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.

Cooperation with rheumatologists on intensive systemic treatment for psoriatic arthritis-related panuveitis with retinal vasculitis: a case report.

BACKGROUND: Patients with psoriatic arthritis (PsA) may develop uveitis, a potentially serious ocular complication. PsA-related uveitis may result in significant morbidity and even vision loss if underdiagnosed or under-treated. We presented a case with long-standing recurrent uveitis and retinal vasculitis successfully managed by fortified systemic immunomodulators for systemic PsA. CASE PRESENTATION: A 47-year-old woman was referred under the impression of acute anterior uveitis in her right eye in recent one month. Ocular examinations showed panuveitis in both eyes with intense vitreous opacity in her right eye. Fundus fluorescence angiography revealed retinal vasculitis in both eyes. Systemic surveys excluded the possibility of infection but showed an elevated inflammation marker. With intensive immunosuppressive treatment, inflammation resolved and the vision improved. CONCLUSION: Our case highlights not only the importance of intensified systemic therapy in treating PsA-related uveitis but the importance of multidisciplinary collaboration. Recurrent uveitis may be an indicator of disease activity prior to other inflammatory markers.

Successful Treatment Bilateral Panuveitis with Multiple Systemic Infection in HIV/AIDS Patient: A Case Report.

There is an increasing number of HIV/AIDS patients in Indonesia, starting from <0.1% in 2010 to 0.4% in 2012, which warrants awareness of ocular manifestation in HIV. This might appear in 70-100% of patients with HIV. A 47 years old man came to the infection and immunology clinic with blurry vision on both eyes. He had been treated before but there was no clinical improvement. Examination showed both eyes had vitreous haziness. Visual acuity was 1/60 in both eyes with appearance of flare and cells within +3. Uveitis workup showed positive results for HIV, HSV and syphilis. Patient was given 100 mg of doxyciclin two times daily and fixed dose tablet which contains the combination of antiretroviral. Three months later, final acuity was 6/10 on the right eye and 6/18 on the left eye. Prompt diagnosis and treatment warrant good prognosis including multidisciplinary approach by ophthalmologist, clinical allergist and immunologist, and dermato-venerologist.

[OCULAR SARCOIDOSIS].

INTRODUCTION: A 68-year-old healthy female, with no past systemic or ocular history, presented with decreased vision in both eyes. On initial examination, there were extensive vitreal opacities in both eyes. On the following visit, bilateral panuveitis was newly observed, without otherwise systemic clinical manifestations. The patient responded well to treatment with systemic and topical steroids. Initial workup excluded tuberculosis and syphilis. Angiotensin-converting enzyme (ACE) was within normal range. In order to make a definitive diagnosis and to exclude lymphoma, we decided to perform a vitreous biopsy after 2-weeks of steroids withdrawal. After cessation of steroids, new lesions appeared beneath the right eye and over the nasal bridge. Moreover, iris nodules over the stroma, pupil's margin and anterior chamber angle were noticed in both eyes. Biopsy from a skin lesion demonstrated non-caseating granulomas, supporting diagnosis of sarcoidosis. A chest computed tomography (CT) scan demonstrated compatible radiologic findings of bilateral hilar and mediastinal lymphadenopathy. Sarcoidosis is a chronic multisystem, autoimmune, granulomatous disease. Ocular involvement is common among patients with sarcoidosis, with the most common ocular manifestation being uveitis. We report a case that presented with bilateral panuveitis due to sarcoidosis with no systemic manifestations or elevated serum biomarkers. Definitive diagnosis was verified in histopathological findings from a skin biopsy and radiological findings in chest CT.

A case of recalcitrant pediatric Vogt-Koyanagi-Harada disease successfully controlled with adalimumab.

Vogt-Koyanagi-Harada (VKH) disease is uncommon in the pediatric population and can have an aggressive course with serious visual sequelae. A 12-year-old Han Chinese American female, who presented with mild headaches and panuveitis with diffuse serous retinal detachments, was diagnosed with VKH. Despite treatment with a combination of high-dose systemic corticosteroids, intravitreal triamcinolone injection, and mycophenolate mofetil, ocular inflammation was inadequately controlled. Addition of adalimumab allowed for inflammation remission, improvement of vision, and tapering of systemic corticosteroids. Escalation of immunosuppression until remission appears to be critical in this population. Further research is needed to understand the complex pathophysiology of VKH and investigation for similar efficacy of other anti-tumor necrosis factor-alpha agents will need to be performed.

Vision-threatening bilateral panuveitis and TRAPS in a child: an uncommon association.

