Optical Coherence Tomography: Focus on the Pathology of Macula in Scleritis Patients.

Optical coherence tomography (OCT) is a non-invasive imaging technique for high-resolution, cross-sectional tissue imaging of the eye. During the past two and a half decades, OCT has become an essential tool in ophthalmology. It is a painless method for examining details of ocular structures in vivo with high resolution that has revolutionized patient care following and treating scleritis patients. METHODS: Twenty-four patients diagnosed with scleritis were selected for this study. All of the patients went through basic ophthalmological examinations, such as visual acuity testing (VA), intraocular pressure measurement (IOP), slit lamp examination, ophthalmoscopic examination, and OCT. OCT examinations were taken by SD-OCT Spectralis OCT system (Heidelberg Engineering, Heidelberg, Germany). RESULTS: Twenty-seven eyes of 24 patients (7 males and 17 females) were included in this study, who were diagnosed with scleritis. OCT examinations showed epiretinal membrane (ERM) in three patients (12%), cystoid macular edema (CME) (three cases, 12%), diffuse macular edema (DME) (one case, 4%), and serous retinal detachment (SRD) (one case, 4%). CONCLUSIONS: OCT proved to be a valuable, non-invasive method for detecting macular pathology in patients with scleritis. Despite the best treatment regimen applied, macular involvement resulting in reduced visual acuity (VA) can develop, which we could detect with OCT since macular edema (ME) is the leading cause of decreased vision due to the damaged outer blood-retina barrier (BRB) in inflammation. OCT investigation is a highly important method for early detection of ocular complications in scleritis in order to prevent blindness.

Biological therapy of uveitis in children / A biológiai terápia helye a gyermekkori uveitis ellátásában

Introduction: Biological therapy can be used in uveitis in children since 2016. With ophthalmological indication only adalimumab therapy can be started. Adalimumab is a monoclonal antibody that inhibits tumor necrosis factor alpha.Objective: To summarize our experience with patients receiving adalimumab for pediatric non-infectious uveitis.Patients and methods: We investigated our juvenile patients of non-infectious uveitis treated with adalimumab be-tween 2017 and 2021 in a retrospective case series at the Department of Ophthalmology, Szeged University. Results: Between 01 January, 2017 and 31 May, 2021, we examined 46 children with uveitis. The mean age of these 23 girls and 23 boys was 11 years. 21 of them had juvenile idiopathic arthritis, 14 had infectious uveitis, 3 had hae-matological disorders, 8 had idiopathic uveitis. Adalimumab was given to 11 patients because of severe, chronic uveitis. There were 3 boys and 8 girls, their mean age was 10 years. Adalimumab was given according to the licence of the European Medicines Agency. Indication was anterior uveitis at 6 children, panuveitis at 5 children. Adali-mumab can be given to children over 2 years, who have chronic, non-infectious, anterior uveitis. Children with panuveitis received the therapy by the help of a pediatric rheumatologist.Conclusion: The significance of pediatric uveitis and its therapy is emergent. Our aim was to preserve vision and de-crease the possibilities of side effects and to provide a better life for these uveitic children. Early diagnosis, adequate therapy and regular ophthalmological check-ups are important. Children treated with adalimumab have good visual acuity due to the effectiveness of the therapy. No new ocular side effect was detected at the children treated with adalimumab.

White Dot Syndrome Report in a SARS-CoV-2 Patient.

Our purpose was to report clinical features in bilateral white dot syndrome in a 47-year-old female patient who was tested positive for the SARS-CoV-2. A 47-year-old female visited our department with complaints of bilateral photophobia and blurred vision in both her eyes. She visited our department during the pandemic period after her PCR-proven SARS-CoV-2 positivity. Her symptoms were chills and fever with a temperature of 40.0°C, associated with fatigue, sweat, and complete loss of taste. Besides basic ophthalmological examinations, ocular diagnostic testing were made to differentiate between specific white dot syndromes with suggestive features of fluorescein angiography, optical coherence tomography, and fundus autofluorescence. Laboratory tests were ordered, including immunserological and haematological ones. Eye examination revealed mild bilateral vitritis and white dots in the fundus of both eyes, including the macula explaining the blurred vision. Herpes simplex virus reactivation was proved, after the SARS-CoV-2 infection. Local corticosteroids were given according to the European Reference Network's recommendations for patients with uveitis during the COVID-19 pandemic. Our report demonstrates that white dot syndrome with blurred vision could be associated with SARS-CoV-2 infection, being potentially sight-threatening because of macular involvement. Ophthalmological examinations found posterior uveitis white dot syndrome, and this should call attention to the risk of acute 2019-CoV infection or occurred 2019-CoV infection. Immunodeficiency favours the occurrence of other viral infections, such as herpes virus infections. Everybody should be aware of the risk of 2019-CoV infection, especially professionals, social workers, and those who work or live with elder people and people with immunodeficiency.

