Ocular Relapse Research Articles

Leukemic occult infiltrative optic neuropathy presenting as optic neuritis: a case report

Isolated ocular relapses of leukemia are rare and typically present as infiltrative lesions of the optic nerve. This report presents a case of an isolated ocular relapse of leukemia in a female patient, characterized by occult infiltrative optic neuropathy. Despite the absence of leukemic cell infiltration on MRI, a 33-year-old female patient presented with optic neuritis, which resolved after intrathecal administration of chemotherapeutic agents. However, due to her non-compliance with the treatment regimen, leukemia progressed to infiltrative optic neuropathy. She refused radiation therapy, and while intrathecal injections provided temporary stabilization, they were insufficient for sustained disease control. This case uniquely documents the extended progression of an isolated ocular relapse of leukemia, evolving from an occult infiltrative lesion initially presenting as optic neuritis to a more pronounced infiltrative lesion. It suggests that leukemic optic nerve infiltration can occur in patients with leukemia even in the absence of MRI-detectable abnormalities. Radiation therapy emerges as a critical modality in the management of infiltrative lesions of the optic nerve.

INTEREST OF REGULAR ASSAYS OF AQUEOUS HUMOR INTERLEUKIN-10 LEVELS IN MONITORING OF VITREORETINAL LYMPHOMA.

To investigate the variation of interleukin-10 (IL-10) levels in the aqueous humor (AH) of patients with vitreoretinal lymphoma (VRL) throughout therapy and follow-up and analyze the relation of these variations with VRL clinical course and relapse. This study retrospectively included consecutive patients diagnosed with VRL in a single center. AH IL-10 samples and patient clinical course were evaluated. The response to treatment was evaluated according to the criteria set by the International Primary Central Nervous System Lymphoma Collaborative Group. A total of 59 eyes of 34 patients were included. Interleukin-10 levels decreased significantly at first AH sample after therapy induction (median [IQR] 3.0 [2.8-3.6] months) among patients in complete clinical remission (P < 0.001). Among patients in complete clinical remission with residual detectable IL-10 in AH after therapy induction (85.3% systemic chemotherapy, 11.8% intravitreal methotrexate, 2.9% palliative care), 87.5% experienced ocular relapse within 5 years. The detection of IL-10 in AH at the first visit after induction for complete clinical remission obtained a sensitivity of 77.8% (95% CI 0.45-0.96) and a specificity of 96.4% (95% CI 0.82-0.99) to predict ocular relapse. For relapsing eyes (N = 26), IL-10 significantly increased between the last IL-10 measurement and the time of the first ocular relapse (P < 0.001). In 76.0% of cases, an increase in IL-10 was detected earlier than clinical relapse with a mean (SD) of 4.0 (2.4) months. The present study suggested the usefulness of IL-10 in the prognosis of VRL. This study showed a relation between IL-10 in AH and tumoral activity, and for the first time with disease relapse.

Utility of PD-1, PD-L1, and IDO-1 Stains in Ocular Extranodal Marginal Zone Lymphoma (MZL) and Diffuse Large B-cell Lymphoma (DLBCL).

Extranodal marginal zone lymphoma (EMZL) is the most common subtype of ocular lymphomas. Diffuse large B-cell lymphoma (DLBCL) and EMZL with large-cell transformation present diagnostic challenges. Radiotherapy is the standard treatment for ocular lymphomas, but complications and relapse are common. Diagnostic utility in challenging cases, as well as treatment options using immune checkpoint inhibitors, are unclear in ocular lymphomas. We herein investigated the PD-1, PD-L1, and IDO1 staining patterns in 20 cases of ocular lymphomas, including EMZL (n=14), EMZL with increased large cells (n=2), and DLBCL (n=4). PD-1, PD-L1, and IDO1 staining was not detected in lymphoma cells in any cases but was observed within the tumor microenvironment in all cases. Positivity for PD-1, PD-L1, and IDO1 in inflammatory cells was seen either intratumorally or peritumorally. In all 6 cases with significantly more large B cells, the density of PD-1, PD-L1, and IDO1 expression in the tumor microenvironment was higher than that of the remaining 14 cases without large B cells ( P -value<0.0001), whereas other clinicopathologic features showed no statistical correlation. Increased expression of PD-1, PD-L1, and IDO1 in the inflammatory milieu in cases with large cells may provide diagnostic utility in small biopsies as well as therapeutic potential.

