Person: DİRESKENELİ, RAFİ HANER
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DİRESKENELİ
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RAFİ HANER
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Publication 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 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.Publication Metadata only The relationship between perceived stress with anxiety, depression, sleep quality, insomnia and drug adherence in patients with systemic lupus erythematosus during the covid-19 pandemic(2022-05-01) DİRESKENELİ, RAFİ HANER; BARUTÇU ATAŞ, DİLEK; ARIKAN, İZZET HAKKI; GÖKMEN YILDIRIM, KARDELEN; VELİOĞLU, ARZU; ALİBAZ ÖNER, FATMA; TUĞLULAR, ZÜBEYDE SERHAN; AŞICIOĞLU, EBRU; TUĞCU, MURAT; GÖKMEN YILDIRIM K., BARUTÇU ATAŞ D., TUĞCU M., VELİOĞLU A., ARIKAN İ. H., ALİBAZ ÖNER F., DİRESKENELİ R. H., TUĞLULAR Z. S., AŞICIOĞLU E.AIMS: Sleep disorders, depression and anxiety are commonly reported in patients with systemic lupus erythematosus (SLE). Public health emergencies such as pandemics can also increase these psychosocial distresses. Early diagnosis and treatment of these disorders will substantially affect patients' quality of life and medication adherence. The aim of this study was to evaluate both medication non-adherence and the incidence of perceived stress, anxiety, depression, sleep quality and insomnia during the COVID pandemic in patients with SLE. METHOD: This was a cross-sectional, descriptive survey study. A total of 211 participants, including 160 SLE patients aged 18 years and older and 51 healthy volunteers who were similar in age and gender, were included. A questionnaire of socio-demographics and COVID-19 status, Medication Compliance Reporting Scale (MARS-5), Perceived Stress Scale (PSS), Hospital Anxiety and Depression Scale (HADA and HAD-D), Pittsburgh Sleep Quality Index (PSQI) and Insomnia Severity Index (ISI) scales was assessed. The participants were interviewed face to face, and the answers were recorded by the researcher. RESULTS: The mean age of the patients was 41.85 ± 12.97 years and 142 (88.7%) of the patients were female. There was no significant difference between the patient and control groups in terms of the history of COVID-19 infection, symptoms and hospitalization. Fifty-nine (36.9%) patients had high perceived stress, 16 (10.0%) had anxiety, 45 (28.1%) had depression, 77 (48.1%) had poor sleep quality and 62 (38.8%) patients had insomnia. PSS (23.64 ± 7.86 versus 19.73 ± 4.80, P = .001), HAD-D (5.60 ± 3.40 versus 4.08 ± 2.21, P = .003), PSQI (6.31 ± 3.62 versus 4.43 ± 2.20, P = .001) and ISI (6.81 ± 4.98 versus 4.53 ± 2.83, P = .002) scores were significantly higher in the patient group than controls. Patients with PSS score ≥ 25 were categorized as patients with a high PSS score. Presence of anxiety, depression, poor sleep quality and insomnia were significantly higher in patients with a high PSS score. Medication non-adherence was detected in 79 (49.4%) of the patients. Interestingly, there was no difference in MARS-5 scores between high and low PSS groups. Comparison of baseline characteristics and clinical data of the patients according to PSS score is shown in Table 1. The high PSS score was positively correlated with HAD-A, HAD-D, PSQI and ISI scores. Regression analysis revealed that high perceived stress is an independent predictor of depression [Exp(β) 95% CI 1.488 (1.245-1.779), P < .001], and anxiety [Exp(β) 95% CI 1.235 (1.026-1.487), P = .026]. CONCLUSION: SLE patients demonstrated increased levels of perceived stress, depression, poor sleep quality and insomnia compared to the healthy population during the COVID-19 pandemic. SLE patients with high perceived stress had more depression, anxiety, poor sleep quality and insomnia than those without. It needs to be determined whether these findings will have an impact on patient outcomes during long-term follow-up. Palavras-chave adult; anxiety; conference abstract; controlled study; coronavirus disease 2019; depression; female; follow up; gender; Hospital Anxiety and Depression Scale; hospitalization; human; incidence; insomnia; Insomnia Severity Index; major clinical study; male; medication compliance; outcome assessment; pandemic; Perceived Stress Scale; physiological