To estimate the prevalence and incidence of Takayasu arteritis (TA) among the residents of the ci... more To estimate the prevalence and incidence of Takayasu arteritis (TA) among the residents of the city of Izmir, third biggest metropolis of Turkey. Five tertiary care teaching hospitals, which were the only ones that provided rheumatology specialty care during the study period in the city of Izmir from 2006 through 2010, were invited to take part in the present study. Case search was performed electronically in the information systems of these hospitals using The International Classification of Diseases Tenth Revision (ICD-10) code for Takayasu arteritis (M31.4). The diagnosis was confirmed through chart review by a rheumatologist according to the 1990 American College of Rheumatology (ACR) criteria. Annual prevalence was calculated based on the number of patients that were alive at the end of 2010. Age- and sex-adjusted prevalence rates were standardised according to the 2010 Turkish population, based on 2010 Turkish Census. A total of 41 patients were confirmed to have TA and also t...
Revista Brasileira de Reumatologia (English Edition), 2015
Objetivo: A artrite gostosa e a febre familiar do Mediterrâneo (FFM) compartilham algumas caracte... more Objetivo: A artrite gostosa e a febre familiar do Mediterrâneo (FFM) compartilham algumas características clínicas e patológicas, como ser classificada como uma doença autoimune inflamatória, ter associação com o inflamassoma, manifestar artrite intermitente de curta duração e boa resposta a tratamentos com colchicina e anti-interleucina-1. Como o gene da febre familiar do Mediterrâneo (MEFV) é o fator causador da FFM, este estudo teve como objetivo investigar a prevalência de mutaç ões do gene MEFV e seu efeito sobre as manifestaç ões da doença em pacientes turcos com artrite gotosa. Métodos: Foram incluídos no estudo 97 pacientes com diagnóstico de artrite gotosa primária (93 M e 4 F; 54 [37-84] anos) e 100 controles saudáveis (94 M e 6 F; 57 [37-86] anos). Todos os indivíduos foram submetidos à análise do genótipo à procura de variaç ões no MEFV. Também foi registrado o número de crises de gota, o uso de diuréticos e a história de nefrolitíase e presença de tofos. Resultados: A frequência de portadores de mutaç ões no MEFV em pacientes e controles foi de 22,7% (n = 22) e 24% (n = 24), respectivamente. A comparação entre os pacientes e os controles não produziu diferença estatisticamente significativa em termos de frequência de portadores de mutaç ões no MEFV (p = 0,87). As frequências alélicas de mutaç ões no MEFV nos pacientes foram de 11,9% (n = 23) e 14% (n = 28) nos controles (p = 0,55). A presença de variantes do MEFV não mostrou qualquer associação com as características clínicas da artrite gotosa. A análise por subgrupos de pacientes revelou que aqueles com artrite gotosa com mutaç ões tinham frequências semelhantes de tofo, história de nefrolitíase e podogra em comparação com os indivíduos sem mutaç ões (p > 0,05). Conclusões: As mutaç ões no gene MEFV não exercem um papel relevante em pacientes turcos com artrite gotosa.
BACKGROUND The postinfectious irritable bowel syndrome (PI-IBS) frequently follows bacterial infe... more BACKGROUND The postinfectious irritable bowel syndrome (PI-IBS) frequently follows bacterial infections. Since AND AIM: people suffering from PI-IBS and Trichinella spiralis-infected mice develop similar findings, this animal model has been successfully used for PI-IBS studies; however, IBS has never been studied in humans after trichinellosis. The aim of this study was to evaluate the IBS symptoms in people who had acquired trichinellosis. PATIENTS A large outbreak of trichinellosis caused by Trichinella britovi occurred in Izmir, Turkey, in AND METHODS: 2003-2004. The diagnosis of trichinellosis was confirmed by serology and muscle biopsy. After clinical and laboratory evaluations, 72 patients (38 women, 34 men, mean age 33.2 ± 10.4 yr) with trichinellosis without preexisting IBS were enrolled in the study. Noninfected people (N = 27) were used as control group. A questionnaire was developed to assess symptoms of IBS and alarm symptoms. The first interview was done face-to-face. After 2, 4, and 6 months of the first interview, the questionnaire was readministered to the patients without any information on the occurrence of a previous trichinellosis syndrome, while it was applied after a year only to the patients who had suffered IBS symptoms according to at least one of the previous interviews. RESULTS: According to Rome II criteria, PI-IBS developed in 10 of 72 (13.9%) people with confirmed trichinellosis, who had no preexisting IBS. The rate of PI-IBS was 13.9% and 13.9% at the 4th and 6th months, respectively. The symptoms of PI-IBS persisted in five of them up to 1 yr. People without trichinellosis did not develop any IBS symptoms. CONCLUSIONS: This is the first report of the development of PI-IBS after T. britovi. Consequently, IBS can be considered as a secondary syndrome induced by trichinellosis.
