Prevalence of Sjögren's syndrome in patients with dry mouth in the region of Central Hungary.

OBJECTIVE: One-third of the Hungarian population suffers from xerostomia. Since there is no evidence of the actual prevalence of Sjögren's syndrome (SS) in Hungary, this study aimed to evaluate the same. MATERIALS AND METHODS: Data were collected from the Faculty of Dentistry, Semmelweis University from 2008 to 2015. A diagnosis of SS was established based on the American College of Rheumatology and European League Against Rheumatism criteria. RESULTS: Of the 1076 patients examined with sicca symptoms, 188 patients had confirmed SS. Primary SS (pSS) was diagnosed in 135 patients and secondary SS (sSS) was confirmed in 53 patients. According to the available statistical records of the public health service of Hungary, there were an average of 16 (0.0014%, 5-26) newly diagnosed SS cases in the entire population and 141 SS patient-practitioner consultations (49-232) per 100,000 inhabitants in the country over the past 10 years (based on the past 10 years: 2011-2020). CONCLUSION: Results revealed that approximately 1/5th-1/6th of patients with sicca symptoms have SS, among whom 72% and 285 have pSS and sSS, respectively. Global Hungarian records simultaneously revealed that the number of both new diagnoses and doctor-SS patient encounters has significantly decreased (by 50%) yearly over the last decade.

Ocular paraneoplastic pemphigoid / Szemészeti érintettséggel járó paraneoplasiás pemphigoid

Paraneoplastic mucous membrane pemphigoid, a rare pemphigoid variant is associated with primary malignancy, and characterised by fulminant progression and frequent ineffectivity of classical systemic immunosuppression. In this paper, the clinical features, diagnostic and therapeutical challenges are presented through three cases. Detailed history and analysis of the immunofluorescent samples help the diagnosis. The therapeutic goal is to prevent the progression with systemic immunosuppressive treatment, which can be contraindicated during the ongoing oncological therapy. In absence of consent in the exact diagnostic criteria and management protocol of this rare condition, consultation with other specialists (ophthalmologist, dermatologist, dentist, ear-nose-throat specialist, immunologist) has high importance in early diagnosis and treatment.

Graft survival using cadaver and multiorgan donors between 2008 and 2017 in our department / Cadaverbol és multiorgan donorból származó szaruhártyák túlélése 2008 és 2017 között klinikánkon.

Összefoglaló. Bevezetés: Az elso szaruhártya-bank 1944-es alapítása óta jelentos változásokon ment át. A szaruhártya túlélését számos tényezo befolyásolja, így a tárolási mód, melynek a szövet lejárati ideje szerint rövid, közép és hosszú távú módszereit fejlesztették ki. Célkituzés: Retrospektív vizsgálatunk célja a 2008. január 1. és 2017. december 31. között perforáló és lamelláris keratoplasztika során felhasznált cadaverbol és multiorgan donorból származó szaruhártyák túlélésének vizsgálata volt a Semmelweis Egyetem Szemészeti Klinikáján. Módszer: Feljegyeztük a recipiens nemét, életkorát, a mutétet indikáló klinikai diagnózist, a mutét idopontját, a szövettani vizsgálat eredményét, valamint, hogy a beültetett szaruhártya cadaverbol vagy multiorgan donorból származott. Meghatároztuk, hogy a recipiens életkora korrelált-e a rekeratoplasztikáig eltelt idovel. Eredmények: 1451 szaruhártya-átültetés történt 1088 beteg (44,6% férfi) 1159 szemén (életkor 62,8 ± 18,5 év), melyek között 938 (64,6%) cadaver és 262 (18,0%) multiorgan donor került felhasználásra, 251 esetben (17,2%) nem állt rendelkezésre adat. A leggyakoribb primer diagnózis a szaruhártya-dekompenzáció volt (325 eset, 28%). A primer keratoplasztikák során felhasznált szaruhártyák 740 esetben (63,8%) cadaverbol, 212 esetben (18,2%) multiorgan donorból származtak, 207 esetben (17,8%) nem állt rendelkezésre adat. Elso rekeratoplasztika a primer keratoplasztikák közül 217 esetben (18,7%) történt. A leggyakoribb szövettani diagnózis az endothelsejt-degeneráció volt (130 esetben, 60,4%). 146 esetben (67,2%) korábban cadaver, 31 esetben (14,2%) multiorgan donor esetén került sor ismételt mutétre, 40 esetben (18,4%) nem állt rendelkezésre adat. Következtetés: Klinikánkon elsosorban cadaverbol származó donorok biztosítják a szaruhártya átültetésekhez szükséges szövetet. Cadaverbol vagy multiorgan donorból származó szaruhártyák esetén nem kerül gyakrabban sor rekeratoplasztikára. A szaruhártya-banki tevékenység további fejlesztésével növelheto a donorok túlélése hazánkban. Orv Hetil. 2021; 162(13): 488-496. INTRODUCTION: Corneal banking methods have been changing since the foundation of the first corneal bank in 1944. Corneal graft survival may be affected by several factors, among others the storage method, which may be short-, middle- and long-term storage. OBJECTIVE: To investigate corneal graft survival at the Department of Ophthalmology, Semmelweis University between 1 January 2008 and 31 December 2017, using cadaver and multiorgan donors for penetrating and lamellar keratoplasty, retrospectively. METHOD: Recipient sex, age, clinical diagnosis, date of surgery, histological examination results and origin of donors (cadaver or multiorgan donor) were recorded. Correlation between recipient age and time to repeat keratoplasty was also analyzed. RESULTS: There were 1451 keratoplasties in 1159 eyes (age 62.8 ± 18.5 years) of 1088 patients (44.6% male) using 938 (64.6%) cadaver and 262 (18.0%) multiorgan donors, data was not available in 251 (17.2%) cases. There was repeat keratoplasty in 217 patients (18.7% of first keratoplasties). The most common histological diagnosis was endothelial decompensation (130 cases, 60.4%) in these cases. In patients with a first repeat keratoplasty, in 146 cases (67.2%) the first donor originated from cadavers, in 31 cases (14.2%) from multiorgan donors and in 40 cases (18.4%) data were not available. CONCLUSION: Corneal donors mainly originate from cadavers at our Department. The necessity of repeat keratoplasties does not differ using cadaver or multiorgan donors. With further development of corneal banking, donor survival may be increased in Hungary. Orv Hetil. 2021; 162(13): 488-496.

