Person: DİRESKENELİ, RAFİ HANER
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DİRESKENELİ
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RAFİ HANER
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Publication Open Access A severe case of systemic lupus erythematosus with increased pressure communicating hydrocephalus(AVES, 2015-06-04) TUNCER, EMİNE NEŞE; Ozen, Gulsen; Yilmaz-Oner, Sibel; Tuncer, Nese; Akbas, Turkay; Tuglular, Serhan; Direskeneli, HanerNormal/increased pressure hydrocephaly is an unusual manifestation of systemic lupus erythematosus (SLE), and the pathogenesis is still unclear. We report the case of an 18-year-old white female with severe refractory renal and pulmonary involvement who developed stupor during intensive immunosuppressive treatment. Enlarged ventricles on imaging and increased intracranial pressure with the exclusion of infectious and hemorrhagic/thrombotic processes suggested increased pressure communicating hydrocephalus associated with SLE. Few case reports are reviewed, and potential pathophysiologic mechanisms are discussed.Publication Open Access C-reactive protein to albumin ratio is associated with disease activity in anti-neutrophil cytoplasmic antibody associated vasculitis(2023-01-01) ALİBAZ ÖNER, FATMA; DİRESKENELİ, RAFİ HANER; TUĞLULAR, ZÜBEYDE SERHAN; AŞICIOĞLU, EBRU; Atas D. B., Sahin G. K., ŞENGÜL Ş., KAYA B., PAYDAŞ S., ALİBAZ ÖNER F., DİRESKENELİ R. H., TUĞLULAR Z. S., AŞICIOĞLU E.Objective/Aim: C-reactive protein to albumin ratio (CAR) has recently been recognized as an independent prognostic marker for vasculitides. This study aims to investigate CAR and its relationship with disease activity and damage in prevalent ANCA associated vasculitis (AAV) patients. Methods: Fifty-one patients with AAV and 42 age-sex-matched healthy controls were enrolled in this crosssectional study. Birmingham vasculitis score (BVAS) was used to assess vasculitis activity and vasculitis damage index (VDI) to provide information on disease damage. Results: The median (25th-75th) age of the patients were 55 (48-61) years. CAR was significantly higher in AAV patients than controls (1.9±2.7 vs 0.7±0.4; p=0.006). The 75th percentile of BVAS was defined as high BVAS (BVAS≥5) and ROC curve analysis showed that CAR≥0.98 predicted BVAS≥5 with 70.0% sensitivity and 68.0% specificity (AUC:0.660, CI: 0.482-0.837, p=0.049). When patients with CAR≥0.98 were compared to those without, BVAS [5.0 (3.5-8.0) vs. 2.0 (0-3.25), p<0.001], BVAS≥5 [16 (64.0%) vs 4 (15.4%) patients, p:0.001], VDI [4.0 (2.0-4.0) vs. 2.0 (1.0-3.0), p=0.006], and CAR [1.32 (1.07-3.78) vs. 0.75 (0.60-0.83), p<0.001] were higher whereas albumin [3.8 (3.1-4.3) vs. 4.1 (3.9-4.4) g/dL, p=0.025] and haemoglobin [12.1 (10.4-13.4) vs. 13.0 (12.5-14.2) g/dL, p=0.008] were lower. Multivariate analysis revealed that BVAS [OR(95% CI):1.313 (1.003-1.719), p=0.047] was an independent factor associated with CAR≥0.98 in patients with AAV. Furthermore, correlation analysis showed that CAR significantly correlated with BVAS (r: 0.466, p=0.001). Conclusion: In this study, we observed that CAR was significantly associated with disease activity in AAV patients and can be used to monitor disease activityPublication Metadata only Health-related quality of life assessed by LupusQoL questionnaire and SF-36 in Turkish patients with systemic lupus erythematosus(SPRINGER LONDON LTD, 2016) DİRESKENELİ, RAFİ HANER; Yilmaz-Oner, Sibel; Oner, Can; Dogukan, Fatih Mert; Moses, Toklong Filam; Demir, Kubra; Tekayev, Nazar; Atagunduz, Pamir; Tuglular, Serhan; Direskeneli, HanerThe LupusQoL is a disease-specific health-related quality of life (HRQoL) measure for patients with lupus. We conducted this study to compare the efficiency of LupusQoL-TR (validated Turkish version of the LupusQoL questionnaire) with the 36-item Short-Form Health Survey (SF-36), a generic quality of life (QoL) scale, in Turkish patients with lupus. Both questionnaires were conducted at a single visit to the clinic. Disease activity was measured with the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). Associations between the LupusQoL-TR and SF-36 domains were examined while also examining age, disease duration, and disease activity for each questionnaire. Descriptive statistics, Spearman's correlation coefficients, and