[Ophthalmologic manifestations of Bardet-Biedl syndrome]. / Tapasztalataink Bardet-Biedl-szindrómás gyermekek általános szemészeti és elektrofiziológiai vizsgálatával.

INTRODUCTION: Bardet-Biedl syndrome is characterised by retinal dystrophy, polydactily, obesity and slow mental development. AIM: The aim of the authors was to present ophthalmologic signs and symptoms of the syndrome. METHOD: Between 1980 and 2010, 4 children with Bardet-Biedl syndrome were evaluated at the Department of Ophthalmology, University of Szeged, Szeged, Hungary. Their age at the first visit was between 1 and 10 years. Basic ophthalmological and electrophysiological evaluation, as well as orthoptic examinations were performed. RESULTS: In two cases the electroretinographic curves were subnormal, and in two cases the electroretinographic curves showed no elevation. In the 4 children abnormal electroretinographic curves appeared at the ages of 1, 5, 10, and 18 years. Pigmentary changes on the periphery of the retina were detected in two cases. CONCLUSIONS: The different signs and symptoms of Bardet-Biedl syndrome may manifest at different ages. Electrophysiological changes failed to correlate with retinal alterations is these patients.

Neuroimmune interactions in Sjögren's syndrome: relationship of exocrine gland dysfunction with autoantibodies to muscarinic acetylcholine receptor-3 and mental health status parameters.

OBJECTIVES: Antimuscarinic acetylcholine receptor-3 (m3AChR) autoantibodies have been described in primary Sjögren's syndrome (pSS). The aim of this study was to compare various methods for their detection and to assess the contributions of anti-m3AChR and other immunological and psychosocial factors to the pathomechanism of secondary SS (sSS). METHODS: Sixty-five rheumatoid arthritis (RA) patients, 103 systemic lupus erythematosus (SLE) patients, 76 pSS patients and 50 controls were compared. Three immunodominant epitopes of m3AChR were synthesized and used in ELISA. Two extracellular epitopes were also prepared in fusion with glutathione-S-transferase and one in conjugation with bovine serum albumin. Mental health status was assessed with the 36-item Short-Form Health Survey and Functional Assessment of Chronic Illness Therapy fatigue scale. Correlations were evaluated between glandular function and anti-m3AChR positivities and specificities, features of SLE and RA, and mental health parameters. RESULTS: Fourteen RA and 27 SLE patients had sSS. The autoantibody levels to all epitopes of m3AChR were significantly higher in pSS and SLE patients than in the controls. The fusion protein forms discriminated RA from pSS and SLE; furthermore, the YNIP fusion protein also distinguished pSS from SLE. The prevalence and the mean levels of all autoantibodies did not differ statistically between sicca and non-sicca SLE or RA patients. Glandular dysfunction correlated with higher age in SLE and RA and an impaired health-related quality of life in SLE. CONCLUSIONS: The second and third extracellular loops of m3AChR are antigenic in pSS. Immunoassays with antigens as fusion peptides demonstrate the best performance. Sicca SLE patients have worse mental health status. Anti-m3AChR antibodies represent a peculiar example of neuroimmune interactions.

Role of ultrasound biomicroscopy in diagnosis and treatment of Terrien disease.

PURPOSE: Terrien disease is a rare form of peripheral corneal degeneration characterized by vascularization, opacification, lipid deposition, and corneal thinning. In this study, a high-frequency ultrasound biomicroscope (UBM) was used to detect the morphologic changes before and after surgery and to determine the stages of this disease. METHODS: Two patients with Terrien disease were examined by UBM, corneal topography, and a keratometer before and after surgery (full-thickness keratectomy). RESULTS: The absence of the Bowman layer and thinning of the Descemet layer in the ectatic part of the peripheral cornea were detected by using the UBM before surgery. Earlier, these signs could be detected only with optical and electron microscopy from histologic samples; now we can detect the signs of Terrien disease with noninvasive devices such as the UBM. CONCLUSIONS: The UBM is an effective device for following the progression of Terrien disease and determining the timing of these patients' surgeries.

Significance and prognostic value of lysosomal enzyme activities measured in surgically operated adenocarcinomas of the gastroesophageal junction and squamous cell carcinomas of the lower third of esophagus.

