Ocular Sarcoidosis Research Articles

Altered retinal oxygen metabolism in patients with combined ocular and central nervous system sarcoidosis.

To evaluate retinal oxygen metabolism by retinal oximetry for ocular and CNS diseases in a cross-sectional study of sarcoidosis. Overall 201 eyes from 103 biopsy-verified sarcoidosis patients were included and divided into four groups depending on the organ affection: (i) sarcoidosis without ocular or CNS affection, (ii) ocular sarcoidosis, (iii) CNS sarcoidosis, and (iv) combined ocular and CNS sarcoidosis. Retinal oximetry was obtained and analysed, with the mean retinal arteriolar and venular saturation as well as arteriovenous difference as principal outcomes. Comparison between groups was done in a multi linear regression model adjusted for age, sex, duration of sarcoidosis, best corrected visual acuity and retinal oximetry quality. Mean (s.d.) age was 50.5 (13.4) (95% CI: 47.9, 53.1) years and 52.2% were males. Eyes of the combined Ocular/CNS group had a higher retinal arteriovenous difference than eyes of the Non-ocular/no-CNS group (42.1% vs 37.7%, P = 0.012) but did not differ between other groups. Eyes in the four groups (Non-ocular/no-CNS, Ocular, CNS and Ocular/CNS) did not differ according to retinal arterial (94.5%, 93.5%, 93.5% and 94.5%, respectively) or venular (57.5%, 56.4%, 55.0% and 52.5%, respectively) oxygen saturation. The results of this study suggest that eyes of sarcoidosis patients with combined ocular and CNS affection have an altered oxygen metabolism indicating a subclinical eye affection that is not recognized by conventional screening methods.

Factors Associated with Ocular and Extraocular Recovery in 143 Patients with Sarcoid Uveitis.

Background: Sarcoidosis is one of the leading causes of uveitis. To date, no studies have assessed the factors specifically related with recovery in ocular sarcoidosis. In this study, we aimed to determine factors associated with ocular and extraocular recovery in patients with sarcoid uveitis. Methods: A retrospective study of sarcoid uveitis, with a three-year minimum follow-up in Lyon University Hospital between December 2003 and December 2019. Patients presented biopsy-proven sarcoidosis or presumed sarcoid. Recovery was defined by a disease-free status, spontaneously or despite being off all treatments for three years or more. Results: 143 patients were included: 110 with biopsy-proven and 33 with presumed sarcoid uveitis. Seventy-one percent were women, the median age at presentation was 53 years, and 71% were Caucasian. Chronic uveitis was the main clinical presentation (75%), mostly panuveitis (48%) with bilateral involvement (82%). After a median follow-up of 83.5 months, recovery was reported in 26% of patients. In multivariable analysis, Caucasian ethnicity (p = 0.007) and anterior uveitis (p = 0.008) were significantly associated with recovery, while increased intraocular pressure was negatively associated (p = 0.039). Conclusion: In this large European cohort, one quarter of patients recovered. Caucasian ethnicity and anterior uveitis are associated with ocular and extraocular recovery.

Abstract 15578: Propionibacterium Acnes -associated Cardiac Sarcoidosis Presenting as Constrictive Pericarditis