Henoch-Schönlein purpura (HSP) belongs to the category of systemic small-vessel vasculitis. Altho... more Henoch-Schönlein purpura (HSP) belongs to the category of systemic small-vessel vasculitis. Although long-term outcome is generally good, serious complications may occur. Thrombosis and priapism have been reported only as extremely rare complications of HSP. We describe a 37-year-old man who developed recurrent thrombotic events shortly after he had been diagnosed as having HSP. Although he had additional risk factors for thrombosis, such as prothrombin G20210A mutation and use of celecoxib before the last episode, temporal relation of the thrombotic attacks to the onset of HSP suggest that the disease itself may lead to a prothrombotic state. This case is the first adult HSP patient with priapism, which probably developed secondary to thrombosis of the dorsal penile vein.
Tumor necrosis factor (TNF) alpha inhibitors are effective in the treatment of inflammatory rheum... more Tumor necrosis factor (TNF) alpha inhibitors are effective in the treatment of inflammatory rheumatic diseases. Despite their effectiveness, anti-TNF drugs have some drawbacks such as severe adverse effects including infections and possibly lymphoproliferative disorders. In this report we described a case of juvenile idiopathic arthritis patient who developed thymic enlargement (true thymic hyperplasia), mediastinal lymphadenopathy and pleurisy associated with systemic symptoms under Etanercept treatment. The clinical presentation was highly suggestive of malignancy and the patient underwent diagnostic mediastinoscopy with biopsy.
Osteopoikilosis (OPK) is a rare benign sclerosing disease of the skeleton and inherited as an aut... more Osteopoikilosis (OPK) is a rare benign sclerosing disease of the skeleton and inherited as an autosomal dominant trait. OPK is associated with inflammatory rheumatic disorders, such as rheumatoid arthritis, scleroderma, reactive arthritis and familial Mediterranean fever (FMF). We report a rare case of OPK coexistent with ankylosing spondylitis and FMF. The patient presented multiple sclerotic lesions within and around the sacroiliac joints and a series of radiological diagnostic challenges.
Rheumatic manifestations are the most common extraintestinal findings of inflammatory bowel disea... more Rheumatic manifestations are the most common extraintestinal findings of inflammatory bowel disease (IBD), although there are wide variations among different studies. The only previous Turkish study reported a rather high prevalence of spondyloarthritis (SpA) in patients with IBD. We aimed to determine the frequency of SpA and ankylosing spondylitis (AS) in patients with IBD attending a gastroenterology clinic from a referral centre. The study was conducted in 122 patients with established diagnosis of IBD [28 with Crohn's disease (CD) and 94 with ulcerative colitis (UC)]. A detailed medical history was obtained and a complete physical examination was performed in all the patients. Standard pelvic X-rays for examination of the sacroiliac joints were performed only when clinically indicated. The X-rays were read blindly by an experienced rheumatologist and reported according to the established grading system. The modified New York criteria were used to classify AS, and the European Spondyloarthropathy Study Group criteria for SpA. The prevalence of AS and SpA in patients with IBD was 8.2 and 28.7%, respectively. SpA was found to be significantly more common in the patients with CD compared to patients with UC, but the frequency of AS was not different between these two groups. There was no correlation between localisation or extent of the intestinal inflammation and presence of AS and SpA. A higher frequency of women was observed in patients diagnosed as SpA. Almost half of the patients with SpA (45.7%) had not been diagnosed before the study, although they had a history of IBP and/or peripheral arthritis. This study suggests that the prevalences of SpA and AS in Turkish patients with IBD are similar to those in many other populations. There may be a significant female predominance of SpA among patients with IBD.