Resolution of Anterior Uveal Infiltration of Acute Lymphoblastic Leukemia After Chimeric Antigen Receptor T-Cell Therapy

Purpose: To present a case of acute B-cell lymphoblastic leukemia (B-ALL) ocular relapse treated with chimeric antigen receptor T-cell (CAR T) therapy. Methods: A retrospective case review was evaluated. Results: A 69-year-old woman with a history of B-ALL presented with bilateral hypopyons and 3+ anterior chamber cells that responded to topical prednisolone. Six months later, she developed recurrent hypopyons, iris neovascularization, and iris nodularity in both eyes. A fine-needle aspiration biopsy confirmed ocular infiltration of B-ALL. A single infusion of CAR T therapy resulted in remission of the systemic and ocular findings. The clinical course was complicated by cytokine release syndrome and neurotoxicity, which improved with medical management. The patient remained in remission for 7 months after a single CAR T infusion. Conclusions: CAR T therapy may be effective in treating systemic leukemia and uveal infiltration, with an ocular side-effect profile and visual outcomes. The systemic side effects of CAR T therapy may be managed medically.

Effectiveness and Safety of Biosimilars in Pediatric Non-infectious Uveitis: Real-Life Data from the International AIDA Network Uveitis Registry.

Since many biological drug patents have expired, biosimilar agents (BIOs) have been developed; however, there are still some reservations in their use, especially in childhood. The aim of the current study is to evaluate the efficacy and safety of tumor necrosis factor (TNF) inhibitors BIOs as treatment for pediatric non-infectious uveitis (NIU). Data from pediatric patients with NIU treated with TNF inhibitors BIOs were drawn from the international AutoInflammatory Disease Alliance (AIDA) registries dedicated to uveitis and Behçet's disease. The effectiveness and safety of BIOs were assessed in terms of frequency of relapses, risk for developing ocular flares, best-corrected visual acuity (BCVA), glucocorticoids (GCs)-sparing effect, drug survival, frequency of ocular complications, and adverse drug event (AE). Forty-seven patients (77 affected eyes) were enrolled. The BIOs employed were adalimumab (ADA) (89.4%), etanercept (ETA) (5.3%), and infliximab (IFX) (5.3%). The number of relapses 12months prior to BIOs and at last follow-up was 282.14 and 52.43 per 100 patients/year. The relative risk of developing ocular flares before BIOs introduction compared to the period following the start of BIOs was 4.49 (95% confidence interval [CI] 3.38-5.98, p = 0.004). The number needed to treat (NNT) for ocular flares was 3.53. Median BCVA was maintained during the whole BIOs treatment (p = 0.92). A significant GCs-sparing effect was observed throughout the treatment period (p = 0.002). The estimated drug retention rate (DRR) at 12-, 24-, and 36-month follow-up were 92.7, 83.3, and 70.8%, respectively. The risk rate for developing structural ocular complications was 89.9/100 patients/year before starting BIOs and 12.7/100 patients/year during BIOs treatment, with a risk ratio of new ocular complications without BIOs of 7.1 (CI 3.4-14.9, p = 0.0003). Three minor AEs were reported. TNF inhibitors BIOs are effective in reducing the number of ocular uveitis relapses, preserving visual acuity, allowing a significant GCs-sparing effect, and preventing structural ocular complications. ClinicalTrials.gov ID NCT05200715.

Efficacy and safety of Janus kinase inhibitors in non-infectious inflammatory ocular diseases: a prospective cohort study from the international AIDA network registries.