Sarcoidosis is an antigen-mediated chronic multisystem inflammatory disease characterized by the formation of non-caseating epithelioid granulomas that disrupt tissue architecture, trigger fibrosis, and precipitate organ dysfunction. Cardiac involvement portends a poorer prognosis, presenting with conduction disease, heart failure, pulmonary hypertension, valvular disease, pericardial effusions, tamponade, ventricular arrhythmias, and sudden death. A 75-year-old man with a history of heart failure with mildly reduced ejection fraction, suspected ocular sarcoidosis, monoclonal gammopathy of uncertain significance and 6 months of exertional dyspnea and lower extremity edema presented with acute-on-chronic volume overload. His labs were notable for elevated inflammatory markers. Cardiac MRI showed pericardial thickening, complex pericardial fluid. The differential diagnosis included tamponade, restrictive cardiomyopathy or constrictive pericarditis secondary to paraproteinemia, viral/idiopathic, vasculitis, tuberculosis, lupus, or sarcoidosis. Simultaneous right and left heart catheterization was consistent with constriction. The patient underwent surgical pericardiectomy. The pathology was consistent with sarcoidosis and intraoperative cultures grew Propionibacterium acnes. This case reviews the mechanism and diagnosis of sarcoidosis, the diagnosis of constrictive pericarditis, and a possible role for P. acnes in the development of sarcoidosis. Constrictive pericarditis is a rare presentation of cardiac sarcoidosis, particularly when the only other suspected involvement of sarcoidosis is uveitis. P. acnes has been associated with granuloma formation in sarcoid, although the mechanism is still unknown. P. acnes may represent a potential therapeutic target once a mechanism is further elucidated.

Ocular Manifestations of Sarcoidosis in a South Florida Population.

ObjectiveTo describe the ocular manifestations of sarcoidosis in a South Florida population and identify risk factors for the presence of ocular disease.DesignRetrospective consecutive case series.MethodsMedical charts of individuals with sarcoidosis seen in the University of Miami pulmonary department were reviewed for ocular disease. Odds ratios were used to identify risk factors for ocular sarcoidosis.ResultsFourteen of 108 individuals with sarcoidosis had ocular involvement. The mean age of the 14 individuals was 56±15 years. Seventy-one percent were female, 50% were black, and 21% were Hispanic. Twelve had uveitis of which panuveitis was the most common subtype. Five had ≤20/70 vision in at least one eye due to uveitis. Neurosarcoidosis was a risk factor for ocular sarcoidosis (OR 6.14, p=0.03, 95% CI 1.21–31.09).ConclusionOcular manifestations occurred in a minority of individuals in a pulmonary sarcoidosis clinic in South Florida. Uveitis was the most common ocular manifestation. Neurosarcoidosis was a risk factor for ocular involvement.

DIAGNOSING SARCOIDOSIS IN THE EYE OF THE STORM

SESSION TITLE: Fellows Diffuse Lung Disease Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: October 18-21, 2020 INTRODUCTION: Sarcoidosis is a granulomatous disease that typically affects the lungs but can involve all organ systems to a different extent and degree. The diagnosis requires a step wise approach that includes identifying affected organs amenable for biopsy. Up to 30% of patients may present with extra-pulmonary manifestations of sarcoidosis and ocular involvement is seen in up to 25% of these cases. We discuss a case of difficult to diagnose sarcoidosis in a patient with difficult to control asthma that presented with choroidal lesions concerning for ocular lymphoma which prompted additional workup. CASE PRESENTATION: 68 year-old woman with past medical history of difficult-to-treat, severe asthma and obesity with mixed restrictive/obstructive pulmonary functions referred to our institution for a second opinion. She had a long history of asthma since early in childhood that became very difficult to control requiring multiple burst of steroids, use of biologics, and high dose inhaled steroids. She endorsed daily productive cough of white sputum and mild malaise. She denied any other respiratory symptoms. She had undergone an extensive work including serologies and two bronchoscopies with transbronchial and endobronchial biopsies. Chest CT revealed patchy, ill-defined bilateral tree-in-bud opacities without bronchiectasis. Routine exam by her ophthalmologist prompted referral to a regional oncologic ophthalmologist and she was given the presumptive diagnosis of choroidal lymphoma and recommended to undergo choroid biopsy. A PET scan was positive only in the tonsillar beds and biopsy revealed reactive lymphoid hyperplasia. After discussion with ophthalmology the differential diagnosis of choroidal lymphoma, it was decided to proceed to a cryobiopsy of the lung to exclude the possibility of sarcoidosis. She underwent a bronchoscopy and bronchioalveolar lavage revealed 69% lymphocytes with normal flow cytometry. Cryobiopsy revealed non-necrotizing granulomas in a lymphangitic distribution without interstitial inflammation or bronchiolitis. After excluding other causes of granulomatous diseases coupled with eye lesions, patient was diagnosed with systemic sarcoidosis. She was started on oral prednisone with improvement in her respiratory symptoms. DISCUSSION: Sarcoidosis can present with mixed obstructive and restrictive physiology and mimic or exacerbate asthma. It can also present with both anterior and posterior uveitis and is the presenting symptom in 5% of cases. Choroidal and retinal disease can exist without symptoms yet result in blindness. CONCLUSIONS: It is important to recognize this potential presentation of sarcoidosis to start prompt treatment and prevent late complications such as secondary glaucoma and impaired vision. Reference #1: Y. Jamilloux, L. Kodjikian, C. Broussolle, P. Sève. Sarcoidosis and uveitis. Autoimmun Rev. 2014 Aug;13(8):840-9. Epub 2014 Apr 3. Reference #2: B. Bodaghi, V. Touitou, C. Fardeau, C. Chapelon, P. LeHoang. Ocular sarcoidosis. Presse Med, 41 (6 Pt 2) (2012), pp. e349-e354 Reference #3: M. Drent, U. Costabel. European respiratory monograph 32: sarcoidosis. European Respiratory Society (2005) DISCLOSURES: No relevant relationships by Sarah Hackman, source=Web Response No relevant relationships by Jay Peters, source=Web Response No relevant relationships by Jorge Villalpando, source=Web Response