The aim of this study was to examine the prevalence of hyperuricemia and its associated factors i... more The aim of this study was to examine the prevalence of hyperuricemia and its associated factors in an urban area of Izmir, located in western Turkey. Our study group was selected by computerized sampling from the participants of a larger population-based study searching for the prevalence of rheumatoid arthritis in Balcova and Narlidere districts of Izmir. A total of 132 subjects (69 women and 63 men) were included in this study. Serum uric acid, glucose, creatinine and lipid levels were studied. Body composition along with body fat percentage was determined anthropometrically. A total of 16 subjects had hyperuricemia (4 women and 12 men). The overall prevalence of hyperuricemia was 12.1% and the mean uric acid level was 4.9 +/- 1.3 mg/dl. Males had significantly higher uric acid levels than females (P < 0.05; 5.5 +/- 1.3 vs. 4.3 +/- 1.1 mg/dl, respectively). The prevalence of hypertension, diabetes, obesity and metabolic syndrome was 24.4, 5.3, 28 and 26.5%, respectively. There was no gouty subject. Sum of skinfold thickness (SFT) measurements and creatinine levels were the independent predictors of hyperuricemia (beta = 0.45, 0.47, respectively). Uric acid measurement is important not only for inflammatory rheumatic disorders but also for predicting metabolic syndrome and related coronary artery disease. There is sex difference in uric acid levels in favor of women most probably explained by gonadal hormones. Hyperuricemia is significantly predicted by anthropometric measure of SFT which is a simple clinical screening method along with creatinine levels.
The objective is to report a case of atypical acute infectious mononucleosis in a juvenile ankylo... more The objective is to report a case of atypical acute infectious mononucleosis in a juvenile ankylosing spondylitis patient who was treated with infliximab. A 20-year-old man was hospitalized for the evaluation of lymphadenopathy and systemic symptoms. His symptoms developed at the eighth week of the infliximab treatment and he required hospitalization. Lymph node biopsy was performed and he was diagnosed as atypical infectious mononucleosis (absence of fever, pharyngitis, lymphocytosis and negative atypical lymphocytosis on blood smear). Infections have become major concerns in patients treated with TNF-blocking agents. In theoretical base, it is not surprising as TNF-alpha has a crucial role in the body's defense against both bacterial and viral invasion. Blocking the action of TNF may also change the course of the disease and could lead to a delay in the diagnosis. TNF-alpha-blocking treatment may mask the typical symptoms of infectious mononucleosis and atypical cases should be included in the differential diagnosis of lymphadenopathy in patients receiving anti-TNF-alpha agents.