Non-malignant conjunctival epithelial masses with ocular surface squamous neoplasia-like optical coherence tomography features.

PURPOSE: To observe and describe the anterior segment optical coherence tomography features of limbally localised non-malignant epithelial mass lesions METHODS: Thirteen patients (age: 66.9 ± 16.3 years) with conjunctival mass suggesting ocular surface squamous neoplasia with biomicroscopic examination were imaged using anterior segment ocular coherence tomography (anterior segment optical coherence tomography)/Cirrus HD-OCT, Model 4000, Carl Zeiss Meditec, Inc., Dublin, CA, and Spectralis HRA + OCT system, Heidelberg Engineering, Vista, CA/. Cases with ocular surface squamous neoplasia-like anterior segment optical coherence tomography (hyperreflective, thickened epithelium and an abrupt transition from normal to abnormal) were included in the study. Maximal thickness of the epithelium was measured. Histological diagnosis was gained from an excisional or incisional biopsy or impression cytology specimens. RESULTS: In six patients (age: 68.5 ± 15.4 years) with ocular surface squamous neoplasia-like anterior segment optical coherence tomography features, the histological diagnosis was other than ocular surface squamous neoplasia (papilloma, parakeratosis and a keratotic plaque with mild dysplasia), and ocular surface squamous neoplasia in seven cases (age: 65.6 ± 18.0 years). The maximal epithelial thickness was between 250 and 859 µm in non-ocular surface squamous neoplasia cases and between 252 and 596 µm in ocular surface squamous neoplasia cases. CONCLUSION: Non-malignant epithelial lesions can mimic ocular surface squamous neoplasia on anterior segment optical coherence tomography.

[Diagnostics and treatment of infectious keratitis]. / Fertozéses keratitisek diagnosztikája és kezelése.

We summarize up-to-date diagnostic and treatment of infectious keratitis using literature data and some clinical examples. In the clinical practice, most commonly bacterial, herpetic, mycotic and acanthamoeba keratitis occur. Beside slitlamp examination, for diagnostic purpose, we analyse corneal sensitivity, perform in vivo confocal microscopy, polymerase-chain-reaction (PCR), in vitro culture and histological examination of the corneal sample. As conservative treatment we use primarily topical moxifloxacin or cephasolin with fortified tobramycin or gentamycin in bacterial, topical antiviral gel (in some cases in combination with systemic antiviral treatment) in part in combination with topical corticosteroids in herpetic, voriconasole or amphotericin-B in mycotic, and topical-triple-therapy (diamidine, biguanid and antibiotics) in acanthamoeba keratitis. In case of early diagnosis and initiation of topical therapy, most cases of infectious keratitis recover successfully. However, beside conservative treatment, penetrating keratoplasty, amniotic membrane transplantation and crosslinking therapy may be necessary. Crosslinking is solely contraindicated in herpetic keratitis. Orv Hetil. 2017; 158(31): 1203-1212.

