Spiroplasma infection as a cause of severe congenital keratouveitis, cataract and glaucoma.

BACKGROUND: Only seven cases of ocular Spiroplasma infection have been reported to date, all presenting as congenital cataracts with concomitant intraocular inflammation. We describe the first case of Spiroplasma infection initially presenting as a corneal infiltrate. CASE PRESENTATION: A 1-month-old girl was referred for a corneal infiltrate in the left eye. She presented in our hospital with unilateral keratouveitis. Examination showed a stromal corneal infiltrate and dense white keratic precipitates in the left eye. Herpetic keratouveitis was suspected and intravenous acyclovir therapy was initiated. Two weeks later, the inflammation in the left eye persisted and was also noticed in the right eye. Acute angle-closure glaucoma and a cataract with dilated iris vessels extending onto the anterior lens capsule developed in the left eye. The inflammation resolved after treatment with azithromycin. Iridectomy, synechiolysis and lensectomy were performed. Bacterial metagenomic sequencing (16 S rRNA) and transmission electron microscopy revealed Spiroplasma ixodetis species in lens aspirates and biopsy. Consequently, a diagnosis of bilateral Spiroplasma uveitis was made. CONCLUSIONS: In cases of congenital cataract with concomitant intraocular inflammation, Spiroplasma infection should be considered. The purpose of this case report is to raise awareness of congenital Spiroplasma infection as a cause of severe keratouveitis, cataract and angle-closure glaucoma in newborns. Performing molecular testing on lens aspirates is essential to confirm diagnosis. Systemic macrolides are suggested as the mainstay of treatment.

Posterior segment parameters after uveitic cataract surgery: A prospective study with 1-year results.

PURPOSE: To evaluate the changes in posterior segment after uncomplicated cataract surgery in uveitic patients. METHODS: Retinal nerve fiber layer thickness (RNFLT), ganglion cell layer thickness (GCLT), central macular thickness (CMT), and choroidal thickness (CT) of 38 eyes of 28 patients were measured pre- and postoperatively on day 1, week 1, and month 1, 3, 6, 9, and 12. RESULTS: The RNFLT increased after surgery. Although the measurements taken were higher than the baseline CMT at all postoperative times, no significant difference was detected between the paired comparisons. The GCLT was found to be higher than the baseline value in all quadrants at the 12th month. A decrease in CT was observed at 5 measured points on the 1st day compared to the baseline. CONCLUSION: During the 1-year follow-up, the effect of cataract surgery on the retina and choroid in uveitic eyes was most evident at the postoperative month 1.

Unilateral Acute Retinal Necrosis with Contralateral Non-necrotizing Herpetic Uveitis.

PURPOSE: The objective of this study is to report a case of unilateral acute retinal necrosis (ARN) with contralateral eye presenting as non-necrotizing herpetic uveitis. CASE REPORTS: Case 1: A 48-year-old female presented at our clinic with blurred vision in the right eye for 7 days. She was diagnosed with ARN in the left eye 2 weeks ago. Ophthalmic examination revealed reduced visual acuity in the right eye (20/33) with the presence of optic disc swelling and macular exudation without peripheral necrotic lesions. With systemic antiviral therapy, optic disc swelling of the right eye vanished gradually, and the visual acuity improved to 20/20. Loss of retinal nerve fiber layer (RNFL) and decreased retinal thickness in the corresponding area occurred during follow-up. CONCLUSION: Non-necrotizing herpetic uveitis may occur in the contralateral eye of unilateral ARN under rare conditions. Structure abnormities, including loss of RNFL and focal decreased retinal thickness, are irretrievable.

Association between Tubulointerstitial Nephritis and Uveitis Syndrome and Small-Vessel CNS Vasculitis: A Case of Polyautoimmunity.