stress; Pittsburgh Sleep Quality Index; questionnaire; sleep quality; sociodemographics; systemic lupus erythematosus; young adultPublication Metadata only Fatigue in systemic lupus erythematosus Association with disease activity, quality of life and psychosocial factors(SPRINGER HEIDELBERG, 2017) ALİBAZ ÖNER, FATMA; Yilmaz-Oner, S.; Ilhan, B.; Can, M.; Alibaz-Oner, F.; Polat-Korkmaz, O.; Ozen, G.; Mumcu, G.; Kremers, H. M.; Tuglular, S.; Direskeneli, H.Objective. The aim of the study was to determine which disease-related factors and non-disease features can explain the presence of systemic lupus erythematosus (SLE)-related fatigue in Turkish patients. Methods. This cross-sectional study was carried out with 99 SLE patients and 71 healthy controls. To assess fatigue and health-related quality of life (HRQoL) the participants were asked to complete two questionnaires: the short form-36 health survey (SF-36) and the multidimensional assessment of fatigue (MAF) scale. Anxiety and depression of participants were assessed by the hospital anxiety and depression scale (HADS). Results. A total of 99 patients (female/male 95/4) and 71 controls (female/male 40/31) were studied. The mean age and standard deviation (+/- SD) of patients and controls were 43.3 +/- 12.2 years and 43.2 +/- 12.1 years, respectively. The mean (SD) disease duration was 7.8 +/- 5.3 years and median SLE disease activity index (SLEDAI) score was 0 (range = 0-16). The level of fatigue was higher in patients compared to controls with mean MAF scores of 24.7 +/- 12.2 and 12.8 +/- 9.9 (p < 0.001), respectively. The HADS-D and HADS-A scores were also significantly higher in SLE patients (6.6 +/- 4.3 vs. 3.6 +/- 2.9, p < 0.001 and 7.2 +/- 4 vs. 4.9 +/- 4, p = 0.007, respectively). There were no significant associations between the MAF and SLEDAI scores (r = 0.05, p = 0.63) but MAF scores positively correlated with age, HADS-A and HADS-D scores and negatively correlated with physical component summary (PCS), mental component summary (MCS) and each domain of SF-36 except role emotional in SLE patients. Conclusion. Fatigue is an important factor influencing patient daily life independent from disease activity in our study. The SLE patients with severe fatigue should also be assessed for other possible underlying causes such as anxiety, depression and poor quality of life.Publication Metadata only FRAX scores are increased in patients with ANCA-associated vasculitis(SPRINGER, 2021) VELİOĞLU, ARZU; Cetin, Betul; Cetin, Emin Ahmet; Arikan, Hakki; Velioglu, Arzu; Alibaz-Oner, Fatma; Direskeneli, Haner; Tuglular, Serhan; Asicioglu, EbruPurpose Prognosis in ANCA-associated vasculitis (AAV) has greatly improved with immunosuppressive use whereas incidence of treatment-related comorbidities such as osteoporosis has increased. However, studies investigating bone disease in AAV are limited. Fracture Risk Assesment Tool (FRAX) was developed to estimate 10-year hip and major osteoporotic fracture risks. Aim of this study was to estimate FRAX scores in AAV patients and compare them to healthy controls. Methods 30 AAV patients and 20 healthy controls were included. Demographic, disease, and medication history were recorded from patient files. Femoral neck, lumbar spine and forearm bone mineral densitometry, and thoracolumbar radiographs were performed. FRAX fracture risk scoring was assessed for all participants. Results There were 18 male and 12 female patients. Mean age was 58.5 +/- 11.7 years. Osteoporosis and osteopenia were present in 23.3% and 50% of patients, respectively. There were fractures in eight patients (26.7%). FRAX major fracture (9.4 +/- 7.3% vs 5.9 +/- 3.2%, p = 0.02) and hip fracture (2.2 +/- 3.2% vs 0.9 +/- 0.8%, p = 0.03) scores were higher in patients than controls. In seven (23.3%) patients, the 10-year probability of hip fracture was >= 3% and in five (16%) patients the 10-year risk of a major osteoporosis-related fracture was >= 20%. None of the controls exceeded these thresholds. Conclusion AAV patients are at high risk for future fractures as calculated with FRAX. Life-long monitoring for bone disease and fractures are essential. Large studies with longer follow-up are needed to determine the accuracy of FRAX risk scoring in predicting fractures.