum were recorded as well as patients' and diseases' characteristics. The main outcome measures included the rate and severity of uveitis attacks and the effect on ocular complications and therapies. RESULTS: Included were 32 women (70 pregnancies, mean of 2.6 pregnancies/patient), with a mean follow-up time of 6.5 years. The most common uveitis types were anterior (31%) and pan-uveitis (31%). Flare-ups were more frequent in the year prior to pregnancy, in the first trimester, and in the postpartum period and decreased markedly during pregnancy. Women who experienced a flare-up during pregnancy had a higher rate of flare-ups in the year prior pregnancy than those who did not experience a flare-up during pregnancy (p-0.047). The rate of flare-ups 12 months' postpartum was also higher compared to women without any flare-up during pregnancy (p = 0.01). Severity of flare-ups in the postpartum period was worse in women who experienced a flare-up during pregnancy compared to women without flare-ups (p = 0.001). The severity of flare-ups was higher in the first pregnancy compared to subsequent pregnancies. CONCLUSIONS: Women who had active or non-controlled uveitis prior to pregnancy have higher disease activity and severity during pregnancy as well. The first pregnancy seems to behave differently from subsequent pregnancies, in terms of disease severity.

Posterior ocular manifestations following BNT162b2 mRNA COVID-19 vaccine: a case series.

PURPOSE: To report the occurrence of posterior ocular adverse events following the administration of the BNT162b2 mRNA vaccine against SARS-CoV-2. METHODS: A retrospective consecutive case series, in which the medical files of patients presenting with ocular adverse events within 30 days of the vaccine inoculation, were analyzed. RESULTS: Four patients (2 females) were included in the study. The diagnoses included: posterior scleritis, paracentral acute middle maculopathy, herpes panuveitis, and Vogt-Koyanagi-Harada (VKH)-like uveitis. Three of the patients had no relevant ocular history, but the patient who developed scleritis was in remission without medical therapy for four years, until the flare-up, which occurred one day after the vaccine. All patients improved with treatment. CONCLUSION: Though a causal relationship cannot be definitively established, the temporal relationship suggests a possible link between the COVID-19 vaccine and the posterior ocular complications. The benefits of vaccination clearly outweigh the potential adverse effects; however, ophthalmologists should be aware of the potential for vaccine-associated uveitis.

Enhanced S-Cone Syndrome Masquerading as TORCH in an Infant and a Toddler.

PURPOSE: To report two cases masquerading as TORCH but eventually diagnosed with Enhanced S-cone Syndrome (ESCS). METHODS: Descriptive case report. RESULTS: Case 1: A ten-month-old boy presented with high hypermetropia, strabismus and bilateral chorioretinal pigmented scars with a history of cat scratch of his mother during pregnancy. He was treated for suspected toxoplasma retinitis. Choroidal neovascular membranes (CNV) were diagnosed bilaterally and treated with intravitreal bevacizumab. Genetic testing showed homozygote mutation in NR2E3 gene. Case 2: A two-year old girl presented with bilateral high hypermetropia and strabismus. Funduscopy revealed extrafoveal chorioretinal lesions and surrounding subretinal fibrosis. An elevated titer of anti-toxocara IgG antibodies was detected and managed accordingly. LE CNV was diagnosed and treated with intravitreal bevacizumab. Genetic testing disclosed homozygote mutation in NR2E3. CONCLUSION: Ocular manifestations in ESCS can be reminiscent to TORCH. CNV may develop with an incidence of 15%. We report the youngest patient with ESCS-associated CNV.

Long-term Experience with Anti-tumor Necrosis factor- α Therapy in the Treatment of Refractory, Non-infectious Intermediate, Posterior, and Panuveitis.

