Systemic Immunomodulatory Therapy Research Articles

Mucous Membrane Pemphigoid with Ocular Involvement: The Clinical Phenotype and Its Relationship to Direct Immunofluorescence Findings

This study explored the validity of the First International Consensus on Mucous Membrane Pemphigoid (MMP) guidance, which recommends that clinically indistinguishable patients, who have direct immunofluorescence (DIF)-negative biopsies, be excluded from a diagnosis of MMP. Misdiagnosis, or delayed diagnosis, of MMP with ocular involvement leads to the inappropriate use of topical therapy, the standard of care for causes of cicatrising conjunctivitis other than MMP, rather than systemic immunomodulatory therapy, resulting in irreversible clinical deterioration in patients with MMP. Prospective, cross-sectional study. Patients meeting the clinical criteria of ocular MMP, including those with positive and negative DIF findings. A case report form was used to collect the demographic details, the clinical history, and the results of a detailed clinical assessment by ophthalmologists, otolaryngologists, dermatologists, and oral medicine specialists. All anatomic sites potentially affected by MMP were examined apart from the esophagus (and larynx in a subset). The DIF results were recorded. Differences between DIF-positive and -negative patients in demography, sites of involvement, and disease severity as determined by the degree of conjunctival scarring (using Tauber staging), central corneal disease (vascularization, scarring, ulceration, and conjunctivalization), history of conjunctival or lid surgery, and requirement for systemic immunotherapy at the time of screening. A total of 73 patients with ocular MMP were recruited, of whom 20 of 73 (27.4%) had ocular-only disease. There was no significant demographic or clinical difference between patients with positive and negative DIF results. This finding included differences in disease severity for which the only significant difference was that of more severe central corneal disease in DIF-negative patients. Asymptomatic disease at different sites was frequent. These findings do not support the classification of DIF-negative patients, meeting the clinical criteria for ocular MMP, as having a different disease. This category of patients should be accepted as having DIF-negative MMP, for clinical management purposes, with patients having inflamed eyes being treated with systemic immunomodulatory therapy. The frequent finding of asymptomatic ocular, oral, and nasopharyngeal MMP is clinically significant and implies that these sites should be routinely screened in asymptomatic patients.

Drug-induced Rowell syndrome, a rare and difficult to manage disease: A case report.

Rowell syndrome is defined as the association between lupus erythematosus, erythema multiforme-like lesions and characteristic immunological changes including positive tests for rheumatoid factor, speckled antinuclear antibody, positive anti-Ro or anti-La antibodies. The present report presents the case of a 45-year-old female patient who was previously diagnosed in January 2010 with terbinafine-induced subacute cutaneous lupus erythematosus and was admitted for a skin eruption consisting of erythematous-papular erythema multiforme-like lesions, primarily on the trunk and limbs. The associated symptoms consisted of fatigability, myalgia and gonalgia. In October 2015, the illness reoccurred ~1 week after the initiation of Helicobacter pylori eradication treatment. Anti-Ro antibodies, rheumatoid factor and antinuclear antibody tests were positive. Given the patient's medical history, clinical manifestations, and laboratory, histopathological and immunofluorescence microscopy findings, a diagnosis of Rowell syndrome was made. Systemic corticosteroids (methylprednisolone; 0.5 mg/kg/day) and immunomodulatory therapy (azathioprine; 50 mg/day) were administered with the associated medication (omeprazole, 20 mg/day; KCl, 1 g/day) and topical dermocorticoids (fluticasone propionate 0.05% cream; 1 application/day), with a favorable outcome. The major diagnostic criteria for Rowell syndrome are the presence of lupus erythematosus (acute, subacute or systemic), erythema multiforme-like lesions and positive testing for antinuclear antibodies. The minor diagnostic criteria for Rowell syndrome are chilblains, the presence of anti-Ro antibodies and positive testing for rheumatoid factor. A diagnosis of Rowell syndrome is made if the patient exhibits all major criteria and at least one minor criterion. The present case met all diagnostic criteria, excluding the presence of chilblains. Notably, in this case there was a co-occurrence of subacute lupus erythematosus and Rowell syndrome lesions, which was drug-induced.

