Describing the Clinical and Laboratory Features and HLA-B Pattern of Adult-Onset Idiopathic Autoimmune Uveitis at a Tertiary Hospital in South India: A Cross-Sectional Study.

INTRODUCTION: There is a scarcity of information available on clinical and laboratory features of adult-onset idiopathic autoimmune uveitis. Therefore, we conducted a single centre descriptive cross-sectional study. Patients and Methods. A chart review of all patients with idiopathic autoimmune uveitis with onset after 18 years of age who were referred to the rheumatology department between January 2017 and December 2018 was performed. Their clinical features, demographic features, and HLA-B genotypes were documented and described. RESULTS: Out of 210 patients referred to rheumatology, 66 were found to have uveitis, and 16 of these had an adult-onset idiopathic autoimmune uveitis. Apart from a slight female preponderance (62.5%), our patients were characterized by a high proportion of panuveitis (4 out of 16, i.e., 25%). There was an increased frequency of occurrence of synechiae (5 out of 16, i.e., 31.3%), retinal vasculitis (4 out of 16, i.e., 25%), optic disc edema (3 out of 16, i.e., 18.8%), and cystoid macular edema (seen in 2 patients, i.e., 12.5%). These features correlated with the anatomical subtypes. Retinal vasculitis and optic disc edema present in three fourth of all panuveitis cases were the most prominent features. The odds of finding HLA-B∗35 in retinal vasculitis were 33 times higher than odds of finding it in idiopathic autoimmune uveitis patients not having retinal vasculitis (OR 33; 95% CI 1.6-698). CONCLUSION: Idiopathic autoimmune uveitis in our patients is characterized by a high frequency of panuveitis and retinal vasculitis, and complications with a probable association between HLA-B∗35 and retinal vasculitis.

Features of ectopic lymphoid-like structures in human uveitis.

Persistent non-infectious uveitis has a significant morbidity, but the extent to which this is accompanied by inflammation driven remodelling of the tissue is unclear. To address this question, we studied a series of samples selected from two ocular tissue repositories and identified 15 samples with focal infiltration. Eleven of fifteen contained lymphocytes, both B cells (CD20 positive) and T cells (CD3 positive). In 20% of the samples there was evidence of ectopic lymphoid like structures with focal aggregations of B cells and T cells, segregated into anatomically different adjacent zones. To investigate inflammation in the tissue, an analysis of 520 immune relevant transcripts was carried out and 24 genes were differentially upregulated, compared with control tissue. Two of these (CD14 and fibronectin) were increased in ocular inflammation compared to control immune tissue (tonsil). We demonstrate that in a significant minority of patients, chronic persistent uveitis leads to dysregulation of ocular immune surveillance, characterized by the development of areas of local ectopic lymphoid like structures, which may be a target for therapeutic intervention directed at antibody producing cells.

Observation and Clinical Pattern in Patients with White Dot Syndromes: The Role of Color Photography in Monitoring Ocular Changes in Long-Term Observation.

BACKGROUND The aim of this study was to assess the clinical course and distinctive features of different white dot syndromes (WDS) in patients attending the Ophthalmology Department, Medical University of Warsaw in the years 1995-2015. MATERIAL AND METHODS Sixty-two (62) patients (43 females and 19 males), aged 18 to 77 years, referred with a WDS were included in this prospective study, with observation period ranging from 5 months to 16 years. All patients underwent a complete ophthalmological examination and multimodal imaging studies. RESULTS In this cohort of 62 patients, the following WDS entities were identified: multifocal choroiditis with panuveitis (MFCPU), multifocal choroiditis (MFC), punctate inner choroidopathy (PIC), birdshot, acute posterior multifocal placoid pigment epitheliopathy (APMPPE), subretinal fibrosis and uveitis, multiple evanescent white dot syndrome (MEWDS), serpiginous choroiditis, and single cases of acute annular outer retinopathy (AAOR). CONCLUSIONS The study was performed at a Polish referral center and may to some extent reflect the varied geographical distribution of white dot syndromes, as none of the subjects was found to suffer from acute zonal occult outer retinopathy (AZOOR), acute macular neuroretinopathy (AMN), or diffuse unilateral subacute neuroretinitis (DUSN). Long-term follow-up is warranted by the evolution of lesions in the eye fundus, while management depends on correct diagnosis of WDS. When the posterior pole is involved in some cases of the WDS an immunosuppressive treatment, the use of the PDT or anti-VEGF injections were necessary.

