Fundus Autofluorescence in Posterior and Panuveitis-An Under-Estimated Imaging Technique: A Review and Case Series.

Fundus autofluorescence (FAF) is a prompt and non-invasive imaging modality helpful in detecting pathological abnormalities within the retina and the choroid. This narrative review and case series provides an overview on the current application of FAF in posterior and panuveitis. The literature was reviewed for articles on lesion characteristics on FAF of specific posterior and panuveitis entities as well as benefits and limitations of FAF for diagnosing and monitoring disease. FAF characteristics are described for non-infectious and infectious uveitis forms as well as masquerade syndromes. Dependent on the uveitis entity, FAF is of diagnostic value in detecting disease and following the clinical course. Currently available FAF modalities which differ in excitation wavelengths can provide different pathological insights depending on disease entity and activity. Further studies on the comparison of FAF modalities and their individual value for uveitis diagnosis and monitoring are warranted.

Extranodal Rosai-Dorfman disease manifesting as Sjögren's syndrome combined with panuveitis and hypertrophic pachymeningitis: a case report and review of literature.

Rosai-Dorfman disease (RDD) is a rare non-Langerhans cell histiocytosis characterized by massive lymphadenopathy and systemic extranodal lesions. We present the case of a 28-year-old woman who presented with recurrent blurred vision in her right eye for 3 months. She developed blindness and atrophy in her left eye a decade prior to presentation. She subsequently developed headache, fever, and impaired mental status. Cranial magnetic resonance imaging indicated hypertrophic pachymeningitis (HP), and 18F-fluoro-2-deoxy-2-d-glucose (FDG) positron emission tomography/computed tomography revealed significant FDG uptake in the left dura mater. Autoimmune testing revealed elevated anti-nuclear, anti-SS-A, and anti-SS-B antibody levels. Incisional biopsy of the atrophic eyeball revealed RDD with marked polyclonal plasmacytosis. The patient was diagnosed with RDD accompanied by multisystem involvement, including Sjögren's syndrome (SS), panuveitis, and HP. Treatment with methylprednisolone for several weeks resulted in significant improvement. This is the first reported case of RDD presenting with SS in combination with panuveitis and HP. Although RDD is rarely diagnosed in young patients, interdisciplinary collaboration is essential to prevent a delayed diagnosis.

OUTCOMES OF INFECTIOUS PANUVEITIS ASSOCIATED WITH SIMULTANEOUS MULTI-POSITIVE OCULAR FLUID POLYMERASE CHAIN REACTION.

PURPOSE: To evaluate features of infectious panuveitis associated with multiple pathogens detected by ocular fluid sampling. METHODS: Single-center, retrospective, consecutive case series of patients with aqueous/vitreous polymerase chain reaction testing with >1 positive result in a single sample from 2001 to 2021. RESULTS: Of 1,588 polymerase chain reaction samples, 28 (1.76%) were positive for two pathogens. Most common pathogens were cytomegalovirus (n = 16, 57.1%) and Epstein-Barr virus (n = 13, 46.4%), followed by varicella zoster virus (n = 8, 28.6%), Toxoplasma gondii (n = 6, 21.4%), herpes simplex virus 2 (n = 6, 21.4%), herpes simplex virus 1 (n = 6, 21.4%), and Toxocara (n = 1, 3.6%). Mean initial and final visual acuity (logarithm of the minimum angle of resolution) were 1.3 ± 0.9 (Snellen ∼20/400) and 1.3 ± 1.1 (Snellen ∼20/400), respectively. Cytomegalovirus-positive eyes (n = 16, 61.5%) had a mean final visual acuity of 0.94 ± 1.1 (Snellen ∼20/175), whereas cytomegalovirus-negative eyes (n = 10, 38%) had a final visual acuity of 1.82 ± 1.0 (Snellen ∼20/1,320) ( P < 0.05). Main clinical features included intraocular inflammation (100%), retinal whitening (84.6%), immunosuppression (65.4%), retinal hemorrhage (38.5%), and retinal detachment (34.6%). CONCLUSION: Cytomegalovirus or Epstein-Barr virus were common unique pathogens identified in multi-PCR-positive samples. Most patients with co-infection were immunosuppressed with a high rate of retinal detachment and poor final visual acuity. Cytomegalovirus-positive eyes had better visual outcomes compared with cytomegalovirus-negative eyes.