Specificity of in vivo confocal cornea microscopy in Acanthamoeba keratitis.

PURPOSE: To report on the presence of 4 different structures visualized by confocal microscopy in patients whose clinical presentation suggested infection by Acanthamoeba. METHODS: Data and charts of 28 consecutive patients were analyzed in a retrospective study. Four types of structures were recognized by confocal microscopy performed with HRT II Rostock Cornea Module: trophozoites, double-walled cysts, signet rings, and bright spots. The 28 patients (mean age 30.8 years, range 17-61 years, 10 male, 18 female) were divided into 4 groups according to the diagnosis established later by microscopic examination of smear, culture, response to therapy, and the course of keratitis. The 4 groups were Acanthamoeba keratitis (AK), Acanthamoeba suspect (AK-suspect), bacterial keratitis (BK), and fungal keratitis (FK). RESULTS: The rate of patients in AK, AK-suspect, FK, and BK groups where bright spots were found were 100%, 100%, 40%, and 55%, respectively. The sensitivity of presence of bright spots in the in vivo confocal microscopy in Acanthamoeba keratitis was 100% (95% confidence interval [CI] 73.5% to 100.00%) and specificity was 50% (CI 24.7% to 75.4%). When cases where the only signs of Acanthamoeba were bright spots were excluded, and only those cases were counted where any of cysts, trophozoites, or signet rings were also found, the sensitivity was 67% (95% CI 34. 9% to 90.1%) and the specificity was 94% (95% CI 69.8% to 99.8%). CONCLUSIONS: The relatively high rate of bright spots in non-Acanthamoeba keratitis challenges the assumption that bright spots seen by confocal microscopy are a specific indication of Acanthamoeba keratitis.

The role of complement activation in the pathogenesis of Fuchs' dystrophy.

PURPOSE: Inflammation can be an etiologic factor of Fuchs' dystrophy according to previous studies. Our aim was to analyse the activation of the complement system in the aqueous humor in this pathological condition. METHODS: 100 µl aqueous humor sample was taken during keratoplasty of 11 Fuchs' dystrophic patients and during phacoemulsification surgery of 18 control patients. The samples were mixed with EDTA and stored at -80 °C. Concentrations of C1rC1sC1Inh and C3bBbP complexes as markers of the activation of the classical and alternative complement pathways, respectively, were measured with ELISA method. The results of the patient group and the control group were compared with statistical analysis (non-parametric Mann Whitney test). RESULTS: Both the concentrations of C1rC1sC1Inh [4.3 (3.2-20.2)AU/ml] and of C3bBbP [15.3 (7.8-22.6)AU/ml] were significantly higher in the Fuchs' dystrophic group than in the control group [C1rC1sC1Inh: 0.0 (0.0-5.6)AU/ml, C3bBbP: 1.4 (0.0-7.8)AU/ml]. The median value is shown along with the (25% and 75% percentiles). CONCLUSIONS: Based on our results, the complement system may be activated both through the classical and alternative pathways in the aqueous humor of the patients with Fuchs' dystrophy.

[Postoperative therapy after penetrating keratoplasty in herpes simplex keratitis]. / Herpes simplex keratitisekben végzett perforáló keratoplasztika posztoperatív terápiája.

INTRODUCTION: Keratitis due to herpes simplex infection is a common cause of corneal damage resulting in impaired vision. AIM: The aim of this study was to assess the outcome of penetrating keratoplasties in patients treated with systemic antiviral and immunosuppressive drugs. METHOD: The authors retrospectively analysed data of 12 patients who underwent penetrating keratoplasty. The average age at onset of the first keratitis preceding surgery was 18 years (between 5 and 40 years). The indication for surgery in 9 cases was to improve vision and in 3 patient to prevent corneal perforation. Nine patients were given both acyclovir and mycophenolate mofetil, as anti-viral agent and immunosuppressive treatment, respectively. Two patients were treated with anti-viral agent only while one patient received no systemic therapy. The average follow-up time was 53.1 months (between 16 and 84 months). RESULTS: Of the 9 patients who underwent surgery for improving vision, 8 patients had transparent grafts during follow up without vascularization. All eight patients had been treated with acyclovir and mycophenolate mofetil. In one patient who had no systemic treatment recurrence and graft rejection was observed. Only one of the surgeries performed in acute stage of inflammation resulted in a properly healed transparent graft without recurrence and rejection. In this patient acyclivir and mycophenolate mofetil therapy had been given previously. In two cases the preventive - full or partial - systemic treatment had no effect. The visual acuity improved in all cases. In three patients visual acuity was influenced by some other factors as well. CONCLUSIONS: The systemic acyclovir and mycophenolat mofetil therapy is fairly successful in perforating keratoplasty due to herpes simplex infection. Acyclovir decreases the risk of recurrence, while mycophenolate mofetil may prevent graft rejection. The timing of surgery is decisive; it leads to better results when performed in a scarred, noninflammatory state.