Non-infectious inflammatory ocular diseases pose significant challenges in diagnosis and management, often requiring systemic immunosuppressive therapy. Since Janus kinase (JAK) inhibitors may represent a novel therapeutic option for these disorders, the present study aimed to expand current knowledge about their efficacy and safety in patients with these conditions. This prospective cohort study included 12 adult patients from the international AutoInflammatory Disease Alliance (AIDA) Network registries dedicated to non-infectious ocular inflammatory conditions. We assessed ocular flares, visual acuity, disease course, and complications before and after initiating JAK inhibitor therapy. Ocular inflammation was related to a systemic disease in 8 (66.7%) patients as follows: spondyloarthritis (n = 3), peripheral psoriatic arthritis (n = 1), rheumatoid arthritis (n = 1), antinuclear antibodies (ANA) positive juvenile idiopathic arthritis (n = 1), Behçet's syndrome (n = 1), Vogt-Koyanagi-Harada syndrome (n = 1). In total, 4 patients received baricitinib, 1 patient received tofacitinib, and 7 patients underwent upadacitinib treatment. The overall average duration of JAK inhibitors treatment was 8.6 ± 5.5 months (ranging from 3 to 20 months). At the last assessment, ocular disease control was complete in 12/12 patients. One patient discontinued baricitinib due to poor compliance after a 12-month relapse-free period. The incidence of ocular flares was 125 episodes/1.000 person-months prior to the initiation of JAK inhibitors and 28.6 episodes/1.000 person-months thereafter. The incidence rate ratio for experiencing a relapse before starting a JAK inhibitor compared to the following period was 4.37 (95% CI 1.3-14.7, p-value: 0.02). JAK inhibitors demonstrate efficacy and safety in controlling ocular inflammatory relapses, confirming that they represent a valuable treatment option for patients with non-infectious inflammatory ocular diseases resistant to conventional treatments.

Ocular and central nervous system relapse of Blastic Plasmacytoid Dendritic Cell Neoplasm.

Ocular and central nervous system relapse of Blastic Plasmacytoid Dendritic Cell Neoplasm.

Efficacy and Safety of Adalimumab in Pediatric Non-infectious Non-anterior Uveitis: Real-life Experience From the International AIDA Network Uveitis Registry.

Scientific evidence of the effectiveness of the tumor necrosis factor inhibitor adalimumab (ADA) in pediatric patients with non-infectious non-anterior uveitis is still limited. The aim of this study is to investigate the therapeutic role of ADA in a cohort of pediatric patients with non-anterior uveitis. This is an international multicenter study analyzing real-life data referred to pediatric patients treated with ADA for intermediate uveitis/pars planitis, posterior uveitis and panuveitis. Data were drawn from the AutoInflammatory Disease Alliance (AIDA) registry for patients with uveitis. Twenty-one patients (36 affected eyes) were enrolled, and all patients benefited from ADA administration. In detail, 11 patients (19 affected eyes) did not experience further ocular inflammation after ADA introduction; 10 cases (17 affected eyes) showed a significant clinical improvement consisting of a decrease in severity and/or frequency of ocular relapses. The number of ocular flares dropped from 3.91 to 1.1 events/patient/year after ADA introduction (p = 0.0009); macular edema and retinal vasculitis were respectively observed in 18 eyes and 20 eyes at the start of ADA and in 4 eyes and 2 eyes at the last assessment. The mean daily glucocorticoid dosage significantly decreased from 26.8 ± 16.8mg/day at the start of ADA to 6.25 ± 6.35mg/day at the last assessment (p = 0.002). Intermediate uveitis/pars planitis (p = 0.01) and posterior uveitis (p = 0.03) were more frequently observed in patients with full response to ADA; panuveitis (p = 0.001) was significantly more frequent among patients continuing to experience uveitic flares. This could be related to a higher use of systemic glucocorticoids (p = 0.002) and conventional immunosuppressants (p = 0.007) at the start of ADA when treating intermediate uveitis/pars planitis. Regarding the safety profile, only one adverse event was reported during ADA treatment, consisting of the development of generalized adenopathy. ADA proved to have an effective therapeutic role in all pediatric patients with non-anterior uveitis enrolled in the study. An overall glucocorticoid-sparing effect was observed despite the severity of cases enrolled. A more aggressive treatment of panuveitis and posterior uveitis at start of ADA could increase the likelihood of full response to therapy.