Seasonal Hyperacute Panuveitis (SHAPU) in Bhutan.

PURPOSE: To report the first series of Seasonal Hyperacute Pan Uveitis (SHAPU) from Bhutan. METHODS: We retrospectively analyzed the patients with clinically diagnosed SHAPU treated in the referral center in the last 5 years. Data included demographics (age, sex, laterality), region, time of presentation (year, season) and treatment received. RESULTS: The series included 3 males and 2 females. The mean age was 16 years. Three patients had presented in autumn of 2021, and two in autumn of 2019. Four patients presented within 2 weeks of the onset of symptoms. All patients had either exposure or contact with white moths. All patients presented with unilateral sudden painless reduction in vision and low Intraocular pressure. Four patients required vitrectomy. The final visual acuity of 3 patients was >6/36, and one patient was 6/60. CONCLUSIONS AND IMPORTANCE: The time of presentation and early treatment intervention are crucial in achieving good visual prognosis in SHAPU.

Clinical Features and Course in Patients Diagnosed with Ocular Sarcoidosis without Previously Known Systemic Disease at a Tertiary Referral Center in Turkey.

PURPOSE: To evaluate the ocular features, course, and prognosis of uveitis in the ocular sarcoidosis (OS) subgroups without previously known systemic sarcoidosis. METHODS: Ninety-one eyes of 49 patients were included. Definite OS patients were classified as group 1 (n = 15), presumed OS patients as group 2 (n = 15), and probable OS patients as group 3 (n = 19). RESULTS: The most common presentation of OS was panuveitis in all groups. During the follow-up, bilateral ocular involvement was observed in 85.7% of the patients. The most common extraocular involvement was pulmonary involvement, which was detected in 61.2% of the patients at the time of diagnosis. CONCLUSION: Ophthalmologists should be aware that uveitis may be the first sign of systemic sarcoidosis. In all subgroups of OS, uveitis tends to be bilateral and the most common type is panuveitis. Therefore, the eye without obvious clinical findings should be carefully evaluated clinically and angiographically.

A case of panuveitis caused by caterpillar setae.

INTRODUCTION: Panuveitis is a serious inflammatory disease. Setae are fibers produced by many insects and plants. Many case reports have described caterpillar and spider fibers entering the eye. These hairs are covered with tiny barbs that help them enter and migrate into the eye tissue, leading to severe inflammation. Normally, they are buried mainly in the conjunctiva and cornea. However, in the present case, they entered the posterior segment of the eye, which is very rare. CASE DESCRIPTION: A female patient presented with a complaint of repeated foreign body sensation, redness, pain, and photophobia in the left eye for 9 years following initial exposure to caterpillars. She visited the doctor in January after aggravation of symptoms. Slit-lamp examination, ultrasound biomicroscopy, ultrasound B-scan, fluorescein angiography, and indocyanine green angiography revealed the presence of a foreign body in the vitreous, which caused discomfort in the patient's left eye and subsequently, panuveitis of the left eye. The symptoms improved significantly after foreign body removal using vitrectomy. Microscopic examination revealed that the foreign body was caterpillar setae. CONCLUSION: Clinicians should be more vigilant about history of contact with caterpillars while examining uveitis that has recurred for many years. Identification of the root cause of the disease can ensure better treatment.

Ocular syphilis on the rise: a 10-year analysis from 2010 to 2020.