Blau Syndrome–Associated Uveitis: Preliminary Results From an International Prospective Interventional Case Series

Provide baseline and preliminary follow-up results in a 5-year longitudinal study of Blau syndrome. Multicenter, prospective interventional case series. Baseline data from 50 patients from 25 centers worldwide, and follow-up data for patients followed 1, 2, or 3 years at the end of study enrollment. Ophthalmic data were collected at baseline and yearly visits by means of a standardized collection form. Median age at onset of eye disease was 60months and duration of eye disease at baseline 145months. At baseline 38 patients (78%) had uveitis, which was bilateral in 37 (97%). Eight patients (21%) had moderate to severe visual impairment. Panuveitis was found in 38 eyes (51%), with characteristic multifocal choroidal infiltrates in 29 eyes (39%). Optic disc pallor in 9 eyes (12%) and peripapillary nodules in 9 eyes (12%) were the commonest signs of optic nerve involvement. Active anterior chamber inflammation was noted in 30 eyes (40%) at baseline and in 16 (34%), 17 (57%), and 11 (61%) eyes at 1, 2, and 3 years, respectively. Panuveitis was associated with longer disease duration. At baseline, 56 eyes (75%) were on topical corticosteroids. Twenty-six patients (68%) received a combination of systemic corticosteroids and immunomodulatory therapy. Blau uveitis is characterized by progressive panuveitis with multifocal choroiditis, resulting in severe ocular morbidity despite continuous systemic and local immunomodulatory therapy. The frequency and severity of Blau uveitis highlight the need for close ophthalmologic surveillance as well as a search for more effective therapies.

Treatment Results in Serpiginous Choroiditis and Multifocal Serpiginoid Choroiditis Associated with Latent Tuberculosis

Objectives:To compare the results of systemic antitubercular therapy (ATT) and immunomodulatory therapy (IMT) in patients with serpiginous choroiditis (SC) or multifocal serpiginoid choroiditis (MSC).Materials and Methods:The clinical records of 28 patients with SC and MSC were reviewed. Patients were divided into 2 groups according to the treatment applied. Group 1 included 12 patients with MSC and 5 with SC treated with ATT and corticosteroid (CS); group 2 included 9 patients with MSC and 2 with SC treated with conventional IMT, interferon alpha-2a, and/or CS monotherapy.Results:In group 1, clinical remission was achieved in 12/12 MSC and 3/5 SC (total 15/17) patients with administration of ATT for 1 year. Two patients (1 SC, 1 MSC) had reactivation 2 and 7 months after cessation of ATT. Two patients with recurrence after completion of ATT and 2 patients resistant to ATT received IMT ± CS therapy. In group 2, clinical remission was achieved in 7/9 MSC and 2/2 SC (total 9/11) patients after 1 year of treatment. Recurrent inflammation was observed in 2 MSC patients 2 and 112 months after initiation of therapy, but responded well to local/systemic CS or IMT modification, and clinical remission was achieved in 7.8±4.3 months. Cumulative dose of CS was higher in group 2 (p=0.057). Nine of 12 MSC patients treated with ATT and 4/9 MSC patients treated with IMT achieved remission (p=0.203). One of 5 SC patients treated with ATT and 2/2 SC patients treated with IMT achieved remission (p=0.142).Conclusion:Although a statistically significant result could not be achieved in this small case series, our results suggest that ATT may be an appropriate first choice in the treatment of MSC associated with latent tuberculosis, and may be administered in patients with SC who are unresponsive to IMT.

The 2016 Bowman Lecture Conjunctival curses: scarring conjunctivitis 30 years on.