INTRODUCTION: We report a case study of two male pediatric patients presenting with anterior uveitis and elevated renal function parameters. Both were diagnosed with tubulointerstitial nephritis and uveitis syndrome and subsequently developed diffuse cerebral symptoms such as headache, fatigue, and diziness. METHODS: Magnetic resonance images (MRIs) of the brain showed T2-hyperintense lesions with and without gadolinium enhancement leading to brain biopsy and diagnosis of small-vessel central nervous system (CNS) vasculitis in both cases. Both patients were treated according to BrainWorks small-vessel vasculitis protocol and symptoms vanished over the course of treatment. Follow-up MRIs up to 12 months after initiation of therapy showed no signs of recurrence indicating a monophasic disease. CONCLUSION: Small-vessel CNS vasculitis can occur simultaneously to other autoimmune diseases (ADs) in the scope of polyautoimmunity. As clinical findings of CNS vasculitis are often unspecific, neurological symptoms in nonneurological ADs should be adressed thoroughly. Under suspicion of small-vessel CNS vasculitis brain biopsy is still the gold standard and only secure way of definitive diagnosis.

Persistent Monkeypox-Associated Keratouveitis in a Post-LASIK Cornea: A Case Report With Flap Removal.

PURPOSE: This study aimed to report a case of persistent keratouveitis associated with mpox virus infection in an immunocompetent patient with a history of laser-assisted in situ keratomileusis (LASIK), leading to the need for flap removal. METHODS: A comprehensive literature review was conducted, and a detailed case report was presented. RESULTS: A 44-year-old immunocompetent male patient with a history of LASIK and HIV infection presented with conjunctival and corneal lesions indicative of mpox infection. Despite initial topical treatment, the condition worsened, leading to severe epithelial and stromal keratitis with anterior chamber inflammation. Polymerase chain reaction testing confirmed the presence of mpox virus in conjunctival, corneal, and aqueous humor samples. The patient underwent various treatments, including tecovirimat, oral and topical corticosteroids, and topical trifluridine, and eventually flap removal with amniotic membrane application. Subsequent polymerase chain reaction testing of the aqueous humor yielded negative results, and the cornea gradually reepithelialized without inflammation. After 8 months, the patient's eye remained stable, with a central stromal scar. CONCLUSIONS: This case highlights the rare occurrence of mpox-associated keratouveitis and emphasizes the challenges faced in its management. The successful outcome achieved through a combination of antiviral therapy, antiinflammatories, and surgical intervention underscores the importance of a multidisciplinary approach in managing such cases.

Outcome of cataract surgery in children with presumed trematode-induced granulomatous anterior uveitis.

PURPOSE: To examine the 6-month visual outcomes and complications following cataract surgery in patients with persumed trematode induced granulomatous anterior uveitis. SETTING: Assiut university hospital, Assiut, Egypt. DESIGN: This is a retrospective non comparative case series study. METHODS: Patients presenting with significant cataract secondary to uveitis caused by trematode induced anterior chamber granuloma were included in this study. Cases with active anterior uveitis, within the last 3 months preceding surgery, and those with a history of trauma, were excluded from this study. Data collected included demographic characteristics, history of the condition including when uveitis started, treatment received and history of other health conditions that may be relevant to uveitis.Complete opthalmologic examination including assessment of best corrected visual acuity (BCVA) and OCT macula, if possible, were done. These was repeated 1 week, 1 month, 3 months and 6 months after surgery. Specular microscopy was performed preoperatively and 3 months after surgery. Patients underwent cataract surgery with posterior chamber intra ocular lens and statistical analysis was performed to compare preoperative and postoperative BCVA and corneal endothelial cell counts. Postoperative complications were recorded. RESULTS: Five eyes of 5 patients were included in the study. All study eyes showed improvement in the post-operative visual acuity. A statistically significant improvement was observed in VA in the sixth postoperative month compared to the baseline measurements (p = 0.004). No statistically significant difference was observed between the preoperative and postoperative endothelial cell counts (p = 0.696). Cystoid macular edema did not occur as a postoperative complication. CONCLUSION: Visual outcomes of cataract surgery in eyes with persumed trematode induced granulametous anterior uveitis are favorable. No sight threatening complication was observed in our series.

Simultaneous evaluation of iris area and subfoveal choroidal thickness in Fuchs uveitis syndrome.