PURPOSE: To report a childhood case of tumor necrosis factor receptor-associated periodic syndrome (TRAPS) carrying the R92Q variant with a vision-threatening bilateral panuveitis. METHODS: Case report and review of the literature. RESULTS: A 7-year-old boy presented with an active bilateral panuveitis and a macular rash associated with fever. Fundus examination showed two choroidal lesions on the posterior pole of the right eye, and fluorescein angiography revealed early hypofluorescence and late hyperfluorescence of the lesions, which were hyper-autofluorescent. Extensive clinical laboratory analyses ruled out autoimmune diseases and systemic infection. The only remarkable finding was a positive IgG for herpes simplex 1. He underwent two successive diagnostic pars plana vitrectomies as well as cataract and glaucoma surgeries. Genetic analysis revealed a mutation in the TNFRSF1A gene, and the patient was diagnosed with TRAPS-associated bilateral panuveitis. He was treated with adalimumab and has been free of active inflammation since then. CONCLUSIONS: We present here the first case reported of panuveitis in a patient with TRAPS. This finding stresses the increasing importance of genetic analysis in search of autoinflammatory diseases to establish an adequate diagnosis and treatment in cases of uveitis of unknown etiology.

A challenged case of Vogt-Koyanagi-Harada syndrome: when dermatological manifestations came first.

INTRODUCTION: Vogt-Koyanagi-Harada syndrome (VKHS) is an inflammatory systemic autoimmune disease principally affecting pigmented tissues in the ocular, auditory, integumentary and central nervous systems. Patients are generally women in the fourth decade of life. The prognosis is correlated mainly with the time between diagnosis and the start of treatment and number of recurrent episodes of inflammation. Most complications are mainly ocular. The purpose of this paper is to describe a clinical case of VKHS. MATERIAL AND METHODS: A child with a challenging clinical presentation in which the dermatological symptoms occurred before ocular manifestations. DISCUSSION AND CONCLUSION: VKHS is rare in children and can be a diagnostic challenge. It seemed interesting to share this case as an opportunity to expand our knowledge of the clinical spectrum of diseases and reflect about current diagnostic criteria.

Canine panuveitis: a retrospective evaluation of 55 cases (2000-2015).

OBJECTIVE: To determine the most common etiologies of panuveitis in a group of dogs, patient response to therapy, and visual outcome. DESIGN: Retrospective study. MATERIALS AND METHODS: Medical records of 55 dogs diagnosed with panuveitis at the Purdue University Veterinary Teaching Hospital between December 2000 and December 2015 were reviewed. Data collected included signalment, season of diagnosis, descriptions of ocular lesions, diagnostic testing, treatment protocols, etiologic diagnoses (when determined), and visual outcome. RESULTS: The most common clinical sign in the anterior segment was aqueous flare and in the posterior segment was serous retinal detachment. The most common diagnosis was idiopathic/immune-mediated disease followed by systemic blastomycosis, lymphoma, and other causes (leptospirosis, aspergillosis, and histoplasmosis). Of the 43 dogs for which follow-up was available, 40% of dogs had resolution of visible inflammation and retinal reattachment, 32% of dogs had slight to moderate improvement, and 28% of dogs had no improvement or deterioration of lesions. Mean follow-up time was 11 months, and mean time to resolution of visible inflammation and retinal reattachment was 32 days. Sixty-five percent of dogs either retained or regained vision in one or both diseased eyes with initial medical therapy. CONCLUSIONS: Idiopathic/immune-mediated panuveitis was the most common diagnosis. Dogs with idiopathic/immune-mediated panuveitis tended to respond more favorably to medical therapy than dogs with infectious panuveitis. Visual outcome was favorable in those dogs that responded to initial medical therapy, but long-term therapy was required in many dogs to maintain vision and prevent recurrence of disease.

[Panuveitis with oral and genital ulcer misdiagnosed as Behcet's disease: two cases report and literature review].

