Ocular Immunology and Inflammation最新文献

Purpose: To investigate corneal and pupillary characteristics in herpetic anterior uveitis (HAU) caused by herpes simplex virus (HSV), varicella-zoster virus (VZV) and cytomegalovirus (CMV) using corneal densitometry, specular microscopy, and automated pupillometry.

Methods: In this prospective study, 56 patients (112 eyes) diagnosed with HAU were categorized into CMV-associated anterior uveitis (CMV-AU, n = 21) and non-CMV HAU (HSV or VZV, n = 35) groups. Corneal transparency was assessed by Scheimpflug-based densitometry. Endothelial cell morphology was evaluated with specular microscopy. Static and dynamic pupillometry measured pupillary responses under various lighting conditions. Clinical features and ocular findings were compared between groups and with contralateral healthy eyes.

Results: Non-CMV HAU patients demonstrated significantly increased corneal densitometry values indicating stromal haze (p < 0.05) and pupillary constriction deficits compared to fellow eyes (p < 0.05). In contrast, CMV-AU eyes showed significant endothelial cell loss and increased cell size variability (p < 0.05), with relatively preserved corneal transparency. Pupillometry revealed prolonged constriction duration and shortened dilation duration in CMV-AU (p < 0.05). Iris atrophy and posterior synechiae were more frequent in non-CMV HAU. No significant pupillometric differences were observed between CMV and non-CMV groups.

Conclusions: CMV-AU predominantly affects corneal endothelium with endothelial cell loss and altered pupillary dynamics, whereas HSV/VZV-associated HAU leads to stromal corneal haze and sphincter dysfunction. Quantitative imaging and functional assessments provide complementary, noninvasive parameters that may support clinical differentiation among HAU subtypes.

Purpose: To describe the spectrum of ocular inflammation in a series of patients with systemic sclerosis.

Methods: Retrospective case series of six patients diagnosed with systemic sclerosis presenting with either uveitis or retinal vasculitis.

Results: Presenting symptoms included blurry vision, flashes, floaters, photophobia, and eye pain. Visual acuity at presentation ranged from 20/20 to 20/125. Retinal vascular involvement was present in all patients. One patient was symptomatic in only one eye but was found to have bilateral retinal vasculitis. Two patients had findings consistent with retinal artery occlusion. One patient presented with posterior uveitis, one with panuveitis, and two initially presented with anterior uveitis and later developed retinal vasculitis. All six patients required systemic corticosteroid therapy.

Conclusion: Systemic sclerosis in adults rarely causes ocular inflammation; when present, it is most often associated with retinal vasculitis. This is the first case series to report active retinal vasculitis in association with systemic sclerosis. Patients may be asymptomatic despite significant vascular leakage. Retinal vasculitis should be considered in patients with systemic sclerosis who develop ocular complaints.

Purpose: Only a few studies have analyzed the diagnostic utility and prognostic significance of ocular sarcoidosis (OS) in patients with systemic sarcoidosis. Our aim was to analyze the prevalence of OS in sarcoidosis, its possible association with other forms of extrathoracic sarcoidosis, and its prognostic significance.

Methods: Retrospective study including patients diagnosed with sarcoidosis at Bellvitge University Hospital, Barcelona, Spain, between 1980 and 2017. Patients were prospectively followed up at the hospital's sarcoidosis clinic. Clinical data were obtained by reviewing patients' medical records.

Results: Sarcoidosis was diagnosed in 728 patients (494 women and 234 men, mean age 43.45 years, SD 13.833). Radiological stages were: stage 0 in 68 patients (9.34%), 1 in 434 (59.62%), 2 in 167 (22.94%), 3 in 45 (6.18%), and 4 in 14 (1.92%). OS was diagnosed in 98/728 patients (13.5%) and was present at the onset of sarcoidosis in 83 cases (85%). Among patients with OS, 42/98 (42.9%) had persistent systemic sarcoidosis activity for more than 5 years (p < 0.001), and 21/98 (21.4%) had central nervous system sarcoidosis (p < 0.001). Patients with OS also showed a higher frequency of otorhinolaryngological involvement (12.2% vs. 2.2%, p < 0.001) and specific skin lesions (30.61% vs. 20.8%, p = 0.036), while erythema nodosum and arthralgia were less frequent (p < 0.001 and p = 0.001, respectively).

Conclusion: OS was observed in 13.5% of sarcoidosis patients, usually at the onset of the disease. It was associated with systemic disease activity persisting for more than 5 years and with central nervous system sarcoidosis.

Background: Anti-synthetase syndrome (aSS) is a rare autoimmune disorder primarily affecting the lungs and musculoskeletal system, with ocular involvement often underreported. While dry eye disease is the most frequently documented manifestation, other anterior and posterior segment involvement can also occur.

Methods: A case report and a systematic review.