BACKGROUND/AIMS: To simultaneously evaluate iris area (IA) and subfoveal choroidal thickness (SFCT) in eyes with Fuchs Uveitis Syndrome (FUS). METHODS: We prospectively recruited a case series of patients with FUS at our institution, simultaneously measuring IA with anterior segment spectral domain optical coherence tomography (SD-OCT) and SFCT with enhanced depth imaging optical coherence tomography (EDI-OCT). Iris images were analyzed by ImageJ software. We tested the differences in intereye IA and SFCT with the healthy eye (HE) using the Wilcoxon test, and clinical interpretation was controlled by intraclass correlation coefficient (ICC) between two masked specialists. RESULTS: Sixteen patients with unilateral FUS were included. Six were female, and the age range was 37 to 67 (median age 48 years, IQR 41-60). ICC of 98.9%, with a lower confidence interval of 97%. Eyes with FUS had a significant thinning of the total iris median area (p < 0.002), restricted to the temporal and nasal areas compared to the HE (p < 0.01 and < 0.001, respectively). SFCT was also significantly thinner compared to the HE (p < 0.0001). A low correlation was found between iris and choroidal thinning in FUS eyes (rs = 0.21; p = 0.4). CONCLUSIONS: This study found reduced iris area and subfoveal choroidal thickness in eyes with FUS compared to the normal fellow eye.

In Response to Oyeniran E, Katz D, Kodati S's Isolated Optic Disc Granuloma as a Presenting Sign of Sarcoidosis.

We read with great interest the article by Oyeniran E et al. on "Isolated optic disc granuloma as a presenting sign of sarcoidosis." We would like to share our experience with a similar optic nerve head granuloma secondary to sarcoidosis in the absence of any systemic symptoms and no evidence of signs of periocular/intraocular inflammation. However, our case was refractory to oral steroids and methotrexate and required intravitreal dexamethasone implants and mycophenolate mofetil.

Cerebro-Ocular Toxocariasis Revealed by Bilateral Cystoid Macular Edema.

DESIGN: Case Report Case description: This report describes the case of a female patient diagnosed with oculo-cerebral toxocariasis manifesting initially in the form of isolated bilateral cystoid macular edema. Diagnosis was made by means of positive anterior chamber and lumbar puncture western blots. The unusual presentation, ancillary findings and treatment are discussed. The control of intraocular inflammation that was only partially responsive to steroids was eventually achieved with pegylated interferon alfa-2a. CONCLUSION: Isolated macular edema is a rare presentation of ocular toxocariasis. Interferon alfa-2a may prove useful in case of insufficient control of inflammation.

An Unusual Manifestation of Brucella-Associated Uveitis in a Young Male Patient.

This is a unique case of retinoschisis as an ocular manifestation of brucellosis. A 38-year-old male presented with recurrent episodes of bilateral eye redness, predominately in his left eye. His visual acuity was not affected, and he did not report any other symptoms. On slit lamp examination, binocular Koeppe nodules of the iris and cells in the left anterior chamber were observed. Fundoscopy followed by meticulous multimodal imaging confirmed left inferior retinoschisis. The patient was diagnosed with panuveitis, and a series of laboratory examinations revealed positive anti-IgM Brucella antibodies. Ocular brucellosis can cause variable, atypical, and serious presentations, hence, early diagnosis is paramount to avoid complications.

Diagnosis, Treatment, and Prevention of Noninfectious Acute Anterior Uveitis with or without Human Leukocyte Antigen B27 in Adults - Expert Consensus in Taiwan.

BACKGROUND: Anterior uveitis is the most common anatomical type of uveitis. Patients with noninfectious anterior uveitis may develop various ocular complications and eventually visual impairment. Appropriately differentiating the etiologies can help clinicians to predict the outcome, arrange clinical follow-up, and decide the treatment or prevention strategy. Adequate treatment and effective prevention strategies can reduce the frequency of recurrence and the risk of developing complications. Human leukocyte antigen (HLA)-B27 is the most common positive finding in patients with noninfectious AAU in many countries including Taiwan. PURPOSE: To report a consensus from experienced uveitis specialists and rheumatologists was made in Taiwan. METHODS: A panel of nine ophthalmologists from nine different referral centers with expertise in the management of uveitis and an experienced rheumatologist was held on January 16, 2022. A comprehensive literature review was performed. Differential diagnoses for etiologies, general treatments, and prevention strategies were discussed. Each statement in the consensus was made only if more than 70% of the experts agreed. RESULTS: A flow chart and seven statements regarding the differential diagnoses for etiologies, treatments and preventions, and co-management with rheumatologists were included in the consensus. CONCLUSIONS: This article discusses the general diagnosis, treatment, and prevention of noninfectious acute anterior uveitis, with or without HLA-B27, in adults for general ophthalmologists to improve overall outcomes of these patients.