Positive immunostaining for feline infectious peritonitis (FIP) in a Sphinx cat with cutaneous lesions and bilateral panuveitis.

Feline infectious peritonitis (FIP) is a common, fatal, systemic disease of cats. This case report describes the antemortem diagnosis of FIP in a 2-year-old spayed female Sphinx cat that presented with a bilateral panuveitis and multiple papular cutaneous lesions. Histopathologically, the skin lesions were characterized by perivascular infiltrates of macrophages, neutrophils, with fewer plasma cells, mast cells, and small lymphocytes in the mid- to deep dermis. Immunohistochemistry for intracellular feline coronavirus (FeCoV) antigen demonstrated positive staining in dermal macrophages providing an antemortem diagnosis of a moderate, nodular to diffuse, pyogranulomatous perivascular dermatitis due to FIP infection. Obtaining an antemortem diagnosis of FIP can be a challenge and cutaneous lesions are rare in the disease. Recognition and biopsy of any cutaneous lesions in cats with panuveitis and suspected FIP can help establish an antemortem diagnosis of the disease.

Bilateral Granulomatous Panuveitis in A Patient with COVID-19.

PURPOSE: To present a case of panuveitis with granulomatous intraocular inflammation and vasculitis related to coronavirus disease 2019 (COVID-19). CASE REPORT: A 46-year-old female patient had blurred vision in both eyes during 8 days after COVID-19 symptoms. Anterior segment examination revealed anterior chamber cells in the both eyes, mutton fat keratic precipitates and posterior synechiae in the left eye. Small peripheral iris nodules and mild vitritis were accompanying in both eyes. Fundus examination revealed multiple superficial retinal infiltrate lesions in the peripheral retina with retinal periphlebitis in both eyes. Spectral domain optical coherence tomography showed hyperreflective superficial retinal infiltrates. Fluorescein angiography (FA) and indocyanine green angiography demonstrated hypofluorescent foci due to blockade of retinal infiltrates. Peripheral vascular leakage was detected on FA in both eyes. CONCLUSION: This case showed that COVID-19 infection can cause granulomatous panuveitis. Autoimmune mechanisms related to COVID-19 may lead to ocular inflammation.

[Role of Chlamydia trachomatis and Chlamydophila pneumoniae in damage of eye posterior segment structures].

AIM: Study the ability of Chlamydia trachomatis and Chlamydophila pneumoniae to damage structures of eye posterior segment, features of development of such infectious process, its morphological and clinical characteristics. MATERIALS AND METHODS: 6 rabbits with confirmed absence of C. trachomatis, C. pneumoniae were used in the study. 3 animals were infected with C. trachomatis culture and 3 animals--with C. pneumoniae culture. Subconjunctival and intravitreal mode of infectious agent introduction were used, as well as instillation of its culture into conjunctival sac. Microbiological diagnostics included microscopy with direct immunofluorescence, culture method and determination of antibody titers. Infectious process was studied by using ophthalmologic methods and histological examination. Observation period was 4 months. RESULTS: In all the animals a development of infectious process at early stages after the infection was confirmed. Conjunctivitis symptoms, inflammatory exudation into vitreous humor, chorioretinal inflammation loci, disorders in transparency of optical media and detachment of retina were clinical manifestations. In 2 animals infected with C. trachomatis severe panuveitis was noted. In 4 animals infectious process assumed subclinical characteristics (infection with both C. trachomatis or C. pneumoniae). In pathomorphologic studies data on the ability of C. trachomatis and C. pneumoniae to cause damage to cells of retina, pigment epithelium and choroid were obtained. CONCLUSION: C. trachomatis and C. pneumoniae may play a significant role in pathology of vitreous humor, retina, pigment epithelium and choroid.