This review is in two sections. The first section summarises 35 conditions, both common and infrequent, causing cicatrising conjunctivitis. Guidelines for making a diagnosis are given together with the use of diagnostic tests, including direct and indirect immunofluorescence, and their interpretation. The second section evaluates our knowledge of ocular mucous membrane pemphigoid, which is the commonest cause of cicatrizing conjunctivitis in most developed countries. The clinical characteristics, demographics, and clinical signs of the disease are described. This is followed by a review and re-evaluation of the pathogenesis of conjunctival inflammation in mucous membrane pemphigoid (MMP), resulting in a revised hypothesis of the autoimmune mechanisms causing inflammation in ocular MMP. The relationship between inflammation and scarring in MMP conjunctiva is described. Recent research, describing the role of aldehyde dehydrogenase (ALDH) and retinoic acid (RA) in both the initiation and perpetuation of profibrotic activity in MMP conjunctival fibroblasts is summarised and the potential for antifibrotic therapy, using ALDH inhibition, is discussed. The importance of the management of the ocular surface in MMP is briefly summarised. This is followed with the rationale for the use of systemic immunomodulatory therapy, currently the standard of care for patients with active ocular MMP. The evidence for the use of these drugs is summarised and guidelines given for their use. Finally, the areas for research and innovation in the next decade are reviewed including the need for better diagnostics, markers of disease activity, and the potential for biological and topical therapies for both inflammation and scarring.

Intravitreal Dexamethasone Implant for the Treatment of Macular Edema in Chronic Non-infectious Uveitis

ABSTRACTPurpose: To report observations on the single and repeat use of the dexamethasone (DEX) intravitreal implant (Ozurdex; Allergan, Inc, Irvine, CA) for the treatment of macular edema in patients with non-infectious posterior segment uveitis.Methods: A chart review of 15 consecutive patients (25 eyes) was conducted. The primary outcome measure of the first and subsequent implants was central retinal thickness (CRT) on spectral-domain optical coherence tomography (sdOCT). Secondary outcomes were best-corrected visual acuity (BCVA), time to repeat implant, and adverse events. Multilevel mixed-effects linear regression was used to determine the effect of the DEX implant compared with baseline. The Kaplan–Meier estimator was used to examine survival from relapse.Results: A total of 35 implants on 25 eyes of 15 patients were included in the analysis. Of these, 91.4% (32 of 35 eyes) had a reduction in CRT and 80% (20 of 25 eyes) had improved BCVA. After the first DEX implant, CRT decreased from 590 µm (SE: 28 µm) at baseline to 370 µm (SE: 31 µm) at 3 months (p < 0.001). The logMAR VA was 0.614 (SE: 0.089) at baseline and improved to 0.35 (SE: 0.10, p = 0.002), reaching a statistically significant difference at 3 months. A repeat implant led to VA improvement of –0.184 logMAR (SE: 0.171 logMAR) and CRT reduction of –291 µm (SE: 74 µm). There was no significant difference in effect between the first repeat implant and the initial implant. Kaplan–Meier estimates of treatment success were 72% between 3 and 6 months.Conclusions: The DEX implant is an effective adjunct treatment to systemic corticosteroid or immunomodulatory therapy. Additional research is required to determine the efficacy of DEX implant as monotherapy for controlling chronic uveitic macular edema.

Impact of Connective Tissue Disease on Oncologic Breast Surgery and Reconstruction.

The impact of connective tissue disease (CTD) on outcomes following breast surgery and reconstruction is unknown. The purpose of this study was to evaluate the effect of both CTDs and systemic immunomodulatory therapy on outcomes following breast surgery and reconstruction. A retrospective review was performed of all patients from 2005 to 2010 with an active CTD who underwent breast surgery with or without reconstruction. Surgical events were assigned to 1 of 4 groups: ablative surgery alone, autologous reconstruction, implant reconstruction, and revision surgery. Logistic regression was utilized to examine the relationship between complications and type of surgery, CTD diagnosis, and immunomodulatory therapy. Four non-CTD control groups were then compiled for outcome comparison. The a priori P-value was set at P < 0.05, and all tests were 2 sided. Thirty-three patients with CTD underwent112 procedures. Diagnoses included psoriasis/psoriatic arthritis (n = 12), rheumatoid arthritis (n = 10), lupus (n = 4), scleroderma (n = 3), Sjogren syndrome (n = 2), mixed CTD (n = 1), and seronegative polyarthritis (n = 1). Nineteen of 33 (58%) patients who received systemic treatment for CTD in the perioperative period were less likely to experience a minor complication compared with those without treatment (odds ratio= 0.69; P = 0.019). There were no differences in postoperative complications in patients with CTD compared with control groups. Ablative breast surgery and reconstruction among patients with CTDs can be performed safely with low perioperative complication rates. Patients receiving systemic therapy, and continuing their regimens perioperatively, experience complication rates similar to those not requiring therapy.