EP21 Tumour necrosis factor inhibitor for the treatment of refractory extra-pulmonary disease including sarcoid arthritis and myositis

EP21 Tumour necrosis factor inhibitor for the treatment of refractory extra-pulmonary disease including sarcoid arthritis and myositis

Recommendations for the management of ocular sarcoidosis from the International Workshop on Ocular Sarcoidosis

AimsTo establish expert recommendations for the management of ocular sarcoidosis (OS).MethodsA question-based survey on the management of OS was circulated to international uveitis experts (members of the International Uveitis Study...

An update on the diagnosis and management of ocular sarcoidosis.

Ocular sarcoidosis is one of the most common causes of uveitis worldwide. The diagnosis and treatment of patients with ocular sarcoidosis remains challenging in some cases. It is important for clinicians to keep up to date with new diagnostic and treatment tools for this disease. The International Workshop on Ocular Sarcoidosis diagnostic criteria were first proposed in 2009 and revised in 2017. The new criteria contained two parts: ocular presentation and systemic investigation. The diagnostic value of liver enzymes was reduced in the new criteria, whereas the value placed of lymphopenia and the CD4/CD8 ratio in bronchoalveolar lavage fluid were increased. Despite not being included in the criteria, recent studies have also highlighted the diagnostic value of serum soluble interleukin-2 receptors. Recent ophthalmologic imaging also provides useful insights for the differential diagnosis.Many new treatments for ocular sarcoidosis have been developed in recent years. The introduction of biological immunomodulatory agents for uveitis treatment represents a big improvement. Antitumor necrosis factor-alpha antibodies, including adalimumab, have been proven to be effective for treating ocular sarcoidosis. Many studies have also suggested that other biological agents could be effective and well tolerated. Newer intravitreal dexamethasone and fluocinolone implants have been developed. Patients treated with these implants have experienced good and sustained control of their intraocular inflammation. Diagnosis and treatment options for ocular sarcoidosis have changed over time. However, challenges still exist in some difficult patients. Future studies should focus on finding more sensitive biomarkers and developing more effective immunomodulatory treatments with longer efficacy and less side effects.