PURPOSE: The purpose of the study was to evaluate the incidence of ocular syphilis as well as diagnostic parameters, comorbidities, and visual outcomes over a 10-year time period in West Virginia. METHODS: A retrospective chart review included 25 eyes of 17 patients with ocular syphilis between 2010 and 2020. RESULTS: The incidence of systemic syphilis at a large tertiary referral center has increased from 27 cases in 2010 to 105 cases in 2020. Seventeen patients were identified with ocular syphilis. Bilaterality was present in 47.1% of cases. In this study, 70.6% of patients were male and 29.4% were female. The median age of presentation was 40.2 years (range 21-63). Panuveitis was the most common (60.0%) followed by isolated anterior uveitis (16.0%), chorioretinitis (12.0%), inner retinitis (4.0%), and papillitis (8.0%). Forty percent of patients had visual acuity worse than 20/400 on presentation. Post-treatment visual acuity improved in all patients. Rapid plasma reagin (RPR) and Treponema pallidum particle agglutination (TP-PA) tests were positive in 84.6% and 100% of cases, respectively. CSF venereal disease research laboratory (VDRL) was positive in 36.4%, CSF pleocytosis was present in 72.7%, and elevated CSF protein was observed in 81.8%. Human immunodeficiency virus (HIV) co-infection was present in 31.3%. A majority of patients experienced maculopapular rash and/or history of genital chancre. The anatomic classification of presenting uveitis (anterior, intermediate, posterior, and panuveitis) did not correlate with clinical variables including age, gender, HIV status, serologic test, presence of rash, or year of diagnosis (p > 0.05). CONCLUSION: Ocular syphilis is becoming increasingly prevalent and can present with a variety of ocular findings; therefore, it should be considered in the differential diagnosis for patients with ocular inflammation. Visual prognosis is excellent with timely diagnosis and treatment.

Diagnostic Challenges in PD-1 Inhibitor Induced Panuveitis in a Teenage Girl with Chest Soft Tissue Sarcoma - A Case Report.

PURPOSE: To report a case of programmed cell death receptor-1 (PD-1) inhibitor induced panuveitis. METHOD: Observational case report of a 13-year-old Chinese girl presented as panuveitis. The clinical course, imaging performance, laboratory examination, differential diagnosis, treatment and prognosis were described. RESULT: Patient presented with bilateral anterior granulomatous uveitis, vitritis, papillitis, and various creamy yellow nodular lesions in the mid-peripheral fundus. She had a history of biopsy proven alveolar soft tissue sarcoma on the chest wall and pulmonary metastasis, and a PD-1 inhibitor (sintilimab) was intravenously administered. Blood tests, magnetic resonance imaging of the cranium and the orbit, aqueous humor assay of inflammatory cytokines and microbial DNA were performed to distinguish infectious and non-infectious uveitis, choroidal metastases, and intravenous injection-related endophthalmitis. The oncologist evaluated that the sarcoma was stable and terminated sintilimab dosage. After sintilimab withdrawal, the blurred vision improved. Then, the patient received oral corticosteroids, resulted in resolution of the panuveitis. A diagnosis of PD-1 inhibitor induced panuveitis was made. CONCLUSION: For patients taking PD-1 inhibitors, the major diagnostic challenge is to identify whether the cause of the uveitis is due to the antitumor treatment or not. It is suggested to be screened by eye care specialist and timely referral to uveitis specialist with any suspicion of intraocular inflammation for these patients.

A COVID-19 perspective of Vogt-Koyanagi-Harada disease.

Vogt-Koyanagi-Harada (VKH) disease, a bilateral granulomatous panuveitis associated with multisystem involvement, is a T-cell-mediated autoimmune disorder in which cytotoxic T-cell target melanocytes in genetically susceptible individuals. Recently, there has been an increase in literature on the new onset of uveitis and reactivation of previously diagnosed cases of uveitis following Covid-19 vaccinations. It has been postulated that Covid-19 vaccines can lead to an immunomodulatory change resulting in an autoimmune phenomenon in the recipients. VKH following COVID-19 infection was reported in four patients and a total of 46 patients developing VKH or VKH-like disease following COVID-19 vaccinations. There are reports of four patients who had been recovering or recovered from VKH after receiving the first dosage of the vaccine and developed worsening of ocular inflammation after receiving the second dose of the vaccine.