Case presentation: We report a 47-year-old woman with aSS presenting with bilateral panuveitis and occlusive retinal vasculitis, a previously unreported combination. Ophthalmologic examination revealed compromised vision, anterior chamber reaction, posterior synechiae, papillitis, uveitic macular edema, and neovascularization of the disc (NVD). Extensive infectious and autoimmune workups guided the diagnosis, emphasizing the role of ocular findings in identifying aSS. The patient required multidisciplinary assessment and multifaceted treatment approach, including systemic corticosteroids, mycophenolate mofetil, intravenous immunoglobulin, and posterior sub-Tenon's triamcinolone injections, with consideration of anti-VEGF therapy for NVD.

Results: Our systemic review of 19 studies (86 patients) identified dry eye disease as the most common ocular manifestation (36%), with retinal vasculitis reported in only two cases (2.3%). Other reported manifestations included blurry vision, diplopia, ptosis, blepharitis, lagophthalmos, periorbital edema, and corneal disease. Compared to prior reports, our case represents one of the most severe ophthalmic presentations of aSS, highlighting the need for early recognition and intervention to prevent vision threatening sequelae.

Conclusion: This case broadens the spectrum of ocular findings in aSS and highlights the importance of ophthalmologic evaluation in diagnosing systemic autoimmune diseases. Given the potential for vision-threatening complications, multidisciplinary collaboration is essential for holistic patient management.

Introduction: Mevalonate Kinase Deficiency (MKD), which includes the clinical phenotype known as Hyperim munoglobulin D Syndrome (HIDS), is an autosomal recessive autoinflammatory disorder caused by mutations in the MVK gene. While typically characterized by childhood-onset recurrent febrile episodes and systemic symptoms like lymphadenopathy and abdominal pain, ocular involvement is usually limited to conjunctivitis. Uveitis is a rare manifestation, often associated with more severe forms of the disease.

Case presentation: This report describes a 28-year-old male who presented with bilateral, recurrent anterior uveitis and vitritis. Although the patient reported self-limiting febrile episodes during childhood, the presentation of ocular inflammation in adulthood as the primary symptom posed a significant diagnostic challenge. Initial treatment with topical and systemic corticosteroids provided only temporary relief, with the patient developing cystoid macular edema and further recurrences. A multidisciplinary evaluation and genetic testing confirmed the diagnosis of HIDS. Following the diagnosis, the patient was started on targeted anti-IL-1 therapy (anakinra or canakinumab). This intervention led to a significant improvement in symptoms and was essential in halting recurrences and preventing chronic ocular damage.

Conclusion: Recurrent uveitis, even in adult patients, should prompt the investigation of rare autoinflammatory syndromes. A timely genetic diagnosis and a multidisciplinary approach are crucial for initiating targeted biological treatments, which offer a superior visual and systemic prognosis compared to conventional therapies.

Purpose: To evaluate central retinal sensitivity in patients with initial-onset acute uveitis associated with Vogt-Koyanagi-Harada (VKH) disease treated within (early presentation) or outside (late presentation) the therapeutic window of opportunity after discontinuation of immunosuppressive therapy without relapse of inflammation.

Methods: In this retrospective study, 19 patients (38 eyes) presented in the phase preceding anterior segment (AS) inflammation (early presentation) and 17 patients (33 eyes) had AS inflammation at presentation (late presentation). MP-1 microperimetric evaluation of retinal sensitivity in the central 12 degrees was assessed at last follow-up after treatment.

Results: The median follow-up period, duration of treatment, and interval between discontinuation of treatment and last follow-up were 20, 16.5, and 6 months, respectively. None of the eyes in the early presentation group developed "sunset glow fundus" (SGF), whereas in the late presentation group, 81.8% of the eyes developed SGF (p < 0.001). Mean retinal sensitivity was significantly worse in the late presentation group and in the eyes that developed SGF (p < 0.001 for both comparisons).

Conclusions: Prompt institution of effective immunosuppressive therapy within the therapeutic window of opportunity preserves photoreceptor function.

Anterior uveitis, in which the anterior chamber is the primary site of inflammation, is the most frequently encountered form of uveitis. However, several non-neoplastic entities can closely mimic its clinical presentation, posing significant diagnostic challenges. This narrative review focuses on these non-malignant conditions that simulate anterior uveitis and may mislead clinicians in routine practice. Differentiating true inflammatory cells from pigment granules in the anterior chamber can be difficult, often leading to misdiagnosis. Disorders such as Bilateral Acute Iris Depigmentation (BADI) and Bilateral Acute Iris Transillumination (BAIT) may present with similar findings and must be evaluated carefully. Toxic Anterior Segment Syndrome (TASS) represents another important mimic, manifesting as acute sterile inflammation with corneal edema. Additionally, Retinitis Pigmentosa and old retinal detachment may present with anterior uveitis-like features, further complicating diagnosis. Like malignant masquerades, these non-neoplastic entities demand a high index of suspicion. A meticulous ocular examination, aided by ancillary investigations, when necessary, is vital for accurate differentiation. Recognizing these mimickers is essential to avoid inappropriate anti-inflammatory therapy and ensure appropriate management.