resolution of the acute-phase response followed. All pharmacological treatments were withdrawn an... more resolution of the acute-phase response followed. All pharmacological treatments were withdrawn and the patient remains well 9 months following surgery. CD is a rare and heterogeneous group of disorders that can mimic systemic CTDs [3]. In childhood, it is usually of the localized type [4]. Patients are frequently asymptomatic but can occasionally present with systemic symptoms [5]. Treatment of unicentric disease of either histological type involves resection, resulting in cure. One caveat to this may be malignant transformation of unicentric CD [6]. Multicentric CD is usually of the plasma cell type and commonly presents with lymphadenopathy, systemic symptoms, organomegaly and acute-phase response [1]. The prognosis of this variant in adults is poor. Moreover, some cases are associated with Kaposi sarcoma and lymphoma [5, 7]. Such malignancies are less commonly associated with CD of the young. Therapy of this form of CD includes immune modulators such as corticosteroids, IFN-and cytotoxic immunosuppression with or without radiotherapy [8]. Monoclonal antibody therapy including anti-CD20 or anti-Il-6 has been used with anecdotal efficacy [1]. A number of recent observations have cast light on the pathogenesis of CD. The levels of IL-6 and other pro-inflammatory cytokines are raised [1]. There also appears to be an association of multicentric CD and HHV-8 infection [1]. This is one of the few cases of paediatric CD with renal involvement described. Previous reports in adult patients describe various forms of glomerular and interstitial pathology and a few cases of nephrotic syndrome caused by renal amyloidosis [9]. In adult patients, there is an emerging experience of 18 F-FDG PET for the diagnosis and subsequent follow-up of CD [10], but no such data in children. In our patient, initial abdominal lymph node biopsy identified only reactive inflammatory changes. Using 18 F-FDG PET/CT we were able to precisely identify the most metabolically active node with confidence, thus guiding the successful surgical management of this case. We conclude with the message that CD should be considered in children presenting with a lupus-like phenotype recalcitrant to cytotoxic immunosuppression.
Objectives: Trichinellosis is a cosmopolitan parasite infection caused by Trichinella nematodes t... more Objectives: Trichinellosis is a cosmopolitan parasite infection caused by Trichinella nematodes that is acquired from consumption of raw meat from several animal species. Knowledge of the clinical pattern and laboratory features of the disease in childhood is limited. The purpose is to study the clinical pattern of trichinellosis caused by Trichinella britovi in children and to compare it in household adults. Methods: We evaluated all children up to 17 years of age and their adult householders exposed to the consumption of infected meat during an outbreak of trichinellosis. A questionnaire was developed to record clinical data. The blood sample was collected for blood count, muscle enzymes, serum electrolytes, albumin and serology. All exposed children were treated with mebendazole, and severe symptomatic patients received prednisolone. Clinical and laboratory presentations and outcome were recorded. To evaluate the clinical picture of trichinellosis in childhood, clinical and laboratory findings were compared between children and household adults with a confirmed diagnosis who consumed the same amount of infected meat. Results: In 47 (62%) of 76 children with suspected trichinellosis, the diagnosis was serologically confirmed. The main clinical and laboratory findings in children were fever, abdominal pain, myalgia, facial and/or eyelid edema, rash, eosinophilia and increased muscular enzymes. The incubation period was similar in children and adults, but myalgia (66% versus 96%, P Ͻ 0.01), facial and/or eyelid edema (57% versus 86%, P Ͻ 0.05), eosinophilia (52% versus 96%, P Ͻ 0.01) and increased serum creatine kinase (38% versus 79%, P Ͻ 0.01) were less common in children than in adults. Seroconversion occurred in fewer children than adults, but the difference was not statistically significant. Conclusions: T. britovi infection shows a benign course and a milder clinical picture in children than in adults who consumed the same amount of infected meat.
The aim of this study was to determine the prevalence of primary Sjogren's syndrome (pSS)... more The aim of this study was to determine the prevalence of primary Sjogren's syndrome (pSS) in a general Turkish population according to the latest proposed American-European Consensus Group (AECG) criteria and European-1 (EU-1) criteria. The study was conducted in two districts of Izmir and involved 2835 subjects 20 years of age and older. In the first stage, face-to-face interviews were performed at the registered households. In the second stage, subjects reporting symptoms of both dry eye and dry mouth were invited to the hospital for a full examination, which included Schirmer-1, sialometry and serologic tests. In the third stage, a minor salivary gland biopsy was performed as required. A total of 2887 subjects were contacted and a complete interview was obtained for 2835 (1551 female, 1284 male) subjects. A total of 159 subjects (126 female, 33 male) confirmed oral and ocular dryness, and 86 of these patients (54.1%) underwent a detailed clinical examination in the hospital. pSS was diagnosed in 10 patients (nine females) according to the EU-1 criteria, and in six patients (six females) according to the AECG criteria. We found a minimum crude prevalence of 0.21% [95% confidence interval (CI): 0.03-0.29] in the sample population and an age-sex adjusted prevalence of 0.16% (95% CI: 0.06-0.35), according to AECG criteria. According to EU-1 criteria, these prevalence rates were found to be 0.35% (95% CI: 0.10-0.45) and 0.28% (95% CI: 0.13-0.51) respectively. The pSS prevalence rates found in the Turkish population in this study were lower than the estimated prevalence rate in a general population.