A case of APMPPE-like panuveitis presenting with extensive outer retinal layer impairment following COVID-19 vaccination.

BACKGROUND: Vaccination against the worldwide pandemic coronavirus disease 2019 (COVID-19) is underway; however, some cases of new onset uveitis after vaccination have been reported. We report a case of bilateral acute posterior multifocal placoid pigment epitheliopathy-like (AMPPE-like) panuveitis after COVID-19 vaccination in which the patient's pathological condition was evaluated using multimodal imaging. CASE PRESENTATION: A 31-year-old woman experienced bilateral hyperemia and blurred vision starting 6 days after her second inoculation of the COVID-19 vaccination. At her first visit, her visual acuity was decreased bilaterally, and severe bilateral anterior chamber inflammation and bilateral scattering of cream-white placoid lesions on the fundus were detected. Optical coherence tomography (OCT) showed serous retinal detachment (SRD) and choroidal thickening in both eyes (OU). Fluorescein angiography (FA) revealed hypofluorescence in the early phase and hyperfluorescence in the late phase corresponding to the placoid legions. Indocyanine green angiography (ICGA) showed sharply marginated hypofluorescent dots of various sizes throughout the mid-venous and late phases OU. The patient was diagnosed with APMPPE and was observed without any medications. Three days later, her SRD disappeared spontaneously. However, her anterior chamber inflammation continued, and oral prednisolone (PSL) was given to her. Seven days after the patient's first visit, the hyperfluorescent lesions on FA and hypofluorescent dots on ICGA partially improved; however, the patient's best corrected visual acuity (BCVA) recovered only to 0.7 OD and 0.6 OS, and the impairment of the outer retinal layer was broadly detected as hyperautofluorescent lesions on fundus autofluorescence (FAF) examination and as irregularity in or disappearance of the ellipsoid and interdigitation zones on OCT, which were quite atypical for the findings of APMPPE. Steroid pulse therapy was performed. Five days later, the hyperfluorescence on FAF had disappeared, and the outer retinal layer improved on OCT. Moreover, the patient's BCVA recovered to 1.0 OU. Twelve months after the end of treatment, the patient did not show any recurrences. CONCLUSIONS: We observed a case of APMPPE-like panuveitis after COVID-19 vaccination featuring some atypical findings for APMPPE. COVID-19 vaccination may induce not only known uveitis but also atypical uveitis, and appropriate treatment is required for each case.

Panuveitis of undetermined origin after diagnostic pars plana vitrectomy: clinical characterization and long-term outcome from a tertiary referral center.

PURPOSE: To identify the characteristics and outcome of panuveitis of undetermined origin (PUO) after diagnostic vitrectomy. METHODS: Retrospective analysis of all patients who underwent vitrectomy for diagnostic/therapeutic purposes from 2013 to 2020, whose vitreous biopsies turned out negative and final diagnoses were not clinically supported. RESULTS: Of 122 operated eyes, 36 eyes (29.5%) were defined as PUO (67.8 ± 14.9 years). The presenting clinical picture revealed a predominantly bilateral condition (70% of eyes) with significant posterior segment involvement: 3.1 ± 0.6 vitritis, 61.1% of eyes with retinal vasculitis, 44.4% with macular edema, and 30.6% with exudative retinal detachment. Presenting visual acuity was 1.2 ± 0.7 logMAR, and up to 90% remained stable or improved vision over a ~ 3.5 year observation period. None of the presenting clinical features turned out to be predictive of final visual outcome or survival. CONCLUSIONS: PUO is present in up to 30% of cases after diagnostic/therapeutic vitrectomy. This mainly bilateral condition shows chronic and overall stable long-term outcome, generally with retained steady visual function.

SYPHILITIC OUTER RETINOPATHY: A MASQUERADING DIAGNOSIS REVEALED AFTER STEROID-INDUCED PROGRESSION TO PANUVEITIS.