Longitudinal Analysis of Ocular Manifestation and Interleukin During Intravitreal Treatment of Vitreoretinal Lymphoma With Methotrexate

To explore the trend of ocular manifestations and interleukin (IL) during the treatment of vitreoretinal lymphoma (VRL), and to evaluate potential effects of different intravitreal administration schedules on the therapeutic response. Interventional comparative non-randomized clinical study. Patients diagnosed with VRL from January 2011 to January 2022 were included. Intravitreal methotrexate (MTX) injections consisting induction, consolidation and maintenance were scheduled. At baseline and each visit, ocular manifestations and interleukin in aqueous humor were recorded. Effects of the variations (e.g. frequency and number) in the injection schedule on the therapeutic response were analyzed. Fifty-eight eyes of 33 patients were treated with intravitreal MTX chemotherapy. A mean (±SD) of 9±3 injections were given; 52 eyes achieved complete remission (CR). IL-10, keratic precipitates and sub-retinal lesions correlated well with the course of treatment (all P <0.001). Initial injection given twice weekly was correlated with a higher rate of CR (36/36) than given once weekly or less frequently (16/22, P=0.011). Ocular progression occurred in 13 eyes of 8 patients. The completion of schedule was correlated with PFS (induction + consolidation + maintenance: 547[335-874] days, induction + consolidation: 355[322-831] days, induction only: 147[116-187.5] days, P<0.001). IL-10 over 50 pg/mL was a feasible threshold for the detection of ocular relapse (sensitivity 100.0%, specificity 95.1%). Keratic precipitates, sub-retinal lesions and IL-10 could serve as indicators for therapeutic response. Intensive initial administration and adequate injection number would help to improve the response and prognosis. IL-10 over 50 pg/mL could help to detect ocular relapse.

Acute Unilateral Hypopyon Uveitis in A Child with Post-BMT local Relapse of Acute Lymphoblastic Leukemia-Case Report

Relapse into the anterior ocular segment post bone marrow transplantation (BMT) for acute lymphoblastic leukemia (ALL) in a child is rare. We report this uncommon presentation as hypopyon uveitis in a patient in remission with complete chimerism after chemotherapy for ALL. The patient presented with red eye and slit lamp examination revealed hypopyon uveitis. The diagnosis of relapsed ALL was established by anterior segment aspiration followed by documentation of relapse with immunohistochemistry and flow cytometry of the fluid sample. Thus, a high index of clinical suspicion in uveitis cases post BMT, led to the early diagnosis of ocular relapse that prevented vision loss in the patient. Atypical unilateral hypopyon can therefore be an indication of relapsing ALL. Keywords: Acute Lymphoblastic Leukemia, ALL, Ocular Relapse, Hypopyon Uveitis

Clinical Features and Outcomes of Tubulointerstitial Nephritis and Uveitis Syndrome in Spain and Portugal: The IBERTINU Project

ABSTRACT Purpose To assess the clinical features, management and prognosis of patients diagnosed with tubulointerstitial nephritis and uveitis (TINU) syndrome in Spain and Portugal. Methods Retrospective multicenter study, which included all patients diagnosed with TINU syndrome managed in 15 uveitis referral centers from Spain and Portugal. Results Forty-eight subjects with a mean age at diagnosis of 25.0 [14.8; 49.5] years were included. Both eyes were affected in 43 patients (89.6%). The visual outcome was favorable, but immunosuppressive systemic therapy (IST) was needed in 26 (54.16%) due to relapses. Renal function normalized in 35 patients (72.9%). HLA-DQB1*05 was the most common genetic typing (57.1%). The mean follow-up time was 22.5 [11.0; 48.0] months. Conclusions Both visual and renal outcomes were favorable, although IST was frequently used. TINU is under- diagnosed, so further prospective studies would provide more knowledge about its recognition and management. Expert Opinion TINU Syndrome is underdiagnosed because ocular and renal disease are asynchronous. Outcomes are favorable so it has to be highly suspected in cases of bilateral anterior uveitis. According to literature and our experience, systemic immunosuppressive therapy is often required because of ocular relapses. This study adds to the previous knowledge that HLA-DQB1*05 could be an important HLA type amongst the TINU Syndrome Iberian population. HLA typing should be assessed in these patients in order to describe its phenotype-genotype relationship better. A considerable number of patients in our series were diagnosed in their sixties, so TINU Syndrome should also be suspected in elderly patients.