Thyrotoxic hypokalemic periodic paralysis (THPP) is a rare complication of hyperthyroidism and an... more Thyrotoxic hypokalemic periodic paralysis (THPP) is a rare complication of hyperthyroidism and an uncommon form of hypokalemic periodic paralysis. Its differentiation of more common forms of periodic paralysis is important because aggressive treatment can place the patient at risk for rebound hyperkalemia. Treatment of the underlying thyroid dysfunction cures the muscle symptoms. Here we describe a 37-year-old Turkish male with THPP whose paralysis attack recurred soon after administration of radioactive iodine.
Systemic inflammation plays an important role in the development of atherosclerosis (AS). The aim... more Systemic inflammation plays an important role in the development of atherosclerosis (AS). The aim of this study was to evaluate the presence of early AS in patients with familial Mediterranean fever (FMF) that is characterized by recurrent inflammatory attacks of serositis. Sixtyone FMF patients (30 Male/31 Female; 31.5 [18-54] years) and 31 healthy controls (16 Male/15 Female; 31 [22-58] years) were studied. All FMF patients were on regular daily colchicine treatment and during attack-free periods. Both the FMF patients and controls with a history of diabetes mellitus (DM), hypertension, and hyperlipidemia were excluded. Body mass index (BMI) was calculated. Serum lipids, C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR) were assessed. Two-hour oral glucose tolerance test was performed to rule out DM and glucose intolerance. To investigate early AS "endotheliumdependent flow-mediated dilatation (FMD%)," "nitroglyc-erin-induced endothelium-independent peripheral vasodilatation (NTG%)," and intima-media thickness (IMT) of common carotid arteries (CCA) were measured by ultrasonograpy. The median disease duration for FMF patients was 16 (1-45) years. Age, sex, BMI, smoking status, and serum lipids were comparable in patients and controls (p>0.05). However, ESR and standard CRP were significantly higher in the patients group (p<0.05). There were no differences in the measurements of right, left, and averaged IMT of CCA between patients and controls ([0.49 vs 0.5], [0.51 vs 0.52] and [0.5 vs 0.51]; p>0.05, respectively). None of the subjects had carotid artery plaques. FMD% and NTG% were also similar in patients and controls group ([18.2 vs 20.6] and [24.2 vs 22.5]; p>0.05, respectively). This study suggests that the markers of early AS are not impaired in FMF patients on regular daily colchicine treatment. Keywords Arteriosclerosis. Familial mediterranean fever. Ultrasonography Abbreviations AS atherosclerosis BAD brachial artery diameter BMI body mass index CCA common carotid artery CRP C-reactive protein CV cardiovascular DM diabetes mellitus ESR erythrocyte sedimentation rate FMD flow-mediated dilatation FMF familial Mediterranean fever HDL high density lipoprotein HT hypertension IMT intima-media thickness
It is well established that there are people with higher risk of developing acute rheumatic fever... more It is well established that there are people with higher risk of developing acute rheumatic fever (ARF) and rheumatic heart disease (RHD). Mediterranean fever (MEFV) gene mutations might be one of the genetic predisposition factors in the development of ARF/RHD since defect in familial Mediterranean fever (FMF) patients is proposed to be heightened inflammatory response to certain stimuli. Previous clinical observations suggested a relationship between FMF and ARF/RHD. The aim of this study was to investigate the role of the MEFV gene mutations in the susceptibility to RHD in Turkish patients. A total of 100 patients with RHD and 100 healthy controls were included in the study. Diagnosis of RHD was based on echocardiographic findings in which a predominant mitral stenosis was used as an inclusion criterion. Genetic analysis was carried out by sequence analysis investigating two hot spots (exons 2 and 10) for MEFV mutations. Mutation analysis showed that 22 RHD patients (22%) and 24 healthy controls (24%) carried at least one mutated allele. MEFV mutations were identified in 22 of 200 (11%) chromosomes in RHD patients while 26 of the 200 (13%) chromosomes of healthy controls were found to carry a mutated allele. No difference was found in allele frequencies and their distribution between the patients and healthy controls (p = 0.54). MEFV mutations are not associated with a predisposition to develop RHD in adult Turkish patients.