Complement activation in the aqueous humor of pseudophakic bullous keratopathy patients.

BACKGROUND/AIMS: According to some studies, inflammation is a potential etiological factor in pseudophakic bullous keratopathy (PBK). Our aim was to obtain information on the activation of the complement system in the aqueous humor in this disorder. METHODS: Aqueous humor samples were collected during keratoplasty of 12 PBK patients, as well as during phacoemulsification surgery of 18 control patients. The concentrations of the protein-protein complexes generated during complement activation (C1rC1sC1inh and C3bBbP) through the classical and alternative pathways, respectively, as well as of the C3 cleavage product C3a, were measured with ELISA methods. The correlation among the complement factors and between the duration of the edema, the stage of the disease, and the level of the complement activation products was examined. RESULTS: The concentration of C1rC1sC1inh, C3bBbP complex and C3a was significantly higher in the PBK group (p < 0.001) compared to the control group. In PBK patients, a correlation was found between the levels of the C1rC1sC1inh complex and C3a only. CONCLUSION: Our new findings indicate that in PBK the complement system is activated - via the classical pathway - in the aqueous humor. The activated complement may play a role in increased endothelial cell loss.

Both freshly prepared and frozen-stored amniotic membrane cells express the complement inhibitor CD59.

Amniotic membrane proved to be very effective tool in the treatment of a number of ocular surface diseases. The amniotic membrane, however, has to be stored before its transplantation onto the ocular surface followed by mandatory serologic control in order to exclude the transmission of certain viruses. Therefore it is most important to study if cryopreservation of the membrane affects cell surface expression of the molecules. We measured cell surface expression of CD59, a membrane-bound complement inhibitor on the cells of freshly prepared and cryopreserved amniotic membrane. Cells of amniotic membrane were separated mechanically. Epithelial and mesenchymal cells were identified by the intracellular expression of nanog and the cell surface ICAM1 positivity, respectively. Multicolor flow cytometric immunophenotyping was used for determination of the CD59 expression. CellQuest-Pro software program (Becton Dickinson) was used both for measurements and analysis. CD59-positive cells could be detected in all investigated samples and in all investigated cell types, although the expression level of CD59 differed. CD59 was expressed both on freshly prepared and frozen-stored samples. Higher level of CD59 was detected on ICAM1+ mesenchymal cells than on nanog+ epithelial cells. Our findings indicate that amniotic membranes maintain their complement inhibiting capacity after cryopreservation.

C1r-C1s-C1inhibitor (C1rs-C1inh) complex measurements in tears of patients before and after penetrating keratoplasty.

PURPOSE: The aim of this pilot study was to determine the presence of complement activation products in tears from pre- and postkeratoplasty eyes and the fellow eyes in order to investigate the activation of the classical and alternative pathways of the complement system in the early postkeratoplasty period. METHODS: Tear samples from both eyes of 19 prekeratoplasty patients were tested. From 10 patients, samples were taken before operation, one week and 3 weeks after penetrating keratoplasty. Only baseline and 1 weak samples, and baseline and 3 week samples were available from 5 and 2 patients, respectively, while only baseline tear samples were collected from 2 patients. Tear concentration of two complement activation products, C1rs-C1inh and C3bBbP were determined by enzyme-linked immunosorbent assay. RESULTS: There was no difference (p = 0.339) between baseline samples of the eyes waiting for operation (0.93 +/- 0.51 AU/ml, mean +/- SEM) and the fellow eyes (0.33 +/- 0.33 AU/ml) in respect of mean C1rs-C1inh complex concentration. The one-week samples of the operated eyes revealed significantly (p = 0.006) elevated levels of C1rs-C1inh complexes (18.8. +/- 6.37 AU/ml), compared to their baseline samples (1.18 +/- 0.64 AU/ml), whereas the one-week values of the fellow eyes did not differ from the baseline values. Compared to the increased one-week values, the three-week values decreased to the baseline values in the operated eyes. C3bBbP could be detected in 3/68 tear samples. CONCLUSIONS: In our study we demonstrated the increased concentration of C1rs-C1inh complex in several tear samples taken early after human penetrating keratoplasty. These findings provide direct evidence that the classical pathway of complement may be activated in the early postoperative period after penetrating keratoplasty.