Here we reported two patients who presented with panuveitis and were transferred from ophthalmologists to rheumatologists, for both the patients had oral and genital ulcers. They were misdiagnosed with Behcet's disease at first glance. Two young males presented with acute uveitis with history of recurrent oral and genital ulcers. They initially presented with symptoms and signs resembling Behcet's disease and were treated with systemic steroids with suboptimal responses. Routine laboratory test revealed syphilis and human immunodeficiency virus (HIV) infection. After treatment of penicillin and anti HIV virus therapy, the panuveitis was relived. The other patient was lost in the follow up. Recently epidemiological data indicate that syphilis and HIV infection increase, which can mimic the manifestation of Behcet's disease. Diagnosis of sexual transmitted diseases, such as HIV or syphilis needs to be ruled out in all cases that mimic the clinical feature of Behcet's disease, especially for those who had a history of high risk behaviors. Every patient should have history analysis in detail. Screening of sexual transmitted diseases, such as HIV or syphilis is important especially in those rapid progressive panuveitis. Also, other virus infections, such as cytomegalovirus, epstein-barr virus or Herpes simplex virus can cause mucosa ulcers and uveitis. CD4 T cell count is a very important marker to indicate that the patient has immunodeficiency. Erythema nodosa and pseudofolliculitis are the third common clinical manifestation in Chinese Behcet's disease patients. Rheumatologist should watch out for patients without skin involvement when making the diagnosis of Behcet's disease. Syphilis-associated uveitis usually has a good prognosis. Treatment of antibiotics can get good response, 92% uveitis can be relieved, with 67% improved vision. Acute syphilitic posterior placoid chorioretinitis (ASPPC) is a clinically and angiographically distinct manifestation of ocular syphilis. Systemic glucocorticoid can be used in syphilis induced posterior uveitis, sleritis and optic neuritis, and it can also prevent the Hector's reaction. However, for patients diagnosed with both HIV and syphilis, regular antibiotic can not prevent relapse. So doctors need to follow up them regularly. Patients who present with uveitis, oral and genital ulcers can be easily diagnosed with Behcet's disease. Rheumatologists need to be aware of the reemergence of sexual transmitted disease. High degree of clinical suspicion can allow ophthalmologists and rheumatologists to diagnose and treat the disease early. Correct diagnoses timely can get the good treatment response, and rescue the vision. Treatment with regular antivirus and Penicillin can receive the good response, and moreover glucocorticoid can relieve the inflammation.

Role of vitrectomy for syphilitic uveitis in a tertiary hospital in Spain.

Diagnosis and treatment of ocular syphilis can be challenging due to the wide spectrum of clinical presentations of this sexually transmitted disease. In some cases of syphilitic panuveitis, pars plana vitrectomy (PPV) can be useful in management since it plays an important role in improving fundus examination allowing treatment of possible retinal associated lesions when vitreous inflammation is intense. We present 3 cases of patients with ocular syphilis that underwent a therapeutic PPV, vitreous sample was taken and analyzed in two of them.

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.

Unmasking an unusual presentation of Hodgkin's lymphoma masquerading as ocular inflammation: a case report.

BACKGROUND: Hodgkin's lymphoma (HL) is an extremely rare cause of ocular inflammation that is usually not considered in the typical workup of uveitis and other eye diseases. A few cases of ocular inflammation were reported previously showcasing HL with absence of typical symptoms of HL at presentation. Acknowledging the potential ocular inflammation associated with HL can prompt ophthalmologists to broaden their diagnostic approach and collaborate with internal medicine departments to investigate this rare yet significant etiology. CASE PRESENTATION: A 17-year-old Caucasian woman presenting unilateral panuveitis was later diagnosed with HL. The ocular findings were non-necrotizing scleritis, anterior uveitis, vitritis, white/yellowish chorioretinal lesions, papillitis and vasculitis. A left supra-clavicular lymph node biopsy confirmed the diagnosis of nodular sclerosing Hodgkin's lymphoma stage IIB. Other causes of uveitis were excluded. Chemotherapy led to remission of the disease and the ocular lesions became quiescent with persistent pigmented chorioretinal scars. CONCLUSIONS: Hodgkin's lymphoma should be considered in the differential diagnosis of diseases that can occasionally be revealed by unilateral ocular inflammation. A comprehensive, multidisciplinary approach is key to properly assessing such cases.

A Case of Refractory Posterior Scleritis with Marked Retinochoroidal Detachment Associated with Panuveitis.

An 84-year-old man presented with decreased right-eye visual acuity. Upon initial examination, the rightand left-eye visual acuities were 0.03 and 1.2, respectively; moreover, the right- and left-eye intraocular pressure was 12 mmHg and 13 mmHg, respectively. Examination revealed a shallow anterior chamber of the right eye, anterior chamber inflammation, vitreous opacity, and marked retinochoroidal detachment. Optical coherence tomography (OCT) revealed retinal detachment (RD) and choroidal folds; moreover, B-scan ultrasonography (B-scan) showed RD as well as thickened sclera with fluid in Tenon's space. Fluorescent fundus angiography revealed hyperfluorescence in the optic disc and vascular hyperpermeability in the right eye. The left eye lacked extra-ocular symptoms or abnormalities. The right ocular axis measured 23.4 mm with no apparent subretinal fluid migration due to positional changes. Accordingly, the patient was diagnosed with panuveitis associated with posterior scleritis and immediately started on 40 mg prednisolone, which improved his symptoms. However, at 3 post-treatment months, choroidal folds were observed and was restarted on 20 mg prednisolone. The choroidal folds subsequently disappeared, with a current visual acuity of 0.3 in the right eye and no recurrence. Our findings indicated the utility of accurate diagnosis of posterior scleritis by B-scan and prompt systemic steroid administration.

A case of uveitis in adult-onset Still's disease with ophthalmologic symptoms.