The aim of this study was to compare the effect of chronic inflammation on insulin resistance, se... more The aim of this study was to compare the effect of chronic inflammation on insulin resistance, serum leptin levels, and body composition (BC) in patients with ankylosing spondylitis (AS) and healthy controls. Twentyeight AS patients and 17 healthy controls were included in this study. Subjects with hypertension, diabetes, hyperlipidemia, and obesity were excluded. Acute phase reactants and serum levels of glucose, insulin, lipids, and leptin were studied. BC was determined anthropometrically and by foot-to-foot body fat analyzer (BIA, bioelectrical impedance analysis). Quantitative insulin-sensitivity check index, homeostasis model assessment for insulin resistance, and McAuley indices were calculated. Spinal mobility was assessed by the Bath Ankylosing Spondylitis Metrology Index (BASMI). Patients were also evaluated with the Bath Ankylosing Spondylitis Functional Index and the Bath Ankylosing Spondylitis Disease Activity Index. Age, sex distribution, smoking status, serum lipids, insulin concentrations, and insulin resistance indices were comparable between AS patients and controls (p>0.05). However, acute phase reactants were significantly higher and leptin levels were significantly lower in the AS patients than in controls (p<0.05). Fat percent assessed by both BIA and anthropometrical methods was lower in the male and female AS patients than in controls, and this reduced fat level reached statistical significance for men (p<0.05). There were significant correlations between percent body fat, body mass index, leptin, age, and BASMI (p<0.05; r=0.6, 0.75, 0.35, −0.41, respectively). On the other hand, body fat percent, waist-to-hip ratio, C-reactive protein, and BASMI were significantly correlated with serum leptin levels (p< 0.05; r=0.75, −0.42, −0.52, −0.47, respectively). Chronic inflammatory condition in AS may be responsible for the reduced body fat content and lower circulating leptin concentrations. Insulin levels and insulin resistance indices seem similar in patients and controls in the absence of classic vascular risk factors.
Background: Ankylosing spondylitis (AS) is a chronic inflammatory disease of spine and sacroiliac... more Background: Ankylosing spondylitis (AS) is a chronic inflammatory disease of spine and sacroiliac joints; it is characterized by new bone formation, and the disease processes can be accompanied by osteoporosis. In the present study, we investigated changes in bone mineral density (BMD) and in the levels of various bone turnover-related biomarkers and cytokines in a cohort of AS patients, with regard to clinical parameters, disease activity, and treatment regimen. Methods: 55 AS patients and 33 healthy controls included in the study. Spinal mobility was assessed by the Bath Ankylosing Spondylitis Metrology Index (BASMI), and radiologic changes were scored by the Bath Ankylosing Spondylitis Radiologic Index (BASRI). Patients were also evaluated with the Bath Ankylosing Spondylitis Functional Index (BASFI) and the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI). Bone mineral density (BMD) assessed by dual energy X-ray absorptiometry. Various biomarkers and cytokines of bone turnover including osteoprotegerin (OPG), serum band 5 tartrate-resistant acid phosphatase (TRAP-5), soluble receptor activator of nuclear factor kappa-B ligand (sRANKL), secreted frizzled-related protein 1 (sFRP-1), Dickkopf-related protein 1 (DKK-1), and sclerostin were studied. Results: The levels of TRAP-5, NTX, sRANKL, sclerostin, sFRP-1, DKK-1, and IFNγ, were similar between the patients and controls (p > 0.05), while BMD of femoral neck, and OPG levels were significantly lower in AS patients (p < 0.05). In a subgroup analysis, patients with active disease had significantly higher concentrations of OPG compared with the inactive group. Rest of the biomarkers and cytokines of bone turnover were similar between the active and inactive disease groups. Subgroup analysis of patients receiving anti-TNFα agents and conventional therapy revealed that OPG concentrations were significantly lower in the patients receiving biological drugs, while BAP and DKK-1 were significantly higher in the patients treated with conventional agents. Conclusions: In this cross-sectional study we showed that OPG levels were significantly lower in AS patients compared to healthy subjects. On the other hand, the levels of wingless (Wnt) signal pathway inhibitors seem not altered. Ectopic bone formation in AS may be related to dysfunction of these molecules at the cellular level.