Magnetic resonance imaging of pachymeningeal enhancement in Vogt-Koyanagi-Harada disease.

Vogt-Koyanagi-Harada (VKH) disease is a systemic disease consisting of bilateral granulomatous panuveitis combined with cutaneous and neurologic manifestations. However, there have been few reports of brain magnetic resonance imaging (MRI) in VKH disease. A 54-year-old Korean woman presented with severe periorbital pain, blurred vision and meningismus. Ophthalmologic examination disclosed bilateral optic disc edema with peripapillary nerve fiber hemorrhages. Lumbar puncture revealed monocytic pleocytosis. After a diagnosis of VKH disease was made, the patient was treated with high-dose corticosteroid. Brain MRI showed diffusely thickened posterior ocular walls with retinal detachment and perineural infiltrative changes along the optic nerves and adjacent pachymeningeal enhancement of the anterior temporal lobes bilaterally. We report a case of VKH disease with panuveitis and meningeal involvement of the anterior temporal lobe detected by brain MRI.

Ichthyosiform sarcoidosis: A case report and review of the literature.

Sarcoidosis is a potentially life-threatening, multisystem, granulomatous disease that can present with cutaneous manifestations in patients. A rare cutaneous manifestation of this disease may resemble acquired ichthyosis. We report a 45-year-old woman with a several year history of dyspnea on exertion and panuveitis who presented to a county hospital with acquired lower extremity ichthyosis and a biopsy consistent with both acquired ichthyosis and noncaseating, granulomatous sarcoidosis. To our knowledge, this entity has been described in only 22 previous independent cases, with the present case being 1 of only 5 cases to rapidly progress to full systemic involvement. Furthermore, it is important to recognize the manifestations of sarcoidosis in the skin, because these may be the presenting signs of systemic illness.

Epidemic of unilateral panuveitis in children from Brazilian Amazonia: clinical and etiological aspects in seven patients.

OBJECTIVES: To describe clinical presentation and results of diagnostic and therapeutic procedures in seven children from an epidemic of panuveitis in the Brazilian Amazonia, as well as environmental analysis and etiological aspects involved. METHODS: Patients underwent full pediatric and ophthalmic examinations, B-scan, ultrasound biomicroscopy, and serological tests. Ocular samples were thoroughly analyzed, including two enucleation specimens. Environmental investigation encompassed water, soil, and river fauna. RESULTS: All patients had bathed in the waters of a regional river, the Araguaia. Six of them presented with intermediate uveitis, with snowbanking. Five had cataract and four showed inferior endothelial opacity, with localized anterior synechiae. One showed total leukoma, with flat anterior chamber. Only two had active uveitis, one of them with anterior chamber nodule. Serology revealed high prevalence of anti-Toxocara canis immunoglobulin G (IgG) antibodies. In three cases, vitreous and lens samples disclosed spicules of freshwater sponges Drulia uruguayensis and D. ctenosclera, also detected in the waters of the river. CONCLUSION: Freshwater sponge spicules could be potential new etiological agents of ocular pathology, but further studies are needed, considering the heterogeneity of the ocular lesions and results of serological and environmental studies.

CLINICAL PATHOLOGIC CORRELATION OF SARCOIDOSIS-ASSOCIATED MULTIFOCAL CHORIORETINITIS IN AN ELDERLY PATIENT.