Ocular leukemic mass-like relapse treated with CyberKnife stereotactic radiosurgery.

Ocular leukemic mass-like relapse treated with CyberKnife stereotactic radiosurgery.

Prognostic Value of Early 18F-FDG PET/CT Scanner Evaluation in Immunocompetent Primary CNS Lymphoma Patients

▪Primary central nervous system lymphoma (PCNSL) is a rare topographic variant of diffuse large B-cell lymphoma (DLBCL). First-line therapy is based on high-dose methotrexate. Post-induction treatment is recommended in chemotherapy-sensitive patients, but its modalities are matter of debate. While specific prognosis scales are useful in clinical trials, no dynamic prognostic marker is available to consider risk-adapted therapeutic strategies. We report here the prognostic value of early metabolic response by 18F-FDG PET/CT scanner (PET) in 27 newly diagnosed immunocompetent PCNSL patients.Methods: Our patients were treated frontline by 375mg/m² Rituximab and 8g/m² Methotrexate at day 1 and 14, and Temozolomide 150mg/m² days 7 through 11, repeated for four cycles every four weeks (the RMT regimen). Whenever possible they received consolidation therapy by etoposide 40mg/kg on a continuous 96h infusion and cytarabine 2g/m² twice a day during 4 days (the EA regimen), or radiotherapy. Initial PET excluded a systemic localization of lymphoma in all patients. Response to treatment was evaluated according to international PCNSL collaborative group (IPCG) criteria. Brain magnetic resonance imaging (MRI) and PET were performed at diagnosis before any chemotherapy, after two and four cycles of RMT, and after treatment completion.Results: Median age was 68. One patient had concomitant ocular involvement at diagnosis. Twenty patients (74%) completed the 4 cycles of RMT. Among the 7 remaining patients, causes of treatment discontinuation were lymphoma-unrelated death (n=2) and progression (n=5). Then 17 patients underwent a consolidation therapy, by EA in 13 patients, and by radiotherapy in 4 (23.4 Gy, n=3 and 30.4 Gy, n=1). Two patients had a watchful waiting strategy. Median follow-up was 25.4 month (6.1-40.2 month). After two cycles of RMT, 11 (40.7%) patients had a complete response or complete response unconfirmed (CR/CRu), 7 (25.9%) had a partial response (PR), 1 (3.7%) had a progressive disease (PD), 2 (7.4%) had a stable disease (SD) and 5 (18.5%) were not evaluated (NE) due to lymphoma-unrelated death in one and protocol deviation in four. After treatment completion, 19 (70.3%) patients were in CR and 6 (22.3%) had a PD. Two (7.4%) patients were NE due to lymphoma-unrelated death. Positive PET was defined by a new brain lesion or the persistence of 18F-FDG uptake compared to the controlateral parenchyma. Twenty five patients underwent PET after 2 cycle of RMT (PET2). Six (24%) had a positive PET2 (PET2+), and concomitant MRI showed CRu (n=1), PR (n=2), SD (n=2) and PD (n=1). Nineteen patients (76%) had a negative PET2 (PET2-), among whom we observed 10 CR/CRu, 5 PR and 4 NE by MRI. After treatment completion, 4 PET2+ patients (66%) had progressive disease. Only one patient with negative PET2- had a localized intraocular evolution with persistent MRI CR during induction therapy, and one patient in CR died from disease-unrelated cause. Predictive positive and negative values (PPV/PNV) of PET2 on end-of-treatment CR were 66.67% and 94.44%, respectively. During follow-up, one PET2- patient relapsed and another progressed to a fatal lymphoma-related ventriculitis. All SD/PD patients had hypermetabolic lesions, including at relapse. We observed a significant association between PET2 and end-of-therapy response (p=0.0004) and progression free survival (PFS, p=0.0378), while having no significant impact on overall survival (OS, p=0.3699). Two years PFS was 77% (CI 95% 50-91%) and 33% (CI 95% 50-67%) for PET2- and PET2+ patients, respectively (Figure 1). However, PET2 did not retain a significant prognostic value on PFS or OS in multivariate analysis.Conclusion: We assessed for the first time the prognostic value of PET2 in a retrospective series of 27 newly diagnosed immunocompetent PCNSL patients homogeneously treated by the RMT regimen, and observed that PET2 correlated to MRI-assessed response and to PFS. PET2 negativity predicted a favorable outcome in all but one case (ocular relapse), suggesting that early metabolic response may identify chemosensitive patients, as demonstrated in systemic lymphoma. Future PCNSL clinical trials might therefore integrate PET in risk-stratified therapeutic strategies. [Display omitted] DisclosuresNo relevant conflicts of interest to declare.