Characteristics, Etiology, and Clinical Outcome of Retinal Vasculitis in Tertiary Hospital in Thailand.

OBJECTIVE: To analyze characteristics, etiology, and outcome of retinal vasculitis in Central Thailand. METHODS: A retrospective cohort study. RESULTS: Retinal vasculitis was found in 10% of uveitis, 74 from 741 uveitis, noninfectious (64.9%) and infectious group (35.1%). The most common cause was Behcet's disease (48.6%). Behcet's disease was the most common cause of all types of vascular leakage on angiography, including capillary (80.4%), venous (56.3%), and arterial leakage (56%). Final visual acuity was 0.86 ± 0.97 logMAR. Cataract was the most frequent complication (42.5%). Acute clinical course (p = .025) and retinal neovascularization (p = .031) were associated with infectious group. Forty-three percent of vasculitis complicated by ischemia required photocoagulation (33%) and anti-VEGF injection (17%). Furthermore, 17% of vasculitis underwent vitrectomy. CONCLUSION: One-half of the retinal vasculitis in Central Thailand were Behcet's disease. Acute onset and retinal neovascularization may suggest infectious etiology. Retinal ischemia should be cautious and undergo early interventions to prevent sight-threatening complications.

Hydroxychloroquine Therapy in Sarcoidosis-Associated Uveitis.

BACKGROUND/PURPOSE: To assess the efficacy and tolerance of hydroxychloroquine in sarcoidosis-associated uveitis. METHODS: Retrospective study on all patients with sarcoidosis-associated uveitis who were treated with hydroxychloroquine between 2003 and 2019 in a French university hospital. RESULTS: Twenty-seven patients with sarcoidosis-associated uveitis received hydroxychloroquine. The mean duration of treatment was 20.0 ± 10.9 months. At the end of the follow-up, hydroxychloroquine success was achieved in 15 (55.6%) patients. Four of them were also on oral corticosteroids, with a prednisone dose ≤5 mg/day. Under treatment, the median prednisone dose decreased from 20.0 (interquartile range (IQR), 7-25) to 5.0 (IQR, 3-6.5) mg/day (p = .02). The incidence rate of flare decreased from 204.6 to 63.8 per 100 person-years (p = .02). Hydroxychloroquine was discontinued in 12 (44.4%) patients during follow-up, including 8 (29.6%) for ineffectiveness, and three who experienced side effects. CONCLUSION: Hydroxychloroquine appears as an interesting option in sarcoidosis-associated uveitis.Abbreviations: AZA: Azathioprine; BAL: Bronchoalveolar Lavage; BCVA: Best-Corrected Visual Acuity; ENT: Ears, Nose and Throat; HCQ: Hydroxychloroquine; IOP: Intra-Ocular Pressure; IQR: interquartile range; MHC: Major Histocompatibility Complex; MMF: Mycophenolate Mofetil; MTX: Methotrexate; PMSI: Programme de Médicalisation du Système d'Information; SAU: Sarcoidosis-Associated Uveitis; SD: Standard Deviation; SUN: Standard Uveitis Nomenclature.

Hyperproliferation of Elschnig pearl-type posterior capsule opacification and spontaneous regression in two pseudophakic canine eyes.