Purpose: To report two cases of vasoproliferative-like retinal tumor (VLRT) associated with Behçet's syndrome (BS), highlighting their contrasting clinical courses and management strategies, supported by multimodal imaging.

Methods: This is a retrospective description of two patients with BS who developed VLRTs. Case 1 involved a 40-year-old woman with a 33-year history of BS, followed for 18 months after VLRT detection. Case 2 involved a 16-year-old boy with a 5-year history of BS, followed for 4 months after incidental VLRT detection. Multimodal imaging included widefield fundus photography, fluorescein angiography, and optical coherence tomography.

Results: Case 1 developed an exudative VLRT that responded transiently to three monthly intravitreal bevacizumab injections with adjuvant photocoagulation, but recurred within one month, requiring systemic immunosuppression, a switch to aflibercept, and planned cryotherapy. In contrast, Case 2's lesion remained stable and asymptomatic under adalimumab therapy, with cryotherapy planned due to the lesion's characteristics.

Conclusion: VLRTs can occur in BS irrespective of prior inflammatory control and may follow variable clinical courses. These cases highlight the importance of widefield imaging surveillance in BS and suggest that optimal management may require individualized combinations of anti-VEGF therapy, ablative procedures, and systemic immunosuppression.

Background: Multiple studies have associated PTGER4 polymorphisms with susceptibility to several autoimmune diseases, particularly ankylosing spondylitis (AS)-a condition strongly linked to the onset of acute anterior uveitis (AAU). Given this association, we investigated whether PTGER4 variants are associated with AAU susceptibility in Chinese populations.

Methods: To identify PTGER4 disease susceptibility loci, we recruited 904 healthy subjects (AS-AAU-) and 402 AAU patients (AAU+AS-). Genotyping used the MassArray system (iPLEX Gold). SNP allele and genotype frequency differences between groups were assessed with chi-square tests. To account for multiple comparisons, we applied the Bonferroni correction. Additionally, haplotype analysis and stratified analysis were conducted to further explore potential genetic relationships.

Results: The two PTGER4 single nucleotide polymorphisms (SNPs) loci (rs10440635, rs4133101) exhibited no significant correlation with susceptibility to AAU. No significant differences in haplotype frequencies were observed between AAU groups and controls following stratified analysis.

Conclusion: Our findings demonstrate that the two loci, rs10440635 and rs4133101 in PTGER4, were not found to be associated with susceptibility to AAU in this population. Specifically, for rs10440635, the odds ratio (OR) was 0.924 (95% CI: 0.757-1.128, p = 0.438), and for rs4133101, the OR was 0.918 (95% CI: 0.777-1.085, p = 0.314). Even after stratifying the analysis by HLA-B27 status, no statistically significant association was observed. To further elucidate the potential role of PTGER4 in these conditions, additional studies with larger sample sizes, broader genetic variants, and more diverse ethnic populations are warranted.contribution of PTGER4 to these diseases.

Purpose: To quantify anterior-segment remodelling and pupillary behaviour in unilateral Fuchs uveitis syndrome (FUS) and to evaluate the diagnostic utility of anterior-segment metrics.

Methods: Single-centre, retrospective, paired-eye study of 42 patients with unilateral FUS was conducted. Each patient's FUS eye was compared with the contralateral clinically healthy eye. Anterior-segment parameters (K1, K2, Kmax, central corneal thickness, anterior chamber depth (ACD), anterior chamber volume, iridocorneal angle (ICA)) were obtained with a Scheimpflug system. Static pupillometry recorded pupil diameter under scotopic (0.04 lx), mesopic (4 lx), and photopic (40 lx) conditions. The discriminative performance of parameters was assessed by receiver-operating characteristic (ROC) analysis.

Results: FUS eyes showed a wider ICA and deeper ACD (p < 0.001, for each). K2 and Kmax were steeper in FUS (both p < 0.001). On pupillometry, the photopic pupil was larger in FUS (p = 0.001), whereas scotopic and mesopic diameters were comparable (p = 0.78 and 0.64, respectively). The mesopic to photopic constriction amplitude was reduced in FUS (p = 0.002); the scotopic to photopic amplitude was borderline (p = 0.061), and scotopic to mesopic was not different (p = 0.739) between eyes. In ROC analysis, ACD discriminated FUS from fellow eyes with an AUC of 0.747 (95% CI, 0.638-0.846); the cutoff was 3.05 mm, with a sensitivity of 0.67 and a specificity of 0.79.

Conclusions: Unilateral FUS exhibits a coherent structural-functional signature: wider angles and deeper ACD, axis-selective anterior steepening, and attenuated photopic constriction with a larger photopic pupil. Combining Scheimpflug anterior-segment metrics with standardized pupillometry provides practical, adjunctive markers that may strengthen diagnostic confidence and sharpen hypotheses on FUS pathophysiology.