PURPOSE: To study the efficacy and long-term effects of infliximab and adalimumab in patients with active refractory non-infectious intermediate, posterior, or panuveitis (NIPPU). METHODS: Retrospective, longitudinal study. RESULTS: Included were 61 patients (104 eyes) of whom 34 were males (55.74%). Mean age at diagnosis of uveitis was 26.5 ± 16.14 years. All patients had active uveitis at baseline (time of initiation of biological therapy). Median interval between the start of conventional immunomodulatory therapy (IMT) to the introduction of biological therapy was 13.0 (IQR 26.0) months. Ocular inflammation was effectively controlled in 92 eyes (88.46%). The most commonly used TNF-α inhibitor was adalimumab in 47 patients (77%). Mean follow-up time after baseline was 40 ± 34.08 months. In the year preceding the institution of TNF-α inhibitors, the average number of flares was 1.5 ± 1.1/year and it decreased to 0.08 ± 0.29/year in the first year after baseline (p < .0005). Forty-four eyes (42.30%) experienced flare over the entire follow-up period. Mean time to first flare was 14.5 ± 9.26 months. At baseline, the mean dose of prednisone was 25.5-±20.8 mg/day. A marked decrease to a mean prednisone dose of 7.85 ± 9.7 mg/day was observed at 6 months (p = .03). In patients treated with adalimumab, the mean time to prednisone dose ≤7.5 mg/day was 4.02 ± 4.89 months compared to 15.64 ± 21.34 months in patients treated with infliximab (p = .03). 64.3% of patients treated by infliximab had Behçet uveitis compared to 27.7% of patients treated by adalimumab. Eyes treated with adalimumab experienced first flare at a mean time of 14.11 ± 6.29 months, whereas eyes treated with infliximab experienced first flare at 18.29 ± 14.24 months after baseline (p < .0005). The risk for moderate and severe visual loss was lower with shorter duration of uveitis before initiating anti-TNF-α treatment (odds ratio, 0.003; 95% CI, 0.000-0.005; p = .023), better presenting logMAR VA (odds ratio, 0.266; 95% CI, 0.172-0.361; p < .0005) and when adalimumab was used (odds ratio, 0.354; 95% CI, 0.190-0.519, p < .0005). CONCLUSIONS: Anti-TNF-α therapy was successful in controlling refractory NIPPU in the majority of cases. It significantly reduced flare rate, exerted steroid-sparing effects, and preserved visual potential. Adalimumab use, better initial visual acuity, and earlier introduction of anti-TNF- α therapy were associated with a lower risk of visual loss.

[OCULAR MANIFESTATIONS IN SYSTEMIC DISEASES].

INTRODUCTION: Uveitis and neuro-ophthalmic manifestations may indicate an underlying systemic and/or neurologic condition. A systematic approach to patients in the form of comprehensive anamnesis and thorough ocular and physical examination helps to reveal an undiagnosed condition or an evolving illness. Prompt diagnosis is of utmost importance in such cases because of potential sight-threatening and life-threatening nature of such conditions. Both subspecialties of neuro-ophthalmology and uveitis require, in many instances, a multidisciplinary approach, as emphasized in this special issue.

[IDIOPATHIC INTRACRANIAL HYPERTENSION IN A PATIENT WITH ULCERATIVE COLITIS AND UVEITIS].

INTRODUCTION: Idiopathic intracranial hypertension (IIH) is a syndrome mostly affecting young, overweight women, which characteristically causes headaches, transient visual obscuration or double vision, tinnitus, nausea and vomiting. Severe disease may cause irreversible visual loss. IIH may be primary, or it may be secondary to various medications and diseases. We hereby present a case study of a patient with ulcerative colitis and uveitis, who presented with blurred vision, headache and tinnitus and was diagnosed as IIH. We discuss the differential diagnosis with relation to her underlying disease and treatments.

Outer Retina Rupture from Subretinal Blood with Spontaneous Sealing and Visual Recovery in Frosted Branch Angiitis from Familial Mediterranean Fever: A Case Report.

Familial Mediterranean fever (FMF) is a rare autoinflammatory disorder. Ocular involvement is rare. The full spectrum and response to treatment is poorly understood. An 18-year-old girl previously diagnosed with FMF presented with sudden loss of vision in the left eye (LE). Best-corrected visual acuity (BCVA) in the LE was finger counting at 1.5 meters. Angiitis with mild "frosting," hemi-central retinal vein occlusion (HCRVO), and acute outer retina rupture (ORR) were observed in the LE. Systemic steroids were initiated immediately. The ORR was sealed 2 weeks later while vision improved to 6/15 (near vision: J2) 5 months later. No recurrences were observed over 5 years of follow-up. We report a rare manifestation of frosted branch angiitis with concomitant HCRVO and ORR in a young patient with FMF. Closure of ORR was attained and vision recovered after treatment with high-dose steroids.