To estimate the prevalence and incidence of Takayasu arteritis (TA) among the residents of the ci... more To estimate the prevalence and incidence of Takayasu arteritis (TA) among the residents of the city of Izmir, third biggest metropolis of Turkey. Five tertiary care teaching hospitals, which were the only ones that provided rheumatology specialty care during the study period in the city of Izmir from 2006 through 2010, were invited to take part in the present study. Case search was performed electronically in the information systems of these hospitals using The International Classification of Diseases Tenth Revision (ICD-10) code for Takayasu arteritis (M31.4). The diagnosis was confirmed through chart review by a rheumatologist according to the 1990 American College of Rheumatology (ACR) criteria. Annual prevalence was calculated based on the number of patients that were alive at the end of 2010. Age- and sex-adjusted prevalence rates were standardised according to the 2010 Turkish population, based on 2010 Turkish Census. A total of 41 patients were confirmed to have TA and also t...
Revista Brasileira de Reumatologia (English Edition), 2015
Objetivo: A artrite gostosa e a febre familiar do Mediterrâneo (FFM) compartilham algumas caracte... more Objetivo: A artrite gostosa e a febre familiar do Mediterrâneo (FFM) compartilham algumas características clínicas e patológicas, como ser classificada como uma doença autoimune inflamatória, ter associação com o inflamassoma, manifestar artrite intermitente de curta duração e boa resposta a tratamentos com colchicina e anti-interleucina-1. Como o gene da febre familiar do Mediterrâneo (MEFV) é o fator causador da FFM, este estudo teve como objetivo investigar a prevalência de mutaç ões do gene MEFV e seu efeito sobre as manifestaç ões da doença em pacientes turcos com artrite gotosa. Métodos: Foram incluídos no estudo 97 pacientes com diagnóstico de artrite gotosa primária (93 M e 4 F; 54 [37-84] anos) e 100 controles saudáveis (94 M e 6 F; 57 [37-86] anos). Todos os indivíduos foram submetidos à análise do genótipo à procura de variaç ões no MEFV. Também foi registrado o número de crises de gota, o uso de diuréticos e a história de nefrolitíase e presença de tofos. Resultados: A frequência de portadores de mutaç ões no MEFV em pacientes e controles foi de 22,7% (n = 22) e 24% (n = 24), respectivamente. A comparação entre os pacientes e os controles não produziu diferença estatisticamente significativa em termos de frequência de portadores de mutaç ões no MEFV (p = 0,87). As frequências alélicas de mutaç ões no MEFV nos pacientes foram de 11,9% (n = 23) e 14% (n = 28) nos controles (p = 0,55). A presença de variantes do MEFV não mostrou qualquer associação com as características clínicas da artrite gotosa. A análise por subgrupos de pacientes revelou que aqueles com artrite gotosa com mutaç ões tinham frequências semelhantes de tofo, história de nefrolitíase e podogra em comparação com os indivíduos sem mutaç ões (p > 0,05). Conclusões: As mutaç ões no gene MEFV não exercem um papel relevante em pacientes turcos com artrite gotosa.