Intravenous high‐dose methotrexate based systemic therapy in the treatment of isolated primary vitreoretinal lymphoma: An LOC network study

The treatment of primary vitreoretinal lymphoma (PVRL) remains controversial regarding the use of local, systemic, or combined treatments. The aim of this study was to analyze the efficacy and toxicity of intravenous high-dose methotrexate (IV HD-MTX) based systemic therapy in a uniformly treated population of PVRL patients. From a nationwide French database, we retrospectively selected 59 patients (median age: 70 years, median Karnofsky Performance Status: 90%) with isolated PVRL at diagnosis who received first-line treatment with HD-MTX between 2011 and 2018. 8/59 patients also received a local treatment. No deaths or premature discontinuations of MTX due to toxicity were reported. A complete response was obtained in 40/57 patients after chemotherapy. Before treatment, IL-10 was elevated in the aqueous humor (AH) or in the vitreous in 89% of patients. After treatment, AH IL-10 was undetectable in 87% of patients with a CR/uCR/PR and detectable in 92% of patients with PD/SD. After a median follow-up of 61 months, 42/59 (71%) patients had relapsed, including 29 isolated ocular relapses as the first relapse and a total of 22 brain relapses. The median overall survival, progression-free survival, ocular-free survival and brain-free survival were 75, 18, 29 and 73 months, respectively. IV HD-MTX based systemic therapy as a first-line treatment for isolated PVRL is feasible, with acceptable toxicity, even in an elderly population. This strategy seems efficient to prevent brain relapse with prolonged overall survival. However, the ocular relapse rate remains high. New approaches are needed to improve local control of this disease, and ocular assessment could be completed by monitoring AH IL-10.

Tubulointerstitial nephritis and uveitis syndrome: a systematic review.

Tubulointerstitial nephritis and uveitis (TINU) syndrome is defined as the occurrence of tubulointerstitial nephritis (TIN) and uveitis in the absence of other systemic diseases. The most comprehensive review on this condition was published in 2001. We conducted a systematic review of the literature for cases of TINU syndrome. MEDLINE and Embase databases were screened. Full-length articles or letters reporting cases with both TIN and uveitis were selected. We investigated differences between males and females and paediatric and adult cases. Multivariate analysis was performed to identify potential risk factors for chronic kidney disease (CKD) development. A total of 233 articles reporting 592 TINU cases were retained for the analysis. The median age of the included subjects was 17 years (interquartile range 13-46) with a female predominance (65%). Uveitis most frequently (52%) followed renal disease and was mostly anterior (65%) and bilateral (88%). Children tended to have more ocular relapses, while they were slightly less likely than adults to suffer from acute kidney injury and to develop CKD. Adult age as well as posterior or panuveitis were associated with an increased risk of developing CKD. TINU affects both children and adults, with some differences between these two categories. Adult age and the presence of a posterior uveitis or panuveitis appear to be associated with the development of CKD.

Outcomes of intravitreal methotrexate to salvage eyes with relapsed primary intraocular lymphoma

PurposeTo report the outcomes of intravitreal methotrexate (MTX) injections to rescue eyes with relapsed primary intraocular lymphoma (PIOL).MethodsRetrospective case series of patients with ocular relapse of PIOL who had initially...

Adalimumab effectively controls both anterior and posterior noninfectious uveitis associated with systemic inflammatory diseases: focus on Behçet's syndrome.