OBJECTIVE: To describe the hyperproliferation of Elschnig pearl-type posterior capsule opacification and concurrent uveitis in two canine eyes after phacoemulsification, followed by spontaneous resolution of the Elschnig pearls. ANIMAL STUDIED: A 10-year-old castrated male Spitz (Case 1) and a 4-year-old spayed female Bichon Frise (Case 2). PROCEDURE: Elschnig pearls proliferating beyond the anterior capsulotomy site were observed in the right eye 10 months after bilateral diabetic cataract surgery (Case 1) and 7 months after unilateral cataract surgery (Case 2). In both cases, hyperproliferation occurred where the anterior capsule did not overlap with the intraocular lens (IOL), and was accompanied by aqueous flare. In Case 1, the pearls extended from the anterior capsule and adhered to the iris, causing focal posterior synechia. No other possible causes of uveitis were apparent. RESULTS: Initially, uveitis severity improved after the administration of topical and systemic anti-inflammatory drugs. However, uveitis recurred when the dosage of anti-inflammatory treatment was reduced. The Elschnig pearls underwent morphological changes throughout the follow-up period. In both cases, the pearls beyond the anterior capsulotomy resolved spontaneously after 5 months. Only a few pearls remained between the IOL and posterior capsule, and no recurrence of pearl proliferation was observed at the last follow-up. CONCLUSIONS: To the best of our knowledge, this is the first report of spontaneous Elschnig pearl regression in dogs. Lens-induced uveitis (LIU) may have been caused by anterior chamber hyperproliferative pearls. LIU associated with hyperproliferative pearls may be managed with appropriate anti-inflammatory treatment and monitoring.

The risk factors for uveitis among psoriatic arthritis patients: a population-based cohort study.

OBJECTIVE: To assess the frequency of uveitis in patients with psoriatic arthritis (PsA) in the era of biologics and to identify risk factors associated with uveitis. METHODS: A retrospective matched cohort study was conducted within the database of a large healthcare provider. Newly diagnosed 6147 adult PsA patients between 2005 and 2020 were matched by the index date of PsA diagnosis, age, sex, and ethnicity to 23,999 randomly selected controls. This cohort was used to examine the association between PsA and uveitis. An additional analysis was conducted within the PsA group to identify uveitis risk factors, using two analytic approaches: a retrospective cohort study and a nested case-control study. RESULTS: Uveitis was diagnosed in 107 patients in the PsA group (1.7%) vs 187 (0.8%) patients in the control group (adjusted HR, 2.38, 95% CI 1.80-3.15, p<0.005) and was similar when the analysis was confined to patients without past uveitis. Uveitis was diagnosed more in females (2.1% vs 1.3%, HR 1.61, 95% CI 1.09-2.40, p<0.05), and was acute in all cases. Anterior uveitis was documented in 41.1% of the cases, 64.5% unilateral, and 9.3% bilateral. In the PsA group, using nested case control approach, only past uveitis [adjusted OR 136.4 (95% CI 27.38-679.88), p<0.005] and treatment with etanercept [adjusted OR 2.57 (95% CI 1.45-4.57), p=0.001] were independently associated with uveitis. Only one PsA patient with uveitis (out of 107) required systemic oral treatment with prednisone, while the rest of the patients were treated with topical glucocorticosteroids only. CONCLUSION: PsA is associated with increased risk of uveitis. Past uveitis and treatment with etanercept were associated with higher risk of uveitis. Key Points • Psoriatic arthritis (PsA) is a major risk factor for uveitis with hazard ratio of 2.38 compared to healthy individuals without PsA. • Among PsA patients, the past event of uveitis and treatment with etanercept are risk factors for uveitis. • Uveitis in patients treated with biologics for their PsA requires topical therapy only in most of the cases.

Etanercept-associated episcleritis: a pediatric case report of a paradoxical adverse reaction and review of the literature.

Scleritis is an inflammation of the episcleral and scleral tissues, characterized by injection in both superficial and deep episcleral vessels. When only episcleral tissue is involved, it is referred to as episcleritis. Episcleritis is mainly idiopathic but may be secondary to an underlying rheumatologic disease. Despite being rare, drug-associated episcleritis and scleritis should also be included in the differential diagnosis. Tumor necrosis factor-alpha (TNF-α) inhibitors are generally well-tolerated, but etanercept, in particular, has the potential to cause paradoxical adverse reactions including ocular inflammations, such as uveitis, scleritis, and ocular myositis. Etanercept differs in its mechanism of action from other TNF-α inhibitors as it acts as a decoy receptor, and this may partly explain the more frequently reported etanercept-associated ocular inflammation. Etanercept may also be ineffective in preventing ocular inflammation. However, the dechallenge and rechallenge phenomena have proven there is a causative link between etanercept and new-onset ocular inflammation. We report a case of a 15-year-old boy with enthesitis-related arthritis and familial Mediterranean fever who presented with episcleritis and blepharitis while receiving etanercept treatment and subsequently showed dechallenge and rechallenge reactions. Therefore, physicians should also be aware that episcleritis should be considered a paradoxical adverse reaction to etanercept and can occur in pediatric patients. We also reviewed the English literature to provide an overview and evaluate intervention options.