Interleukin-22 serum levels are elevated in active scleritis.

To evaluate serum cytokine profile from patients with active scleritis in a two-centre prospective case-control study. The serum of 20 active scleritis patients not treated with any local, periocular, or systemic immunomodulatory therapy (IMT) was analysed with multiplex assay to determine the levels of 11 cytokines interleukin (IL)-1β, IL-6, IL-2, IFN-γ, IL-10, IL-12p40, IL-13, IL-17A, IL-5, TNF-α, and TNF-β, and with ELISA to determine the levels of TGF-β1, IL-22, and IL-23. Twenty-five age-matched healthy volunteers were used as controls. In a subgroup of 13 patients with active disease, a second serum sample was obtained when the disease was inactive and levels of IL-22 were determined. Serum IL-22 levels from patients with active scleritis were correlated with type of scleritis (non-necrotizing and necrotizing), degree of inflammation (0-4+:≤2+ and >2+), and associated systemic disease. Serum levels of IL-22 were elevated in active scleritis patients compared to controls (6.41±1.52pg/ml versus 1.93±0.39pg/ml, p=0.012) and significantly decreased after scleritis remission with the use of IMT (p=0.005). There was no statistical association with scleritis type, degree of inflammation, or associated systemic disease. The serum levels of other cytokines were not significantly different from controls. In our study cohort, IL-22 serum levels were significantly elevated in active scleritis patients compared to controls and decreased significantly after remission. Our results suggest that IL-22, a T helper (Th) 17- and Th22- derived cytokine, may play a critical role in the physiopathology of scleritis.

A Case of Focal Laser Photocoagulation Added to Systemic Immunomodulatory Therapy for Bilateral Lupus Chorioretinopathy

A Case of Focal Laser Photocoagulation Added to Systemic Immunomodulatory Therapy for Bilateral Lupus Chorioretinopathy

Foveal Serous Retinal Detachment in Juvenile Idiopathic Arthritis-associated Uveitis

Purpose: In juvenile idiopathic arthritis (JIA)-associated uveitis complicated by foveal serous retinal detachment (FSRD), we documented the relationship between best corrected visual acuity (BCVA), level of anterior chamber flare and OCT features.Methods: Nine children (15 eyes) with FSRD were identified among 38 children with JIA-associated maculopathy. Outcome measures included BCVA, ocular inflammatory activity quantified by laser flare photometry and the macular profile analyzed by OCT.Results: The diagnosis of FSRD led to intensification of the treatment using subtenon’s injection of triamcinolone or systemic immunomodulatory therapy. The improvement of BCVA at presentation (0.46 logMAR) was significant at 36 months follow-up (0.15 logMAR). The resolution of FSRD along with visual improvement (p = 0.0032) correlated with improvement in anterior chamber flare (p = 0.01).Conclusion: FSRD is a complication of chronic JIA-associated uveitis that responds well to intensification of immunomodulation. Visual improvement is correlated with FSRD resolution and with flare photometry values.

Therapy of pyoderma gangrenosum in Germany: results of a survey among wound experts.

There are currently no therapeutic guidelines with respect to pyoderma gangrenosum (PG). In order to systematically record currently practiced therapeutic options for PG, a questionnaire was developed and sent to experts at various German dermatology departments. Overall, 50 fully completed questionnaires were returned to us. On average, the diagnosis of PG is made 15 times per year. Of these patients, 11.1 % receive only topical therapy; 22.7 %, exclusively systemic therapy; and 66.2 %, both topical and systemic immunomodulatory therapy. Topically, the experts most commonly use class III (78 %) and class IV corticosteroids (88 %). All experts (100 %) reported the frequent use of systemic corticosteroids in the therapy of PG patients. Cyclosporine is used in 74 %, TNF-α inhibitors in 24 %, and azathioprine or mycophenolate mofetil each in 20 % of patients. Systemic agents such as dapsone (16 %), intravenous immunoglobulins (14 %), methotrexate (6 %), cyclophosphamide (4 %), tacrolimus (4 %), and rituximab (2 %), however, are given less frequently. Worldwide, no uniformly accepted standard is available for the treatment of patients with PG. In our survey among experts, treatment regimens currently used in Germany, and based on expert clinical experience, have now been recorded, analyzed, and benchmarked for the first time.