Students t test were performed to analyze the data. A total of 113 consecutive patients with lupus (F/M 108:5, mean age 40.6 +/- 11.9 years, mean disease duration 8.5 +/- 7.0 years) were included, and 69 % of these were active. The median SLEDAI score was 2 (0-24), the mean global LupusQoL-TR score was 60.9 +/- 23.3, and the mean SF-36 score was 41.2 +/- 9.0. There was a significant correlation between LupusQoL-TR and SF-36 mean scores (r = 0.83; p < 0.001). QoL assessed by LupusQoL-TR and SF-36 did not correlate with disease activity (r = -0.11; p = 0.244 and r = -0.03; p = 0.721, respectively). LupusQoL-TR and SF-36 questionnaires were beneficial instruments in evaluating HRQoL in Turkish lupus patients. However, LupusQoL-TR and SF-36 were not associated with SLEDAI scores, which suggested that QoL might be affected by other factors besides disease activity, especially in clinically inactive or mildly active patients.Publication Metadata only Association of FMF-Related (MEFV) point mutations with secondary and FMF amyloidosis(KARGER, 2004) DİRESKENELİ, RAFİ HANER; Atagunduz, MP; Tuglular, S; Kantarci, G; Akoglu, E; Direskeneli, HBackground: Familial Mediterranean fever (FMF) is the major cause of AA amyloidosis in Turkey. M694V mutation in MEFV gene was suggested to be associated with severe clinical features and amyloidosis of FMF. Methods: In this study, the frequencies of three FMF-related MEFV mutations (M694V, M6801 and V726A) were investigated in FMF patients with (AA-FMF, n=37) and without amyloidosis (non-AA-FMF, n=35), in patients with secondary amyloidosis related to non-FMF inflammatory conditions (S-AA, n=19) and in a non-inflammatory control group (n=185) by molecular genetic studies using polymerase chain reaction with the ARMS (amplification refractory mutation system) method. Results: Both AA and non-AA-FMF patients had significantly higher MEFV mutations compared to non-inflammatory controls (81 and 62.7% respectively vs. 4.2%, p=0.0001). AA-FMF patients carried significantly more MEFV mutations than non-AA-FMF patients (p=0.01). M694V was the most common mutation in both FMF groups (63.5 vs. 51.4%), however allele frequency (p=0.17) and the number of homoyzgous patients for this mutation did not differ between the groups (p=0.77). Although lower compared to FMF patients, S-AA patients also had a significantly higher incidence of MEFV mutations than non-inflammatory controls (21 vs. 4.2%) (p=0.0002). M694V was the only MEFV mutation in this group. Conclusion: MEFV mutations are found to be increased both in FMF and non-FMF associated secondary amyloidosis in our study; however, no clear association between M694V and amyloidosis is observed, except in the non-FMF group. Our results suggest that MEVF mutations may also serve as a severity marker for other inflammatory conditions. Copyright (C) 2004 S. Karger AG, Basel.Publication Metadata only Familial Mediterranean Fever (FMF) in Turkey: Results of a nationwide multicenter study(Lippincott Williams and Wilkins, 2005) DİRESKENELİ, RAFİ HANER; Tunca M., Ozdogan H., Kasapcopur O., Yalcinkaya F., Tutar E., Topaloglu R., Yilmaz E., Arici M., Bakkaloglu A., Besbas N., Akpolat T., Dinc A., Erken E., Tirpan K., Ozer H.T.E., Soyturk M., Senturk T., Balci B., Ozguc M., Dundar M., Akar E., Ozel D., Soylemezoglu O., Gunesacar R., Booth D.R., Hawkins P.N., Touitou I., Aksentijevich I., Matzner Y., Arslan S., Balaban Y., Batman F., Bayraktar Y., Apras S., Calguneri M., Duzova A., Kav T., Ozaltin F., Simsek H., Sivri B., Tatar G., Akkoc N., Kavukcu S., Soylu A., Turkmen M., Unsal E., Arisoy N., Caliskan S., Gogus F., Masatlioglu S., Sever L., Akkok N., Cakar N., Kara N., Kocak H., Ozalp S., Bilge I., Sevinc E., Gul A., Kamali S., Sadikoglu B., Selcukbiricik F., Sirin A., Sucu A., Bek K., Bulbul M., Delibas A., Demircin G., Erdogan O., Oner A., Mesiha M., Ozkaya N., Tekin M., Demirkaya E., Erdem H., Gok F., Pay S., Islek I., Kabasakal Y., Keser G., Ozmen M., Akoglu E., Atagunduz P., Direskeneli H., Temel M., Tuglular S., Buyan N., Bakkaloglu S., Derici U., Goker B., Kalman S., Ozkaya O., Dusunsel R., Gunduz Z., Poyrazoglu M.H., Korkmaz C., Baskin E., Koseoglu H.K., Saatci U., Yucel E., Coban E., Yakupoglu G., Oktem F., Tunc E., Cobankara V.Familial Mediterranean fever (FMF) is an autosomal recessive