Long-term visual acuity outcomes following cataract surgery in eyes with ocular inflammatory disease.

PURPOSE: To evaluate the long-term visual acuity (VA) outcome of cataract surgery in inflammatory eye disease. SETTING: Tertiary care academic centres. DESIGN: Multicentre retrospective cohort study. METHODS: A total of 1741 patients with non-infectious inflammatory eye disease (2382 eyes) who underwent cataract surgery while under tertiary uveitis management were included. Standardised chart review was used to gather clinical data. Multivariable logistic regression models with adjustment for intereye correlations were performed to evaluate the prognostic factors for VA outcomes. Main outcome measure was VA after cataract surgery. RESULTS: Uveitic eyes independent of anatomical location showed improved VA from baseline (mean 20/200) to within 3 months (mean 20/63) of cataract surgery and maintained through at least 5 years of follow-up (mean 20/63). Eyes that achieved 20/40 or better VA at 1 year were more likely to have scleritis (OR=1.34, p<0.0001) or anterior uveitis (OR=2.2, p<0.0001), VA 20/50 to 20/80 (OR 4.76 as compared with worse than 20/200, p<0.0001) preoperatively, inactive uveitis (OR=1.49, p=0.03), have undergone phacoemulsification (OR=1.45 as compared with extracapsular cataract extraction, p=0.04) or have had intraocular lens placement (OR=2.13, p=0.01). Adults had better VA immediately after surgery, with only 39% (57/146) paediatric eyes at 20/40 or better at 1 year. CONCLUSIONS: Our results suggest that adult and paediatric eyes with uveitis typically have improved VA following cataract surgery and remain stable thereafter for at least 5 years.

Tubulointerstitial Nephritis and Uveitis Syndrome: A Report of 6 Cases with Renal Biopsy and Electron Microscopy Evaluation.

Tubulointerstitial nephritis with uveitis syndrome is a rare, immune-mediated entity, characterized by oculo-renal inflammation. Diagnosis requires the exclusion of all other causes of tubulointerstitial nephritis (TIN). We present 6 patients with clinical, laboratory, and renal biopsy findings denotative of tubulointerstitial nephritis with uveitis syndrome. All our patients experienced ocular and renal manifestations, defined by bilateral uveitis and photosensitivity, along with a decline of renal function. In some patients, increased serum creatinine was accompanied by non-nephrotic range proteinuria, glucosuria or "full-blown" Fanconi syndrome. The rest of the laboratory evaluation was normal apart from the presence of elevated erythrocyte sedimentation rate and increased urine ß2-microglobulin, as well as normochromic, normocytic anemia in some cases. All patients underwent renal biopsy. Histochemical (PAS, Masson, silver, Congo-red) and immunohistochemical stains for immune cell populations (CD3, CD20, CD4, CD8, PGM1, CD138) and for the assessment of ß2-microglobulin were conducted. Electron microscopy examination of the biopsies was also performed. Follow-up, ranging from 18 months to 10 years, was available for 4 patients. Histological evaluation revealed interstitial inflammatory infiltration consisting mainly of lymphocytes, with a T-cell predominance, along with several macrophages. Inflammation severity varied among different patients, with some showing scarce foci of immune cell clusters, while others demonstrated a dense, diffuse interstitial infiltration. Interestingly, in 2 cases, a granulomatous pattern, characterized by non-necrotic, ill-defined granulomas was detected. Tubulitis was also encountered in some patients. A divergence was noted regarding the chronicity index, with different levels of tubular atrophy, interstitial fibrosis, and global glomerulosclerosis among different cases. ß2-Microglobulin immunohistochemical evaluation revealed a substantial diminishment of cytoplasmic staining in tubular epithelial cells compared to control kidneys. The most notable finding derived from electron microscopy examination was the presence, in 1 patient, of scattered granular electron-dense deposits along some tubular basement membranes. First-line treatment included steroids, supplemented in some cases by additional immunosuppressive agents. Three patients experienced a partial or complete response, while progressive renal damage was observed in a case with severe chronic lesions and persistence of inflammation-triggering factor. Our cases seem to represent progressive stages within the continuum of disease evolution. Patients with more prominent inflammation might represent a more initial state, while those with a more severe chronicity index, probably depict more advanced stages. While the predominance of T-cells predicates a cell-mediated autoimmune mechanism, as the driving force of the disease occurrence, the presence of immune complexes in more advanced stages might indicate the involvement of humoral immunity as a late event during the disease course.