Dexamethasone intravitreal implant (Ozurdex) for the treatment of pediatric uveitis

To report our experience using Ozurdex (Allergan, Irvine, CA), a biodegradable intravitreal implant containing of 0.7 mg of dexamethasone approved for use in adults with noninfectious uveitis in adults, in the treatment of pediatric uveitis. The medical records of consecutive patients with noninfectious posterior uveitis who were unresponsive to standard treatment and subsequently received the Ozurdex implant from March 2011 to March 2013 were retrospectively reviewed. A total of 14 eyes of 11 patients (mean age, 10.1 years; range 4-12) received 22 Ozurdex implants during the study period. Of the 11 patients, 7 had idiopathic intermediate or posterior uveitis, 1 had sympathetic ophthalmia, 2 had juvenile idiopathic arthritis, and 1 had sarcoidosis. All patients were uncontrolled with standard treatment, including topical or sub-Tenon's or systemic corticosteriods and/or immune-modulation. Visual acuity improved after Ozurdex implant in 5 of 8 patients (63%). Intraocular inflammation was controlled or improved after 17 of 22 of implants (12 eyes [77%]). The frequency of topical corticosteroids was decreased and/or discontinued after 18 of 22 implants (12 eyes [82%]). Complications included implant migration into the anterior chamber (4 aphakic eyes), increased intraocular pressure (5 eyes), and progression of a preexisting cataract (1 eye). The uveitis reoccurred in 57% of eyes at 4.3 months (2-7 months) after injection. The Ozurdex implant in combination with systemic immunomodulatory therapy resulted in improved visual acuity, control of intraocular inflammation, and a decrease in corticosteroid use. In the majority of eyes the uveitis reoccurred around 4 months after injection. The adverse events in our study are similar to those identified in adult studies.

Improved Efficacy of Radiation in Combination With Systemic Immunomodulatory Therapy in a Syngeneic Mouse Model of Colorectal Cancer

Improved Efficacy of Radiation in Combination With Systemic Immunomodulatory Therapy in a Syngeneic Mouse Model of Colorectal Cancer

Efficacy and safety of TNF-α inhibitors in refractory primary complex aphthosis: a patient series and overview of the literature

Background: Otherwise healthy patients with severe recurrent mucocutaneous aphthous ulcerations (complex aphthosis) may require systemic immunomodulatory therapy. However, a subset of patients remain resistant or intolerant to recommended therapeutic agents. Recently, case reports have described that tumor necrosis factor-α (TNF-α) inhibitors may induce remission in these patients. Methods: Data on efficacy and safety of various TNF-α inhibitors used as monotherapy in a case series of 18 patients with refractory primary complex aphthosis are presented. Results: A total of 16 patients (89%) obtained complete or almost clearance of orogenital aphthous ulcerations rapidly after onset of therapy either with etanercept, adalimumab, infliximab or golimumab. Duration of treatment ranged between 3 and 77 months. Nine patients (50%) received more than one TNF-α inhibitor during the course of treatment. Five (28%) patients experienced side effects that could be related to treatment with TNF-α inhibitors. Conclusion: TNF-α inhibitors are an effective and safe treatment option for patients with severe complex aphthosis who do not respond sufficiently to standard therapy as recommended by existing guidelines. However, the final position of TNF-α inhibitors in the therapeutic armamentarium awaits randomized controlled trials.