BACKGROUND/PURPOSE: To describe a case of histopathologic clinical correlation of sarcoidosis-related multifocal chorioretinitis in an elderly individual. METHODS: This is a single case report and review of the pertinent literature. A 72-year-old white woman with a history of asthma was referred with bilateral asymmetric low-grade chronic panuveitis with advancing peripheral punched-out chorioretinal lesions despite systemic corticosteroid treatment. RESULTS: Coalescent chorioretinal punched-out lesions on fundus examination in the more severely affected right eye did not respond to corticosteroid treatment, and the refractory nature resulted in a suspicion of intraocular lymphoma despite a previous inconclusive diagnostic vitrectomy result. A transvitreal chorioretinal biopsy in the vitrectomized eye demonstrated a chorioretinal noncaseating granuloma centered on the choriocapillaris and invading Bruch membrane and the outer retina. This case illustrates an example of the second peak of incidence in sarcoidosis that can occur in ages >50 in Caucasians and Japanese patients, as well as a tendency to develop chronic disease in Caucasians, and to have extrapulmonary sarcoidosis in the elderly. CONCLUSION: This case emphasizes the differences in presentation of ocular sarcoid in older Caucasian women as well as the histopathologic findings in multifocal peripheral chorioretinitis associated with sarcoidosis.

Seasonal Hyperacute Panuveitis in Nepal: A Review over 40 Years of Surveillance.

Purpose: This review aims to provide a detailed update in Seasonal hypercute panuveitis (SHAPU) which is a blinding disease of unknown etiology reported only from Nepal, occurring every odd year since 1975 predominantly in children. Methods: Data sources were literature reviewed using PubMed, Medline, and ISI Databases (since 1975 to late 2017). Search items included SHAPU, seasonal endophthalmitis, hypopyon uveitis, caterpillar induced uveitis alone or in combination with white moth, panuveitis, and review. Results: Epidemics of SHAPU occur between August to December usually in odd years. Two thirds of the children present with unilateral blind eye which eventually becomes soft with shallowing of anterior chamber called as Malignant Hypotension. Hypopyon, fibrinous exudates in anterior chamber, difficult to dilate pupil and inability to visualize the retina because of massive exudation into vitreous producing a "White pupil in a Red eye" with little or no pain is its characteristic features. Contact with moths has been suspected. Because of presence of microbial agents in few cases, an infectious etiology has not been ruled out. Conclusions: Although SHAPU has been reported only from Nepal, lack of its report from ecologically similar terrain along Hindukush Mountains and possible association with moths remain intriguing.

Supposed endogenous endophthalmitis caused by Serratia marcescens in a cat.

An 8-year-old male neutered domestic shorthair cat was presented for evaluation of acute respiratory distress. Respiratory auscultation revealed a diffuse and symmetric increase in bronchovesicular sounds. Thoracic radiographs showed a diffuse unstructured interstitial pulmonary pattern with multifocal alveolar foci. Despite an aggressive treatment with supportive care, including oxygenotherapy and systemic antibiotics, progressive respiratory distress increased. Three days after the presentation, acute anterior uveitis was noticed on left eye. Ophthalmic examination and ocular ultrasonography revealed unilateral panuveitis with ocular hypertension. The right eye examination was unremarkable. Cytological examination of aqueous humor revealed a suppurative inflammation. Serratia marcescens was identified from aqueous humor culture. Primary pulmonary infection was suspected but was not confirmed as owners declined bronchoalveolar lavage. Active uveitis resolved and cat's pulmonary status improved after appropriate systemic antibacterial therapy. Vision loss was permanent due to secondary mature cataract. To the best of authors' knowledge, this is the first report of endogenous bacterial endophthalmitis secondary to S. marcescens infection in a cat.

Ocular manifestations associated with brucellosis: a 26-year experience in Peru.

BACKGROUND: Brucellosis has unusual clinical manifestations. Ocular involvement caused by brucellosis remains poorly recognized in areas in which brucellosis is endemic. METHODS: A prospective study was performed to evaluate patients attending the Instituto de Medicina Tropical "Alexander von Humboldt" and the Hospital Nacional Cayetano Heredia (Lima, Peru) from January 1980 through December 2005 who received a diagnosis of brucellosis with ocular involvement. Diagnosis was made on the basis of clinical findings as well as agglutinations and/or culture positive for Brucella melitensis. RESULTS: During a period of 26 years, 1551 patients with brucellosis were seen, including 52 patients with ocular brucellosis. We found that 7 (0.7%) of 965 patients with acute brucellosis and 45 (7.9%) of 570 patients with chronic brucellosis had ocular brucellosis (P<.001). In 16 patients with brucellosis, the disease stage was unclassified. The most frequent ocular presentation was uveitis, which was found in 43 (82.7%) of the 52 patients with ocular brucellosis. Posterior uveitis was the most frequent uveal syndrome (21 cases; 45.7%). Patients with panuveitis had the worst visual prognosis: 8 of 9 patients with panuveitis were legally blind, including 5 patients with no light perception. CONCLUSIONS: Brucellosis may involve the eye and can lead to serious complications. In patients with brucellosis, early ophthalmologic evaluation can lead to prompt treatment and might prevent blindness from severe ocular damage.