Adult-onset Still's disease (AOSD) is a rare and systemic inflammatory disorder of unknown etiology and pathogenesis. AOSD is characterized by high fever accompanied by a range of systemic symptoms. However, there are rare cases of AOSD with ophthalmologic symptoms as well as with an obvious causation of corticosteroid withdrawal. In this case, a 43-year-old male patient diagnosed with AOSD showed ocular inflammation after withdrawing from corticosteroid treatment. This patient was treated with prednisolone for AOSD and discharged after achieving complete remission of breathlessness, backache, thoracalgia, joint pain, and spiking fever. The patient unauthorizedly stopped taking prednisolone after he was discharged from the hospital and returned to the Department of Ophthalmology with the complaint of decreased visual acuity in both eyes for half a month and sudden vision loss in the left eye for 3 days. After regular ophthalmologic examinations and fluorescence angiography examination, he was diagnosed with acute panuveitis as the manifestation of AOSD. Uveitis was effectively treated with corticosteroid drugs. This case reported a rare manifestation of AOSD in an ophthalmological system that was associated with the withdrawal of corticosteroid treatment. This report highlighted the therapeutic effect of local and systemic corticosteroid use for AOSD manifested with uveitis. This case is interesting for both rheumatologists and ophthalmologists.

Unusual Presentation of Skin Tattoo-Associated Panuveitis.

AIM: We report a case of a young male who developed bilateral panuveitis after extensive tattooing. DESIGN: Case report. METHODS: A 22-year-old male with a history of inflamed tattoos presented with pain in both eyes and blurred vision in the left eye. Clinical examination showed ciliary congestion, flare, vitreous cells in both eyes, and posterior synechiae in the left eye. Optic nerve was swollen in both eyes. OCT scans demonstrated subretinal blood, associated with neurosensory macular detachment in the left eye. The skin tattoo biopsy showed a granulomatous inflammation without evidence of sarcoidosis. Long-term corticosteroid therapy allowed a regression of clinical signs and symptoms with full recovery. CONCLUSION: TAttoo Granulomas with Uveitis (TAGU) is a syndrome with numerous clinical presentations. In our case, optic nerve head oedema and subretinal hemorrhage at the posterior pole were the presentation signs. Ophthalmologists should always consider TAGU as a diagnosis in patients with a history of inflamed tattoos.

PRESUMED PANUVEITIS FOLLOWING COVID-19 VACCINATION IN A PATIENT WITH GRANULOMATOUS TATTOO INFLAMMATION.

PURPOSE: To report a case of panuveitis that developed following COVID-19 vaccination in a patient with a recent history of granulomatous tattoo inflammation. METHODS: Case report. RESULTS: A 25-year-old woman with a recent history of biopsy-proven granulomatous tattoo inflammation developed bilateral eye pain and blurred vision 1 week following her second mRNA-1273 COVID-19 vaccination (Moderna, Inc, Cambridge, MA). Examination revealed bilateral panuveitis. Workup for infectious etiologies and sarcoidosis was negative. The intraocular inflammation initially resolved with systemic prednisone therapy but then recurred following tapering, requiring the initiation of mycophenolate mofetil. CONCLUSION: A case of panuveitis that developed following a COVID-19 vaccination in a patient with a recent history of tattoo inflammation is reported. The temporal relationship between the vaccine and the development of uveitis in this patient may be coincidental and should be interpreted with caution, but multiple vaccines have been associated with uveitis, presumably as a result of their generalized stimulation of the immune system. It is believed that this case of tattoo-associated uveitis may have been exacerbated by the generalized inflammatory effect of COVID-19 vaccination.

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.

Uveitis in Sarcoidosis - Clinical Features and Comparison with Other Non-infectious Uveitis.

PURPOSE: Comparison of sarcoid uveitis with other non-infectious uveitis treatment and visual outcomes. METHODS: Retrospective study of 287 eyes with sarcoid uveitis and 1517 eyes with other non-infectious uveitis (15,029 eye-years follow-up). RESULTS: Sarcoid uveitis patients presented at age 43.1 ± 0.8 years, and 66.2% were female. Panuveitis was the most frequent presentation (48.3%), and 90.1% were bilateral. Moderate visual loss (≤20/50) developed in 19 eyes (6.6%), and severe visual loss (≤20/200) in 13 eyes (4.5%). Sarcoid uveitis had better visual outcomes than other non-infectious uveitis (10-year BCVA anterior uveitis 0.06 vs 0.24 p = .002; posterior disease 0.17 vs 0.38 p = .001). Oral corticosteroid use was more common with sarcoid uveitis (anterior uveitis 45.9% vs 16.4% p < .0005; posterior disease 64.0% vs 61.7% p = .635), but second-line immunosuppression was required less frequently (p = .008). CONCLUSIONS: Compared to other non-infectious uveitis, sarcoid uveitis has better visual acuity outcomes and is less likely to require second-line immunosuppression.