Risk Factors for Temporomandibular Joint Arthritis in Children with Juvenile Idiopathic Arthritis

To determine the prevalence and features of temporomandibular joint (TMJ) arthritis by magnetic resonance imaging (MRI) among children with juvenile idiopathic arthritis (JIA), and to identify risk factors for TMJ arthritis. A retrospective chart review was performed on 187 patients with JIA who underwent a TMJ MRI at Children's Hospital of Alabama between September 2007 and June 2010. Demographic and clinical information was abstracted from the charts. Univariate and multivariate analyses were performed to identify risk factors for TMJ arthritis identified by MRI. MRI evidence of TMJ arthritis was detected in 43% of patients, with no significant difference among JIA categories. The number of joints with active arthritis (exclusive of the TMJ) and the use of systemic immunomodulatory therapies were not associated with TMJ arthritis. Multivariable analysis revealed a strong association between mouth-opening deviation and TMJ arthritis (OR 6.21, 95% CI 2.87-13.4). A smaller maximal incisal opening and shorter disease duration were also associated with an increased risk of TMJ arthritis. TMJ arthritis was identified in a substantial proportion of children with JIA (43%) and affects all JIA categories. TMJ arthritis was present in some patients despite limited or otherwise quiescent disease and in the presence of concurrent systemic immunomodulatory therapy. Routine evaluation for TMJ arthritis by MRI is warranted for all children with JIA.

Pyoderma gangraenosum als Major-Komplikation bei einer Brustrekonstruktion mit freier Doppel-DIEP-Lappenplastik

Pyoderma gangraenosum (PG) as an idiopathic, ulcerative inflammatory skin disease with varying clinical pictures has an estimated incidence of 0.3/100,000 and is due to the non-specific histology primarily a clinical exclusion diagnosis. The rapid progression, as well as the often fatal course of the pathergy phenomenon after surgical intervention is a therapeutic challenge. With the example of the complicated course of a 47-year-old patient with free DIEP flap for bilateral breast reconstruction due to PG, we want to direct attention to this often underrated disease. For bilateral breast reconstruction, a 47-year-old patient received a double free DIEP flap, which had to be resected on the right due to multiple arterial thromboses. The clinical presumptive diagnosis of a PG arose and an IV steroid therapy was started immediately. After 13 days a further debridement and mesh graft transplantation were performed. Now, the histological examination was for the first time compatible with PG. Under the initiated steroid treatment the wounds could be stabilised and the remaining defects could be covered. After 6-week stay, the patient was finally discharged with healed wound conditions, but an unsatisfactory aesthetic result. If unclear, rapidly progressive ulcers occur in surgical interventions one should always think of pyoderma gangraenosum. In particular, since a surgical intervention can result in an uncontrolled exacerbation due to the pathergy phenomenon. Laboratory parameters and histology are not specific. Under a systemic immunomodulatory therapy, a sequential surgical wound management may accelerate wound healing.

Clinical Manifestations of Mucous Membrane Pemphigoid in a Tertiary Center

Mucous membrane pemphigoid (MMP) is a progressive inflammatory disease of autoimmune etiology. We performed a retrospective analysis of clinical signs and treatment on 16 patients. Conjunctival biopsies were performed in all patients and showed typical immuno-deposits at the basement membrane zone. The mean age at presentation was 69 years, 60 % were female.12 patients demonstrated ocular involvement (11 bilaterally). At the time of referral to our hospital, 92 % had reached an advanced stage III or IV. All patients presented conjunctival fibrosis with resultant fornix foreshortening. Trichiasis and symblepharon were found in 11 patients. Keratitis was found in 11 patients resulting in ulceration in 5 cases. Complications required surgical interventions included: entropion surgery (n = 2), tarsorrhaphy (n = 1), amniotic membrane transplantation (n = 2), keratoplasty (n = 1). Systemic immunomodulatory therapy is the treatment of choice. Dapsone (n = 8), steroids (n = 8), azathioprine (n = 5), cyclophosphamide (n = 2), mycophenolate mofetil (n = 4) and methotrexate (n = 1) were used concomitantly or consecutively. Early diagnosis can prevent ocular complications. Immunomodulatory therapy has provided an avenue for preserving vision. The management of MMP requires a multidisciplinary approach.