To compare the efficacy of Adalimumab (ADA) in noninfectious anterior uveitis (AU) and posterior segment (PS) involvement, associated with different conditions, with a focus on Behçet's syndrome (BS). In this retrospective, multicenter post-hoc study, we evaluated the efficacy of ADA in terms of ocular control and relapses in 96 patients with AU and PS uveitis, either idiopathic (IU) or associated with BS or with other systemic disorders (OSD) (Juvenile Idiopathic Arthritis, Psoriatic Arthritis, Ankylosing Spondylitis, Vogt-Koyanagi-Harada, Inflammatory Bowel Disease), followed in three tertiary referral centers. Ninety-six patients (45 AU; 51 PS uveitis) were included. Eleven had IU, 58 BS, and 27 OSD. All patients with AU achieved complete long-term ocular control. In PS uveitis, 89%, 67% and 100% of patients with BS, IU and OSD achieved ocular control at the last follow-up (> 12months), respectively. The lowest ocular relapse rate occurred in patients with AU with BS (1/13) or IU (0/2). ADA accounted for long-term disease control, and no predictors of ocular control and relapse were identified; particularly, ocular relapses seemed not related to systemic ones. Macular edema resolved in 75% and 67% of PS uveitis with BS and IU, respectively. ADA controls both anterior and posterior uveitis, with an efficacy similar in IU, BS and OSD patients. In BS, the efficacy of ADA seems to be independent of demographic and clinical characteristics, and ocular relapses mostly occurred independently from systemic ones. Based on our results, ADA may represent a valid alternative in anterior refractory uveitis.

EVALUATION OF TUMOR NECROSIS FACTOR INHIBITOR THERAPY IN SUSAC SYNDROME.

To evaluate the effect of tumor necrosis factor (TNF) inhibitor therapy on ocular relapses in patients with Susac syndrome. Multicenter retrospective cohort study of patients diagnosed with Susac syndrome according to classical clinical criteria. We evaluated the disease activity before and after introduction of anti-TNF therapy and its value as a steroid-sparing agent. Five patients were included. All were initially treated with a combination of corticosteroids and classical immunosuppressive drugs. Infliximab was started in three patients, and adalimumab was started in two patients. Patients had on average 5 ocular relapses during a mean follow-up time of 2.59 years before introducing a TNF inhibitor, corresponding with on average 1.93 relapses per year. After the introduction of an anti-TNF agent, this number was reduced by factor 5.51 to an average of 0.35 relapses per year for a mean follow-up of 2.86 years (P = 0.10). Before anti-TNF introduction ocular relapses occurred at a mean daily dose of 34 mg of prednisone, whereas with anti-TNF treatment, corticosteroid administration could be completely stopped in four patients with one patient still needing 5 mg daily (P = 0.10). Infliximab and adalimumab generally were well tolerated, and no serious adverse events were reported. Although not statistically significant, our results suggest that anti-TNF therapy can be a valuable option for the treatment of ocular Susac syndrome and may especially be considered in those patients unresponsive to more conventional immunosuppressive treatment.

Optical Coherence Tomography Angiography Findings in Active and Inactive Ocular Behçet Disease

ABSTRACT Purpose To study macular microvascular changes in ocular Behçet disease (OBD) using optical coherence tomography angiography (OCTA). Methods Quantitative and qualitative analyses of OCTA were performed on 23 OBD patients with active or inactive uveitis and compared with healthy controls. Results Deep capillary plexus (DCP) is the most frequently involved in OBD (p < 0.001). Its vessel density (VD) is reduced compared with controls in both active (p < 0.007) and inactive uveitis (p = 0.03). In inactive uveitis, VD is inversely related to the number of uveitis relapses (superficial capillary plexus: r = –0.694, p = 0.004; DCP: r = –0.541, p = 0.037) and it is significantly reduced in patients with a uveitis-free period ≥5 years compared with healthy controls (p < 0.038). Conclusions Macular VD is reduced in Behçet patients with active and inactive uveitis, especially in DCP. In inactive uveitis, VD is inversely related to the number of ocular relapses and cannot be restored during time.

Rare case of ocular adnexal relapse with mantle cell lymphoma treated with ibrutinib monotherapy

Rare case of ocular adnexal relapse with mantle cell lymphoma treated with ibrutinib monotherapy