Pathological findings of multifocal choroiditis with panuveitis and punctate inner choroidopathy.

PURPOSE: To compare the pathological findings between multifocal choroiditis with panuveitis (MCP) and punctate inner choroidopathy (PIC). METHODS: Fourteen eyes of 14 patients clinically diagnosed as having MCP or PIC who underwent surgical excision of choroidal neovascularization (CNV) were studied. Immunohistochemical findings of the excised CNV in MCP (eight eyes) and PIC (six eyes) cases were compared. Antibodies against vascular endothelial growth factor (VEGF), CD68, CD3, and CD20 were used as primary antibodies. RESULTS: Expression of VEGF and CD68 in the CNV was observed in all MCF and PIC cases. In three of eight eyes with MCP, intraocular inflammatory findings were found clinically, while immunohistochemical study demonstrated infiltration of CD20-positive B lymphocytes in the CNV. No B lymphocyte infiltration was found in the six eyes with PIC. No differences in pathological findings were found between the five MCP eyes without intraocular inflammation and the six PIC eyes, with all eyes showing no B lymphocyte infiltration. CONCLUSIONS: In MPC cases showing clinical inflammatory findings, infiltration of B lymphocytes was also observed histopathologically, suggesting that the presence of inflammatory cells in the anterior chamber or vitreous body clinically is an indicator of active inflammatory CNV. However, this study clarifies that MCP eyes without intraocular inflammation and PIC eyes are not different in histopathological findings.

Sarcoid uveitis in a patient with multiple neurological lesions: a case report and review of the literature.

BACKGROUND: Neurosarcoidosis is a rare complication, and cranial neuropathy is the most frequent manifestation of this disease. However, few cohesive reports have discussed multiple cranial neuropathies in Japanese patients with sarcoidosis. The present report discusses the case of a patient with sarcoid uveitis and multiple neurological findings. We further review relevant literature regarding Japanese patients with multiple cranial nerve palsies published within the past 34 years (from January 1982 to December 2016). CASE PRESENTATION: We report findings associated with the case of a 56-year-old Japanese woman with granulomatous pan-uveitis who was later diagnosed as having sarcoidosis by skin and transbronchial lung biopsies. She presented right-sided Bell's palsy and was treated with orally administered prednisolone. However, while prednisolone was tapered, she developed facial (VII) and vagus (X) nerve palsies, followed by brain parenchyma lesions, which were not associated with any additional neurological symptoms. Furthermore, she exhibited increased intraocular pressure in her right eye, and she underwent trabeculectomy. Our review of the literature revealed that 64 Japanese patients with sarcoidosis experienced multiple cranial nerve palsies between 1982 and 2016. The most commonly affected cranial nerves were the facial (VII) (73.4%) and glossopharyngeal/vagus (IX/X) nerves (48.4%). Palsies of two distinct cranial nerves were found in 40.6% of the patients, followed by palsies of three (23.4%) and four (18.8%) nerves. Almost all patients (98.3%) received systemic steroid therapy, and total or partial remission was achieved in almost all patients (96.5%). CONCLUSIONS: According to the literature, patients with multiple cranial nerve palsies associated with sarcoidosis respond well to orally administered steroid therapy. However, our findings suggest that careful follow-up is necessary for patients with neurosarcoidosis due to potential aggravation of neuropathy.