Efficacy and Potential Complications of Difluprednate Use for Pediatric Uveitis

To evaluate the clinical effect of topical difluprednate in pediatric patients for treatment of noninfectious uveitis. Retrospective, observational case series. Twenty-six eyes of 14 pediatric patients with noninfectious uveitis who were treated with topical difluprednate were evaluated. Anterior and posterior cell grade, visual acuity, intraocular pressure (IOP), and cystoid macular edema (CME) were recorded at each visit. Main outcome measures were changes in anterior segment cell, CME, visual acuity, and IOP and development of a visually significant cataract. A significant (≥ 2-grade decrease or decrease to 0 in anterior segment cell) reduction in anterior segment inflammation was observed during treatment with topical difluprednate in 88% of eyes (22/25) when used as an adjuvant to systemic immunomodulatory therapy. In addition, improvement in CME associated with uveitis was seen in response to topical therapy with difluprednate in 78% of eyes with CME (7/9). A significant IOP response (IOP increase of ≥ 10 mm Hg from baseline and IOP ≥ 24 mm Hg) was seen in 50% of eyes (13/26) and in 50% of patients (7/14); 3 eyes of 2 patients required glaucoma surgery. Cataract formation or progression was observed in 39% of eyes (10/26) and in 43% of patients (6/14); 5 eyes of 3 patients required cataract surgery. Difluprednate is an effective agent for both control of anterior segment inflammation and reduction of CME in pediatric patients with uveitis when used as an adjuvant to systemic immunomodulatory therapy. A high rate of steroid-induced IOP elevation and cataract formation is seen in this population. Close monitoring of pediatric patients receiving difluprednate is recommended.

Clinical Features and Prognosis of Boston Type I Keratoprosthesis-associated Corneal Melt

Purpose: To describe the clinical features and outcomes of corneal melt associated with Boston type I keratoprosthesis (KPro) implantation.Methods: Medical records of patients who experienced corneal melt following KPro implantation were reviewed retrospectively.Results: Sixty-six adult patients had KPro implantation from January 2004 to November 2010. Six patients had an underlying inflammatory ocular surface disorder. Four experienced corneal melt (6.1%) 5–42 months after the initial surgery. One patient was diagnosed with Sjögren’s syndrome as a result of diagnostic workup following melt. Three patients were treated with systemic immunomodulatory therapy; two experienced fungal keratitis and subsequent endophthalmitis. KPro had to be explanted and replaced with donor cornea in all cases.Conclusions: KPro-associated corneal melt is uncommon and appears to occur in patients with preexisting inflammatory disorders, which might not have been previously diagnosed. Timely explantation of KPro and replacement with donor cornea may prevent a poor outcome.

Diagnostic Testing and Disease Monitoring in Birdshot Chorioretinopathy

Birdshot chorioretinopathy (BSCR) is a rare form of posterior uveitis in which hypopigmented choroidal lesions are scattered throughout the posterior pole. In order to avoid the poor natural history of BSCR, many practitioners would argue that it is critical to diagnose and carefully monitor the often subtle activity of this disease; BSCR can progress insidiously in a white and painless eye, and treatment algorithms based on visual acuity, vitreous inflammation, and retinal vascular leakage of fluorescein alone have been ineffective. This article reviews the various modalities that can be used to diagnose and monitor BSCR, including the clinical and ophthalmoscopic features, diagnostic criteria, electroretinography (ERG- full field, multifocal, and pattern), fluorescein angiography (FA), indocyanine green (ICG) angiography, optical coherence tomography (OCT), fundus autofluorescence (AF), visual fields, HLA A29 testing, and other laboratory testing. HLA-A29 testing can be useful in diagnostically borderline cases, but a positive test is not as useful as one might think in cases where the clinical suspicion for BSCR is low. Out of all the testing modalities, the ERG has been studied most extensively in its relationship to successful treatment. The key parameter is the 30 Hz flicker implicit time, which is abnormal in 70% of patients at baseline. A normal implicit time is correlated with the chance that a patient can be successfully tapered from systemic immunomodulatory therapy without recurrence. Alternatively, some practitioners use ICG angiography or visual field testing for adjunctive monitoring. OCT is used most commonly to follow macular edema. While there is no consensus on how to best monitor disease activity, our institution uses serial ERGs as an adjunct to the normal exam.