Clinical manifestations and outcomes in tubulointerstitial nephritis and uveitis syndrome: a case report and a systematic review in China.

PURPOSE: Tubulointerstitial nephritis and uveitis (TINU) syndrome is an uncommon disease. We present a confirmed case of TINU syndrome, and a systematic review of epidemiological characteristics, clinical manifestations, management, and outcomes in Chinese patients. METHODS: A systematic search was carried out using defined terms and updated up to September 2022, in PubMed, Web of Science, Wanfang, CNKI, and VIP, to identify reported cases of TINU in China, according to PRISMA guidelines. RESULTS: An 18-year-old boy presented with elevated serum creatinine and 24-h urine protein level of > 2 g. Inspection result revealed acute tubulointerstitial nephritis, and bilateral uveitis. The patient was diagnosed with TINU syndrome and received treatment with methylprednisolone sodium succinate, which resulted in a significant decrease in creatinine and urinary protein levels. Systematic review identified 35 publications that met the inclusion criteria. A total of 71 cases were included in this article, of which 70 were from publications and 1 was from our hospital. The median age at onset was 42 years and was significantly lower in males than females (P < 0.05). The symptoms of uveitis often occurred after kidney injury (54%) and most uveitis was anterior (55%) and bilateral (75%). Among the 51 patients who were followed up for more than 6 months, 24 had recurrent ocular symptoms or progression to chronic uveitis. Twenty patients experienced chronic or progressive kidney disease. CONCLUSION: TINU syndrome is prone to misdiagnosis because kidney damage may not occur simultaneously with uveitis. The incidence of kidney sequelae in children is lower than that in adults, and glucocorticoids are the preferred treatment. INPLASY REGISTRATION NUMBER: INPLASY202350050.

Berlin nodules: About 3 cases.

INTRODUCTION: Granulomatous uveitis is mainly defined by the nature of keratic precipitates and iris nodules. Anterior chamber (AC) granulomas, also known as Berlin nodules (BN), are circumscribed granulomatous nodules that form in the iridocorneal angle (ICA) in response to intraocular inflammation. CASES DESCRIPTION: We present three representative cases of anterior uveitis with berlin nodules to insist on the clinical features of this entity. Case 1: A female adult was referred to our department for bilateral redness and gradual blurry vision. Slit-lamp examination of both eyes (OU) found a yellow-white nodular mass at 6 o'clock on the peripheral iris with irido-crystalline synechiae, vitreous haze and blurred fundus. Purified protein derivative skin test revealed an induration of 20 mm. A diagnosis of presumed ocular tuberculosis was made. The patient received antituberculosis drugs and steroids with good evolution. Case 2: An 11-year-old girl presented for redness and pain OU. Anterior segment examination showed circumciliary congestion, epithelial microcystic edema OU and pearly vascularized lesion against the cornea in the right eye. Fundus examination was unremarkable. A diagnosis of definite ocular sarcoidosis was made based on accessory salivary glands biopsy. The patient was treated with teroids and controlled twice a week. Case 3: A 26-year-old male presented with a redness and pain of the left eye. The slit-lamp examination showed fine keratic precipitates with pearly white nodules within the AC. Fundus examination showed a chorioretinal lesion and occlusive vasculitis. Chest CT was consistent with milliary tuberculosis. Adequate therapy was initiated with favorable outcome. CONCLUSION: BN can be associated with various ocular manifestations of several diseases. They may be the first manifestation of systemic conditions and seem to be well managed with topical steroids.