disease that is prevalent among eastern Mediterranean populations, mainly non-Ashkenazi Jews, Armenians, Turks, and Arabs. Since a large proportion of all the FMF patients in the world live in Turkey, the Turkish FMF Study Group (FMF-TR) was founded to develop a patient registry database and analyze demographic, clinical, and genetic features. The cohort was composed of 2838 patients (mean age, 23.0 ± 13.33 yr; range, 2-87 yr), with a male:female ratio of 1.2:1. There was a mean period of 6.9 ± 7.65 years from disease onset to diagnosis; the period was about 2 years shorter for each decade since 1981. Ninety-four percent of patients were living in the central-western parts of the country; however, their familial origins (70% from the central-eastern and Black Sea regions) reflected not only the ongoing east to west migration, but also the historical roots of FMF in Turkey. Patients' clinical features included peritonitis (93.7%), fever (92.5%), arthritis (47.4%), pleuritis (31.2%), myalgia (39.6%), and erysipelas-like erythema (20.9%). Arthritis, arthralgia, myalgia, and erysipelas-like erythema were significantly more frequent (p < 0.001) among patients with disease onset before the age of 18 years. Genetic analysis of 1090 patients revealed that M694V was the most frequent mutation (51.4%), followed by M680I (14.4%) and V726A (8.6%). Patients with the M694V/M694V genotype were found to have an earlier age of onset and higher frequencies of arthritis and arthralgia compared with the other groups (both p < 0.001). In contrast to other reported studies, there was no correlation between amyloidosis and M694V homozygosity in this cohort. However, amyloidosis was still remarkably frequent in our patients (12.9%), and it was prevalent (27.8%) even among the 18 patients with a disease onset after age 40 years. Twenty-two patients (0.8%) had nonamyloid glomerular diseases. The high prevalence of vasculitides (0.9% for polyarteritis nodosa and 2.7% for Henoch-Schönlein purpura) and high frequency of pericarditis (1.4%) were striking findings in the cohort. Phenotype II cases (those patients with amyloidosis as the presenting or only manifestation of disease) were rare (0.3% or less). There was a high rate of a past diagnosis of acute rheumatic fever, which suggested a possible misdiagnosis in children with FMF presenting with recurrent arthritis. To our knowledge, this is the largest series of patients with FMF reported from 1 country. We describe the features of the disease in the Turkish population and show that amyloidosis is still a substantial problem.Publication Metadata only Activity and damage in granulomatosis with polyangiitis(WILEY, 2013) DİRESKENELİ, RAFİ HANER; Yegin, Ender G.; Can, Meryem; Yilmaz, Neslihan; Aydin, Sibel Z.; Yavuz, Sule; Tuglular, Serhan; Direskeneli, HanerAim To retrospectively analyze disease activity and damage-associated factors in granulomatosis with polyangiitis (GPA) in Turkey. Method A retrospective analysis was carried out in 21 GPA patients. Assessments for activity were performed with the Birmingham Vasculitis Activity Score for GPA (BVAS/GPA) and for permanent organ damage by the Vasculitis Damage Index (VDI). Results Lower BVAS/GPA (P=0.002), absence of renal involvement (P=0.003) and higher creatinine clearence (P=0.000) at diagnosis increased the likelihood of achieving remission at 6weeks. Relapses were associated with high creatinine clearence (P=0.021), low BVAS/GPA (P=0.014), absence of renal involvement (P=0.036) and proteinuria (<0.5/24h) (P=0.013) at diagnosis, whereas achieving remission at 6weeks (P=0.012) was associated with absence of co-trimoxazole usage (P=0.038) and less severe clinical subgroup (P=0.034). Lower cumulative first 6months of cyclophosphamide and methylprednisolone were associated with earlier (12months) relapses (P=0.048 and P=0.083, respectively). Baseline damage (VDI1) was associated with a delay in diagnosis (P=0.032), presentation with milder clinical subgroups (P=0.052) and low serum creatinine (P=0.013). The increase in VDI in the first 12months (early damage) constituted most (91%) of the total damage measured at the end of follow-up. Conclusions Despite high early remission rates, relapse represents a major problem in localized GPA in our study. Baseline damage was associated with