Diagnostic Markers in Ocular Sarcoidosis in A High TB Endemic Population – A Multicentre Study

ABSTRACT Aim To look at markers to differentiate ocular sarcoidosis from ocular tuberculosis in a high TB endemic population. Materials & Methods Retrospective multicenter study involving seven tertiary eyecare centers in India between July 2016 to December 2016. Demographic, clinical and laboratory data were retrieved from respective hospital databases. Group A included biopsy-proven ocular sarcoidosis while group B included ocular tuberculosis. Results Data from 2726 consecutive patients with uveitis were retrieved from the databases. Group A had 61 cases while group B included 307 cases. The mean age in group A was 43 ± 16.55 years while in group B was 38 ± 13.13 years. Females were more common in group A. Uveitis with low Schirmer test (OR-30, CI-95%), candlewax retinal vasculitis (OR-8.69, CI-95%), hilar lymphadenopathy (OR-15.23, CI-95%), fissural nodules (OR-39.11, CI-95%) had higher odds of having ocular sarcoidosis. Conclusion Presence of dry eye, candlewax retinal vasculitis, hilar lymphadenopathy, and fissural nodules if present in a patient with uveitis, could help differentiate ocular sarcoidosis from ocular tuberculosis in a high TB endemic population.

Sympathetic Ophthalmia - a Contribution to Immunology, Clinic and Current Imaging.

Sympathetic ophthalmia (SO) is a rare inflammation of an operated or injured eye that spreads to the fellow eye. It is typically a bilateral granulomatous panuveitis. The traumatized eye is referred to as inciting eye and the fellow eye as sympathizing eye. The pathophysiology of the disease is not entirely understood, but there is strong evidence of an autoimmune genesis. A selective literature search on epidemiology, immunology, clinical features and risk factors of SO was carried out. In addition, our own experience using multimodal imaging for this clinical entity was introduced. In the literature, the incidence after traumatic eye injuries is 0.1 - 3% and approximately 0.01% after intraocular surgery. Among the iatrogenic causes, vitreoretinal surgery has the highest rate of SO, presumably due to disruption of the blood-retinal barrier and involvement of retinal and choroidal tissue, which are susceptible to anterior traction, phthisis and chronic inflammation. In 90% of patients, the disease develops within a year following the eliciting event and is associated with a potentially bilateral risk of blindness. Typical symptoms include bilateral visual impairment with photophobia, dull pain and photopsia. The spectrum of clinical manifestations ranges from granulomatous anterior uveitis and vitritis, to choroiditis, serous retinal detachment and Dalen-Fuchs nodules in the context of posterior involvement. The diagnosis of SO is generally based on clinical presentation and is supported by imaging methods. These primarily comprise fluorescein and indocyanine green angiography, which are increasingly being supplemented by non-invasive methods such as optical coherence tomography. They can provide important information for assessment of severity, differential diagnosis as well as for disease monitoring. The differential diagnosis includes i. a. Vogt-Koyanagi-Harada syndrome, ocular sarcoidosis and the rare phacoanaphylactic endophthalmitis. Immediate systemic high-dose steroid therapy is used as initial treatment. The course of the disease is often relapsing to chronic progressive. Immunomodulators such as ciclosporine A, azathioprine, cyclophosphamide, mycophenolate mofetil, and biologics are increasingly being used and contribute to the significantly better prognosis of the disease. Generally, SO can be triggered by any kind of intraocular intervention. SO remains a threatening clinical diagnosis that poses diagnostic and therapeutic challenges. It can be triggered post-traumatic, but also any intraocular surgery. This should be taken into account when assessing the indication for intraocular eye surgery, especially in eyes with reduced visual outcome.