AIM: To establish whether there are fundamental differences in the biochemistries of adenocarcinomas of the gastroesophageal junction (GEJ) and the squamous cell carcinomas of the lower third of the esophagus (LTE). METHODS: Between February 1, 1997 and February 1, 2000, we obtained tissue samples at the moment of resection from 54 patients for biochemical analysis. The full set of data could be comprehensively analyzed in 47 of 54 patients samples (81%). Of these, 29 were adenocarcinomas of the GEJ Siewert type I (n = 8), type II (n = 12), type III (n = 9), and 18 presented as squamous cell carcinomas of the LTE. We evaluated the mean values of 11-lysosomal enzyme and 1-cytosol protease activities of the tumorous and surrounding mucosae as well as their relative activities, measured as the ratio of activity in tumor and normal tissues from the same patient. These data were further analyzed to establish the correlation with tumor localization, TNM stage (lymph-node involvement), histological type (papillary, signet-ring cell, tubular), state of differentiation (good, moderate, poor), and survival (or=24 mo). RESULTS: In adenocarcinomas, the activity of alpha-mannosidase (AMAN), cathepsin B (CB) and dipeptidyl-peptidase I (DPP I) increased significantly as compared to the normal gastric mucosa. In squamous cell carcinomas of the esophagus, we also found a significant difference in the activity of cathepsin L and tripeptidyl-peptidase I in addition to these three. There was a statistical correlation of AMAN, CB, and DPP I activity between the level of differentiation of adenocarcinomas of the GEJ and lymph node involvement, because tumors with no lymph node metastases histologically confirmed as well-differentiated, showed a significantly lower activity. The differences in CB and DPP I activity correlated well with the differences in survival rates, since the CB and DPP I values of those who died within 24 mo following surgical intervention were significantly higher than of those who survived for 2 years or more. CONCLUSION: Adenocarcinomas of the GEJ form a homogenous group from a tumor-biochemical aspect, and differ from the biochemical characteristics of squamous cell carcinomas of the LTE on many points. When adenocarcinomas of the GEJs are examined at the preoperative phase, the ratio of the performed AMAN, CB, and DPP I enzymatic activity of the tissue sample from the tumor and adjacent intact mucosa within 2 cm of the tumor may have a prognostic value even in the preoperative examination period, and may indicate that ranking of these patients into the neo-adjuvant treatment group should be considered.

Lysosomal enzyme activities: new potential markers for Sjögren's syndrome.

OBJECTIVE: To evaluate the changes in lysosomal enzyme activities in leukocytes of patients with Sjögren's syndrome. METHODS: Leukocytes were obtained from 38 patients with Sjögren's syndrome and 36 healthy subjects. The activities of the following glycosidases were measured: alpha-glucosidase (AGU), beta-galactosidase (BGA), alpha-mannosidase (AMAN), beta-glucuronidase (GCU), beta-hexosaminidase (HEX), and the following proteases: cathepsin B (CATH B), dipeptidyl peptidase I (DPP I), cathepsin H (CATH H), dipeptidyl peptidase II (DPP II), tripeptidyl peptidase I (TPP I), and cathepsin D (CATH D) activity. RESULTS: Activity of the glycosidases beta-galactosidase, alpha-mannosidase, beta-glucuronidase and beta-hexosaminidase, as well as of the peptidases cathepsin B, cathepsin D, dipeptidyl peptidase I, and tripeptidyl peptidase I, was elevated during the first 5 years of SS, and it increased further between 5 and 10 years after diagnosis. CONCLUSIONS: The elevated activities of the lysosomal enzymes in Sjögren's syndrome patients may play a role in tissue damage by accelerated breakdown of glycoproteins in lysosomes.

Lysosomal peptidases and glycosidases in rheumatoid arthritis.

Lysosomal serine and cysteine proteases are reported to play a role in collagen degradation. In this study, the activities of the lysosomal cysteine proteases cathepsin B and H, dipeptidyl peptidase I, and the serine protease tripeptidyl peptidase I and dipeptidyl peptidase II, all ascribed a role in collagen digestion, were compared with those of the aspartate protease cathepsin D, and lysosomal glycosidases in leukocytes from rheumatoid arthritis patients at different stages of the disease. In all patients the activities of cysteine protease cathepsin B, dipeptidyl peptidase I, aspartate protease cathepsin D, and two glycosidases were elevated, but the activities of the serine proteases tripeptidyl peptidase I, dipeptidyl peptidase II, and the cysteine protease cathepsin H was unchanged. The magnitude of the increased activity was correlated with the duration of the disease. Patients with long-standing RA (10 years or more) had higher cysteine protease activity in their leukocytes than did those with disease of shorter duration. This tendency suggests that elevated lysosomal cysteine protease activities, together with aspartate protease cathepsin D and lysosomal glycosidases (but not serine proteases), are associated with progression of rheumatoid arthritis.