PURPOSE: To report a case of syphilitic outer retinopathy revealed after progression to panuveitis after a course of oral steroids for suspected poison ivy. METHODS: Retrospective case report. RESULTS: A 44-year-old diabetic man presented with progressive symptoms of nyctalopia and color vision changes associated with outer retinal disruption on macular imaging but minimal evidence of intraocular inflammation on examination. A short course of oral steroids for an unrelated skin condition induced rapid progression to frank panuveitis with retinal vascular sheathing and retinal whitening. Systemic workup identified syphilis as the etiology. The patient's visual symptoms and disruption of the photoreceptor and retinal pigment epithelial layers on OCT improved after treatment with IV penicillin. CONCLUSION: Syphilitic outer retinopathy represents an unusual manifestation of ocular syphilis that can present with minimal examination findings. We present here a case of oral steroid use resulting in the progression of syphilitic outer retinopathy to a more fulminant form of syphilitic uveitis that ultimately revealed the correct diagnosis and prompted the correct intervention. This case highlights the importance of maintaining a high level of suspicion for this treatable condition.

Response to Comments on 'Acute Bilateral Neuroretinitis and Panuveitis in A Patient with Coronavirus Disease 2019: A Case Report'.

PURPOSE: to respond to comments on our case report on COVID associated neuroretititis. METHODS: We gathered up to date statistics about the prevalence and incidence of epidemic retinitis in Iran and specifically our region, north-east of Iran. RESULTS: Our response to Kawal et al comments includes 3 items. First, the PCR result of the vitreous specimen was positive for COVID-19. Secondly, the clinical course of the patient's illness was typically similar to our numerous COVID patients and we were in the middle of the second peak of COVID at the time. Thirdly, although other causes of epidemic retinitis such as west nile river are relatively rare in our region, we had a significant rise in the incidence of epidemic retinitis in the peak of COVID. So, the most probable cause may be COVID-19. CONCLUSION: Although the ocular findings of our patient was similar to epidemic retinitis caused by other well-known organisms, we believe that based on positive vitreous sample PCR for COVID, typical clinical course of systemic illness, being in the peak of COVID pandemic with significant increase of similar patients during this period, our patient had COVID associated neuroretinitis/ epidemic retinitis.

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.

"Acute Bilateral Neuroretinitis and Panuveitis in A Patient with Coronavirus Disease 2019: A Case Report"- Few Comments.

During the Coronavirus Disease 2019 (COVID-19) pandemic, we have witnessed increase in number of reports of a known uveitic entity being associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Causal relation is yet to be proven for many reports. Perhaps, occurrence of a previously known region-specific endemic disease or closely resembling manifestations of a known disease in a non-endemic region during the COVID-19 pandemic might suggest a causal relationship. Epidemic retinitis (ER) or post fever retinitis is such condition with geographic variation. Occurrence of ER or ER-like manifestations in a non-endemic country during the pandemic should instigate further studies to consider SARS-CoV-2 as a causative organism.

Case Report: Recurrent Severe Uveitis Secondary to Primary Progressive Multiple Sclerosis Responsive to Ocrelizumab.

PURPOSE: To report a case of severe, recurrent bilateral panuveitis secondary to primary progressive multiple sclerosis responsive to ocrelizumab infusions. OBSERVATION: We describe the clinical progression of a 40 year old female who presented with a 3-week history of insidious bilateral visual loss that was clinically consistent with panuveitis. A diagnosis of multiple sclerosis was established with serial magnetic resonance imaging (MRI) that coincided with focal neurological events separated by time. There was initially good response to high dose oral prednisolone; however, the patient would have recurrent uveitis each time the dose was weaned. Under guidance of neurology, we had initiated treatment with ocrelizumab with stability of ocular inflammation for the past 24 months. CONCLUSION: Six-monthly 600mg ocrelizumab infusions may be effective as a steroid sparing option for patients with severe, recurrent bilateral panuveitis secondary to primary progressive multiple sclerosis.