Ocular sarcoidosis: clinical experience and recent pathogenetic and therapeutic advancements

PurposeTo describe the ocular manifestations in a cohort of patients with systemic sarcoidosis (SS). Recent advances in the pathophysiology, diagnosis, and therapy of SS are also discussed.MethodsData from 115 Italian patients diagnosed between 2005 and 2016 were retrospectively reviewed. All but the first 17 patients underwent a comprehensive ophthalmologic examination. The diagnosis was based on clinical features, the demonstration of non-caseating granulomas in biopsies from involved organs, and multiple imaging techniques. Data on broncho-alveolar lavage fluid analysis, calcemia, calciuria, serum angiotensin-converting enzyme levels and soluble interleukin-2 receptor levels were retrieved when available.ResultsOcular involvement, detected in 33 patients (28.7%), was bilateral in 29 (87.9%) and the presenting feature in 13 (39.4%). Anterior uveitis was diagnosed in 12 patients (36.4%), Löfgren syndrome and uveoparotid fever in one patient each (3%), intermediate uveitis in 3 patients (9.1%), posterior uveitis in 7 (21.2%), and panuveitis in 9 (27.3%). First-line therapy consisted of corticosteroids, administered as eyedrops (10 patients), sub-Tenon’s injections (1 patient), intravitreal implants (9 patients), or systemically (23 patients). Second-line therapy consisted of steroid-sparing immunosuppressants, including methotrexate (10 patients) and azathioprine (10 patients). Based on pathogenetic indications that tumor necrosis factor (TNF)-α is a central mediator of granuloma formation, adalimumab, targeting TNF-α, was employed in 6 patients as a third-line agent for severe/refractory chronic sarcoidosis.ConclusionUveitis of protean type, onset, duration, and course remains the most frequent ocular manifestation of SS. Diagnostic and therapeutic advancements have remarkably improved the overall visual prognosis. An ophthalmologist should be a constant component in the multidisciplinary approach to the treatment of this often challenging but intriguing disease.

Recent advances in sarcoidosis genomics: epigenetics, gene expression, and gene by environment (G × E) interaction studies.

We aim to review the most recent findings in genomics of sarcoidosis and highlight the gaps in the field. Original explorations of sarcoidosis subphenotypes, including cases associated with the World Trade Center and ocular sarcoidosis, have identified novel risk loci. Innovative gene--environment interaction studies utilizing modern analytical techniques have discovered risk loci associated with smoking and insecticide exposure. The application of whole-exome sequencing has identified genetic variants associated with persistent sarcoidosis and rare functional variations. A single epigenomics study has provided background knowledge of DNA methylation mechanisms in comparison with gene expression data. The application of machine-learning techniques has suggested new drug repositioning for the treatment of sarcoidosis. Several gene expression studies have identified prominent inflammatory pathways enriched in the affected tissue. Certainly, sarcoidosis research has recently advanced in the exploration of disease subphenotypes, utilizing novel analytical techniques, and including measures of clinical variation. Nevertheless, large-scale and diverse cohorts investigated with advanced sequencing methods, such as whole-genome and single-cell RNA sequencing, epigenomics, and meta-analysis coupled with cutting-edge analytic approaches, when employed, will broaden and translate genomics findings into clinical applications, and ultimately open venues for personalized medicine.

A Validation Study of the Revised Diagnostic Criteria from the International Workshop on Ocular Sarcoidosis at a Single Institute in Japan

ABSTRACT Purpose: To validate the revised criteria of the International Workshop on Ocular Sarcoidosis (IWOS) for the diagnosis of ocular sarcoidosis (OS). Methods: A retrospective chart review study was performed on 323 patients including 51 patients with biopsy-proven sarcoidosis and 272 patients with other uveitis entities. Data on intraocular signs and systemic investigations were collected, and sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and the Youden index were calculated. Results: All intraocular signs and most systemic investigations showed high diagnostic parameters. Sensitivity, specificity, PPV, and NPV of the revised IWOS criteria were 1.000, 0.930, 0.728, and 1.000, respectively. Presumed or probable OS showed lower sensitivity and higher specificity when compared with the best Youden index. Conclusion: The revised IWOS criteria are useful in Japanese patients, but could possibly be improved by modifying the criterion of presumed or probable OS.