longer diagnostic delay and lower baseline serum creatinine. The initial phase of the disease seems to be the most crucial period for mortality and accumulated damage.Publication Open Access Prediction of subclinical left ventricular dysfunction by speckle-tracking echocardiography in patients with anti-neutrophil cytoplasmic antibody--associated vasculitis(2021-12-24) İZGİ, TUBA NUR; ATAŞ, HALİL; VELİOĞLU, ARZU; BARUTÇU ATAŞ, DİLEK; ILGIN, CAN; ALİBAZ ÖNER, FATMA; DİRESKENELİ, RAFİ HANER; ARIKAN, İZZET HAKKI; TUĞLULAR, ZÜBEYDE SERHAN; AŞICIOĞLU, EBRU; Izgi T. N., Atas D., ATAŞ H., Akaslan D., Ilgin C., VELİOĞLU A., ARIKAN İ. H., Alibaz-Oner F., DİRESKENELİ R. H., TUĞLULAR Z. S., et al.Objectives: This study aims to evaluate left ventricular functions using speckle-tracking echocardiography (STE) in patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Patients and methods: Between June 2018 and July 2019, a total of 31 AAV patients (17 males, 14 females; median age: 53 years; range, 47 to 62 years) and 21 healthy controls (11 males, 10 females; median age: 56 years; range, 46 to 60 years) were included in the study. Clinical and biochemical characteristics of all participants were recorded. All participants underwent conventional and two-dimensional STE. The receiver operating characteristic (ROC) curve analysis was performed to determine the cut-off value of serum N-terminal prohormone of brain natriuretic peptide (NT-pro-BNP) that predicted subclinical left ventricular dysfunction. The Spearman correlation analysis was used to determine the correlation between left ventricular global longitudinal strain (LV-GLS) and NT-pro-BNP. Results: The LV-GLS was lower in AAV patients (19.3% vs. 21.7%, respectively; p=0.014). NT-pro-BNP was negatively correlated with LV-GLS (p=0.005, r=0.401). Conclusion: Subclinical left ventricular dysfunction can be detected by STE in patients with AAV who have free of clinically overt cardiovascular disease. The LV-GLS is negatively correlated with serum NT-pro-BNP levels.Publication Metadata only Vitamin D Levels in Patients With Small and Medium Vessel Vasculitis [Niveles de vitamina D en pacientes con vasculitis de pequeños y vasos medianos](Ediciones Doyma, S.L., 2021) AŞICIOĞLU, EBRU; Korkmaz F.N., Ozen G., Unal A.U., Odabasi A., Can M., Asıcıoglu E., Tuglular S., Direskeneli H.Objectives: To determine the prevalence of vitamin D deficiency in patients with small and medium vessel systemic vasculitis. Methods: In this cross-sectional study, 25-hydroxy (OH) vitamin D3 levels were measured in adult patients with systemic small and medium vessel vasculitis including antineutrophil cytoplasmic antibody-associated vasculitis (AAV), cryoglobulinaemic vasculitis (CryV), IgA vasculitis (IgAV) and polyarteritis nodosa (PAN), and age- and sex-matched healthy subjects (HS) and patients with rheumatoid arthritis (RA) as control groups. 25OH vitamin D3 levels < 30 ng/ml and <20 ng/ml were regarded as insufficiency and deficiency, respectively. Results: Fifty-seven patients (42 AAV, 2 CryV, 8 IgA vasculitis, 5 PAN) with systemic vasculitis, 101 HS, and 111 RA patients were included. The mean 25OH vitamin D3 level was 21.8 ± 14.2 ng/mL in patients with vasculitis, 42.7 ± 27.6 ng/mL in HS (p < .001) and 20.1 ± 18.47 ng/mL in patients with RA (p = .54). Vitamin D insufficiency and deficiency were significantly higher in patients with systemic vasculitis compared to HS (75.4% vs 33.7%, p < .001; %50 vs 21.8%, p < .001, respectively). Vitamin D status was not different in patients with systemic vasculitis compared to RA. There was a negative correlation between vitamin D status and CRP levels (=−.364, p = .007). The multivariate logistic regression analysis showed that renal involvement was significantly associated with vitamin D deficiency/insufficiency in patients with vasculitis (OR 22.5 [95% CI 1.6–128.9]. Conclusion: Vitamin D deficiency and insufficiency are more frequent in patients with systemic small and medium vessel vasculitis and RA than HS. Renal involvement is one of the factors associated with vitamin D deficiency/insufficiency in patients with vasculitis. © 2020 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de ReumatologíaPublication Metadata only Fibroscan detection of fatty liver and liver fibrosis in patients with