[A case of panuveitis with optic disc neovascularization associated with juvenile idiopathic arthritis which progressed during a clinical trial of etanercept].

PURPOSE: To report a case of uveitis associated with juvenile idiopathic arthritis that progressed from iritis to panuveitis with disc neovascularization during a clinical trial of etanercept, a tumor necrosis factor a (TNF-alpha) blocker. CASE: A 12-year-old girl with juvenile idiopathic arthritis, which had begun at the age of 1 year 7 months. The patient was enrolled in a clinical trial of etanercept at 11 years of age. The methotrexate which she has been taking was stopped, and prednisolone was decreased gradually from 7 mg. The iritis worsened and progressed to panuveitis with disc neovascularization when the prednisolone dose had been tapered to 2 mg. The uveitis was controlled by treatment with a steroid pulse and a liposteroid. CONCLUSION: When starting etanercept therapy in a patient with juvenile idiopathic arthritis who has uveitis and the antirheumatic drug is stopped and steroid treatment tapered, special care is needed to avoid the exacerbation of uveitis.

Noninvasive Ocular Drug Delivery System of Dexamethasone Sodium Phosphate in the Treatment of Experimental Uveitis Rabbit.

PURPOSE: To investigate the efficacy and safety of dexamethasone sodium phosphate administered through Visulex system (DSP-Visulex) in treating experimental uveitis. METHODS: Uveitis was induced in rabbits by subcutaneous injections of complete Freund's adjuvant and an intravitreal injection of H37RA antigen. After induction, the animals of the control group received no treatment and the others received various treatment regimens of DSP-Visulex. Each regimen was different in DSP strength (4%, 8%, and 15%), application time, or treatment frequency. Efficacy and safety of DSP-Visulex were evaluated by ophthalmic observations and histopathological examinations for ocular inflammations and pathology. RESULTS: The control group exhibited panuveitis with significant inflammation in the vitreous, choroid, and retina, but less in the conjunctiva, cornea, and anterior chamber. The uveitis occurred within 24 h after induction and persisted throughout the study in the control group. All treatments showed some reduction in inflammation in the vitreous, choroid, and retina. The higher dose regimens generally showed more rapid and higher degree of resolution than the lower dose regimens. The posterior eye tissues of the 15% and 8% DSP-Visulex appeared normal with minimal or no inflammation, whereas the untreated eye and the 4% DSP-Visulex eyes showed minimal response. CONCLUSIONS: All DSP-Visulex regimens suppressed the signs of inflammation and were well tolerated over the course of a 29-day study. The 8% and 15% DSP-Visulex treatment regimens were safe and efficacious for anterior, intermediate, and posterior uveitis. On the other hand, the 4% DSP-Visulex regimen may only be considered for anterior and intermediate uveitis.

Choroidal neovascularisation on optical coherence tomography angiography in punctate inner choroidopathy and multifocal choroiditis.

PURPOSE: To describe the findings seen on optical coherence tomography angiography (OCTA) in patients with punctate inner choroidopathy (PIC) and multifocal choroiditis and panuveitis (MCP) complicated by choroidal neovascular membranes. METHODS: This was an Institutional Review Board-approved prospective, descriptive case series. 12 patients with PIC and MCP complicated by choroidal neovascularisation (CNV) were included. Each patient underwent slit-lamp examination by a uveitis specialist followed by conventional spectral domain OCT imaging of the macula. OCTA images of the macula were then obtained. RESULTS: 12 patients were enrolled in the study, out of which 9 patients were followed longitudinally. CNV was identified in 11 of the 12 patients. In all patients where fluorescein angiography (FA) was inconclusive for presence of CNV, OCTA identified CNV. Various lesions on OCT suggestive of activity correlated with changes in the vascular structure of OCTA to confirm suspicion of clinical activity. CONCLUSION: In patients with PIC and MCP complicated by CNV, OCTA successfully identified underlying CNV. Given the difficulty of differentiating inflammatory lesions from early CNV on OCT and FA, OCTA may provide a valuable method of monitoring patients with posterior uveitis highly correlated with development of CNV.