Activation of the interleukin-23/interleukin-17 signalling pathway in autoinflammatory and autoimmune uveitis.

Uveitis is a group of diseases characterized by intraocular inflammation, of which some are driven by autoinflammatory or autoimmune responses, such as Vogt-Koyanagi-Harada disease, Behçet's disease, uveitis associated with spondyloarthritis, ocular sarcoidosis, sympathetic ophthalmia and birdshot chorioretinopathy. These entities have various clinical forms, but genetic and biomarker data suggest that they share a common molecular basis, activation of the Interleukin (IL)-23/IL-17 pathway. Multiple factors including genetic predisposition, various cytokine imbalances, infectious agents and gut alterations are found to trigger an aberrant response of this pathway. The enhanced activity of the IL-23/IL-17 pathway is committed to the expansion and pathogenicity of Th17cells. Evidence from animal models demonstrates that the development of pathogenic Th17cells is responsible for the induction of experimental autoimmune uveitis. Further findings indicate that retinal pigment epithelium (RPE) cells may be a target of IL-17. IL-17 triggers downstream inflammatory cascades and causes dysfunction of RPE cells, which may affect retinal barrier function and thereby promote intraocular inflammation. Currently, several emerging drugs blocking the IL-23/IL-17 pathway have been assessed for the treatment of uveitis in pilot studies. The purpose of this is to summarize updated biological knowledge and preliminary clinical data, providing the rationale for further development and evaluation of novel drugs targeting the IL-23/IL-17 pathway in autoinflammatory and autoimmune uveitis. Future studies may focus on translational medicine targeting the IL-23/IL-17 pathway for the improvement of diagnosis and treatment of uveitis. In conclusion, activation of the IL-23/IL-17 pathway is a critical biological event and can be an important target for the treatment of autoinflammatory and autoimmune uveitis.

Genome-Wide Association Study of Ocular Sarcoidosis Confirms HLA Associations and Implicates Barrier Function and Autoimmunity in African Americans

ABSTRACT Purpose: Identify genes associated with ocular sarcoidosis (OS). Methods: We genotyped 1.1 million genetic variants to identify significant OS associations, defined as those that achieved p < 5 × 10−8 in a genome-wide comparison of OS cases to healthy controls in our European- or African-American cohorts (EA, AA). Potential functional roles of all associated variants were assessed. Results: Eight significant non-HLA variants were found in AA OS cases compared to healthy controls and confirmed as at least suggestive when comparing OS to non-OS cases. Seven of these were within MAGI1 and include transcription factor binding sites and expression quantitative trait loci. Our EA cohort, while showing similar effect sizes at variants within MAGI1, had no significant variants. Association analysis of HLA-DRB1 alleles confirmed association to OS in EA to *04:01. Conclusion: Our results support organ-specific genetic risk in OS in a compelling candidate, MAGI1, known to be associated with barrier function and autoimmunity.

Diagnosis of cardiac sarcoidosis despite negative findings on serial late gadolinium enhancement with cardiac magnetic resonance imaging/18F-fluorodeoxyglucose positron emission tomography examinations

We present a case of a 42-year-old Japanese man with ocular and pulmonary sarcoidosis who eventually led to a diagnosis with cardiac sarcoidosis (CS) through endomyocardial biopsy (EMB), despite negative findings on both late gadolinium enhancement with cardiac magnetic resonance (LGE-CMR) imaging and 18F-fluorodeoxyglucose positron emission tomography (FDG-PET). Cardiac sarcoidosis (CS) develops in only 5% of patients with systemic sarcoidosis. Previous studies have reported that CS was found in up to 50% of autopsy series with fatal sarcoidosis, implying that CS is frequently underdiagnosed with potentially life-threatening consequences. Therefore, the diagnostic accuracy and prognostic value of CS are important. Currently, LGE-CMR and FDG-PET play an important role in establishing a diagnosis of CS with high sensitivity. In the presented case, regardless of serial examinations with LGE-CMR and FDG-PET, confirmed diagnosis of CS could not be achieved; ultimately, a definitive diagnosis of CS was obtained through EMB. To the best of our knowledge, this is the first reported case showing the diagnosis of CS despite negative findings on serial LGE-CMR and FDG-PET examinations.