systemic lupus erythematosus(2022-05-01) YILMAZ, YUSUF; BARUTÇU ATAŞ, DİLEK; ALİBAZ ÖNER, FATMA; DİRESKENELİ, RAFİ HANER; TUĞLULAR, ZÜBEYDE SERHAN; AŞICIOĞLU, EBRU; VELİOĞLU, ARZU; Yetginoglu O., Atas D., Yilmaz Y., Velioglu A., Arikan H., Alibaz-Oner F., Direskeneli H., Tuglular S., Asicioglu E.Objective Although liver dysfunction is not considered the main organ involvement in Systemic Lupus Erythematosus (SLE), the frequency of liver dysfunction or abnormal liver enzyme values may be observed in 50-60% of patients. The aim of this study was to assess fatty liver and liver fibrosis in SLE patients using Fibroscan as well as determine associated factors such as immunosuppressive medications. Methods Sixty SLE patients and 30 healthy controls were included. Patients with HBV, HCV or cirrhosis, malignancy, cardiac disease, or patients on dialysis were excluded. All participants underwent Fibroscan measurements. Results The prevalence of fatty liver disease was similar between SLE patients and healthy controls (21.7 vs 26.7%, p = .597). Liver fibrosis was also similar between the two groups (26.7 vs 10.0%, p = .069). Since the majority of SLE patients were female, we performed a subgroup analysis in female patients (n = 51) and controls (n = 25). Fatty liver disease was similar between female SLE patients and controls (23.5 vs 24.0%, p = .964). However, liver fibrosis in female patients with SLE was increased compared to female controls (29.4 vs 4.0%, p = .011) and was associated with age (Exp (B) 95% CI: 1.083 (1.006-1.166), p = .034) and low-dose cumulative glucocorticoid use (Exp (B) 95% CI: 14.116 (1.213-164.210), p = .034). Conclusion The prevalence of fatty liver was similar between SLE patients and controls, while liver fibrosis was increased in the female patient group as compared to controls. Furthermore, liver fibrosis was associated with age and low dose cumulative glucocorticoid use. Interestingly, fatty liver did not precede liver fibrosis in the majority of cases, contrary to what is observed in the general population. Larger studies are needed to confirm our findings and determine whether immunosuppressive use has any impact on the development of liver fibrosis in SLE patients.Publication Metadata only The prevalence of metabolic syndrome is increased in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis(SPRINGER, 2021) VELİOĞLU, ARZU; Atas, Dilek Barutcu; Atas, Halil; Izgi, Tuba Nur; Velioglu, Arzu; Arikan, Hakki; Oner, Fatma Alibaz; Direskeneli, Haner; Tuglular, Serhan; Asicioglu, EbruPurpose Cardiovascular disease is one of the major causes of mortality in anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). Metabolic syndrome (MetS) is associated with increased cardiovascular risk in the normal population. However, MetS in AAV has not been adequately investigated. We aimed to determine MetS prevalence and associated factors in AAV patients. Methods Thirty-seven AAV patients and 42 healthy controls were enrolled. MetS was determined by International Diabetes Federation (IDF) and National Cholesterol Education Program Adult Treatment Panel III (NCEP-ATPIII) criteria. The relationship between clinical features of AAV and MetS was also investigated. Results MetS was significantly higher in AAV patients than controls by NCEP-ATPIII (51.4% vs. 26.2%, p 0.022) and IDF (62.2% vs. 35.7%, p 0.020). When AAV patients with MetS were compared to those without, there were significant differences in age, CRP, GFR and NT-pro-BNP. Age [58 (13) vs. 50 (8) years p: 0.028], CRP [4.0 (3.6) vs. 3.2 (1.0) mg/l, p 0.021] and NT-pro-BNP [173.5 (343.7) vs. 106.0 (103.0) pg/ml, p 0.013] were significantly higher in AAV patients with MetS than those without; GFR was significantly lower [38 (46) vs. 83 (51) ml/min/1.73 m(2), p 0.004]. ROC curve analysis showed NT-pro-BNP > 58.0 ng/ml predicted MetS with 87.1% sensitivity and 46.7% specificity (Area under curve: 0.71, CI 0.536-0.902, p 0.041). Multivariate analysis revealed age [OR (95% CI): 1.180 (1.010-1.370), p 0.039] and NT-pro-BNP > 58 pg/ml [OR (95% CI): 5.5 (1.02-30.1) p 0.047] were independent predictors of MetS in AAV patients. Conclusion MetS is significantly higher in AAV patients than controls and is associated with age and NT-pro-BNP. Screening and treating MetS may improve prognosis in AAV patients.