The Role of 18F-FDG PET/CT in Suspected Intraocular Sarcoidosis and Tuberculosis

ABSTRACT Purpose: To determine the utility of fluorine-18 fluorodeoxyglucose Positron Emission Tomography Computed Tomography (FDG PET/CT) in patients with suspected ocular sarcoidosis (OS) or intraocular tuberculosis (IOTB) in a resource-constrained, TB endemic area. Methods: Independent review of the FDG PET/CTs, Computed Tomography (CT) scans and chest radiographs (CXRs) of patients with suspected OS or IOTB and inconclusive conventional workup. Results: Twenty-nine PET/CTs and CXRs were reviewed, with 38% of PET/CTs and CTs demonstrating evidence of TB or sarcoidosis, compared to 21% of CXRs. The sensitivity, specificity, positive and negative predictive values for PET/CT and CT were similar - 85.7%, 95.5%, 85.7% and 95.5% for OS, and 33.3%, 100%, 100% and 68% for IOTB respectively and for CXR, 57.1%, 100%, 100% and 88% for OS, and 16.7%, 100%, 100% and 63% for IOTB. Conclusion: PET/CT added no significant additional benefit over Chest CT in patients with suspected OS or IOTB.

Lymphocyte Immunophenotyping and CD4/CD8 Ratio in Cerebrospinal Fluid for the Diagnosis of Sarcoidosis-related Uveitis

ABSTRACT Background: This study aimed to assess the diagnostic relevance of CD4/CD8 ratio in cerebrospinal fluid (CSF) for the etiological diagnosis work-up of uveitis. Methods: We consecutively included patients who were referred to our department for the diagnostic workup of intermediate and/or posterior uveitis. Etiological diagnoses were established in a blind manner regarding CD4/CD8 ratio. Results: Fifty-two patients were included. A diagnosis of ocular sarcoidosis was made in 15 (29%) patients, 21% had another determined diagnosis while 50% remained of undetermined origin. Median CD4/CD8 ratio in CSF was 4.57 (IQR 3.39–5.47) in ocular sarcoidosis, 1.74 (1.60–3.18) in uveitis due to other determined cause (P = .008), and 2.83 (2.34–3.54) in those with uveitis of undetermined origin (P = .007). CD4/CD8 ratio >3.23 was associated with a diagnosis of ocular sarcoidosis. Conclusion: Determination of CD4/CD8 ratio in CSF can be useful for diagnosis work-up since a CD4/CD8 ratio >3.23 in CSF is associated with ocular sarcoidosis.

P30 Extra-ocular manifestations of children with sarcoid-like uveitis

Abstract Background Paediatric sarcoidosis represents a spectrum of disease. Early onset sarcoidosis &amp; Blau syndrome associated with NOD2 mutations are characterized by fever, rash, arthritis &amp; organomegaly. Later onset sarcoidosis has wider organ involvement (lungs, kidneys, lachrymal &amp; extra-ocular glands). Both presentations may lead to long term complications due to end-stage organ damage. Ocular sarcoidosis has a well described uveitis phenotype. We aim to describe a retrospective cohort of children with sarcoid-like uveitis &amp; their systemic manifestations at time of study; compare cohort of patients fulfilling IWOS criteria for ocular sarcoidosis versus who did not; and describe their management in retrospective cohort. Methods We performed a retrospective case review of all children currently followed at GOSH with ocular sarcoidosis phenotype with uveitis (ophthalmologist definition based on IWOS, or diagnosis of idiopathic uveitis with raised ACE level at least once. We collected demographics &amp; all extra-ocular involvement described in sarcoidosis. Results n = 52; 27/52 males. Median age at onset of uveitis 4.20 years (1.41-15.16). 49/52 bilateral uveitis. 27/52 (50%) &lt;8years age at onset &amp; 2/6 NOD 2 + belonged to this group. Median ACE 68 U/L at presentation (0-90U/L). Median maximum ACE 74 (14-420). NOD2 tested in 12 patients: 6+ , 6- . 1/6 positive patient had Blau phenotype. Ethnicity: African 12/52, asian 11/52, caucasian 14/52, unknown 15/52. Uveitis: Anterior 17/52(32.6%), Anterior+Intermediate 1/52, intermediate 5/52(9.6%) , posterior 2/52, panuveitis 25/52(48%), undocumented 2/52. ANA positive (&gt;1:80) in 15/47 (32%). Systemic involvement (n = 52): arthritis 29%, liver 29%, lymphadenopathy 19%, renal 16%, lungs 15.3%, skin 17.3%, spleen 7.7%, glands 1.9%. Patients as per adult IWOS criteria: Definite 12/52, Presumed 6/52, Probable7/52 and Not fulfilling – 27/52. Systemic involvement in patients not fulfilling IWOS criteria (27/52) – renal 14.8%, arthritis 22.2%, hilar or peripheral lymphadenopathy 0 %, skin involvement 7.4%, lung 18.5%, splenomegaly 3.7%. Comparing IWOS fulfilling (25) with the ones who did not (27) – systemic involvement consistently less common in the ones NOT fulfilling but only reaches statistical significance difference for involvement. Lymphadenopathy and skin (p &lt; 0.001 and p &lt; 0.050 respectively). Suggesting that paediatric age group cannot be classified as per the adult IWOS ocular sarcoidosis criteria and needs early systemic screening. Medications used to treat uveitis and/or extra-ocular manifestations: methotrexate alone 25%, methotrexate + adalimumab 21.1%, mycophenolate mofetil 9.61%, only systemic steroids 3.8%. 9 patients- no systemic medications at any time during their disease. Conclusion Most sarcoid-like uveitis patients had at least one systemic involvement. 51.9% patients did not fulfil the IWOS ocular sarcoidosis criteria, still had systemic involvement. Hilar lymphadenopathy criteria cannot be applied to the paediatric population, peripheral more common. ACE not a sensitive biomarker to predict sarcoidosis. 17.3 % had mild phenotype &amp; required no treatment. This study demonstrates the importance of close monitoring for systemic manifestations &amp; highlights good clinical response to steroids, MTX, MMF and anti-TNF. Conflicts of Interest The authors declare no conflicts of interest.

The status in the application of optical coherence tomography angiography in noninfectious uveitis

Noninfectious uveitis refers to a category of inflammatory diseases involving the uvea, vitreous, optic disk and retina, with the exception of infectious factors or masquerade syndrome. These kind of blinding diseases are frequently recurrent, and the diagnosis and follow-up require fundus imaging techniques. OCT angiography (OCTA) is a rapid, noninvasive and quantifiable blood flow imaging modality that provides a depiction of the microvasculature morphology of the retinal and choroidal through different segmentation and detects the abnormal blood perfusion as well as the neovascularization. OCTA plays an important role in the diagnosis, assessment and follow-up for anterior uveitis, posterior uveitis and pan-uveitis such as Vogt-Koyanagi-Harada disease, Behcet’s disease, ocular sarcoidosis, birdshot chorioretinopathy, serpiginous choroiditis, multifocal choroiditis, punctate inner choroidopathy, acute zonal occult outer retinopathy, acute posterior multifocal placoid pigment epitheliopathy, multiple evanescent white dot syndrome, and also provides clue about their pathophysiologic mechanisms. Key words: Uveitis; Tomography, optical coherence; Review