BACKGROUND The postinfectious irritable bowel syndrome (PI-IBS) frequently follows bacterial infe... more BACKGROUND The postinfectious irritable bowel syndrome (PI-IBS) frequently follows bacterial infections. Since AND AIM: people suffering from PI-IBS and Trichinella spiralis-infected mice develop similar findings, this animal model has been successfully used for PI-IBS studies; however, IBS has never been studied in humans after trichinellosis. The aim of this study was to evaluate the IBS symptoms in people who had acquired trichinellosis. PATIENTS A large outbreak of trichinellosis caused by Trichinella britovi occurred in Izmir, Turkey, in AND METHODS: 2003-2004. The diagnosis of trichinellosis was confirmed by serology and muscle biopsy. After clinical and laboratory evaluations, 72 patients (38 women, 34 men, mean age 33.2 ± 10.4 yr) with trichinellosis without preexisting IBS were enrolled in the study. Noninfected people (N = 27) were used as control group. A questionnaire was developed to assess symptoms of IBS and alarm symptoms. The first interview was done face-to-face. After 2, 4, and 6 months of the first interview, the questionnaire was readministered to the patients without any information on the occurrence of a previous trichinellosis syndrome, while it was applied after a year only to the patients who had suffered IBS symptoms according to at least one of the previous interviews. RESULTS: According to Rome II criteria, PI-IBS developed in 10 of 72 (13.9%) people with confirmed trichinellosis, who had no preexisting IBS. The rate of PI-IBS was 13.9% and 13.9% at the 4th and 6th months, respectively. The symptoms of PI-IBS persisted in five of them up to 1 yr. People without trichinellosis did not develop any IBS symptoms. CONCLUSIONS: This is the first report of the development of PI-IBS after T. britovi. Consequently, IBS can be considered as a secondary syndrome induced by trichinellosis.
Henoch-Schönlein purpura (HSP) belongs to the category of systemic small-vessel vasculitis. Altho... more Henoch-Schönlein purpura (HSP) belongs to the category of systemic small-vessel vasculitis. Although long-term outcome is generally good, serious complications may occur. Thrombosis and priapism have been reported only as extremely rare complications of HSP. We describe a 37-year-old man who developed recurrent thrombotic events shortly after he had been diagnosed as having HSP. Although he had additional risk factors for thrombosis, such as prothrombin G20210A mutation and use of celecoxib before the last episode, temporal relation of the thrombotic attacks to the onset of HSP suggest that the disease itself may lead to a prothrombotic state. This case is the first adult HSP patient with priapism, which probably developed secondary to thrombosis of the dorsal penile vein.
Tumor necrosis factor (TNF) alpha inhibitors are effective in the treatment of inflammatory rheum... more Tumor necrosis factor (TNF) alpha inhibitors are effective in the treatment of inflammatory rheumatic diseases. Despite their effectiveness, anti-TNF drugs have some drawbacks such as severe adverse effects including infections and possibly lymphoproliferative disorders. In this report we described a case of juvenile idiopathic arthritis patient who developed thymic enlargement (true thymic hyperplasia), mediastinal lymphadenopathy and pleurisy associated with systemic symptoms under Etanercept treatment. The clinical presentation was highly suggestive of malignancy and the patient underwent diagnostic mediastinoscopy with biopsy.
Osteopoikilosis (OPK) is a rare benign sclerosing disease of the skeleton and inherited as an aut... more Osteopoikilosis (OPK) is a rare benign sclerosing disease of the skeleton and inherited as an autosomal dominant trait. OPK is associated with inflammatory rheumatic disorders, such as rheumatoid arthritis, scleroderma, reactive arthritis and familial Mediterranean fever (FMF). We report a rare case of OPK coexistent with ankylosing spondylitis and FMF. The patient presented multiple sclerotic lesions within and around the sacroiliac joints and a series of radiological diagnostic challenges.
Rheumatic manifestations are the most common extraintestinal findings of inflammatory bowel disea... more Rheumatic manifestations are the most common extraintestinal findings of inflammatory bowel disease (IBD), although there are wide variations among different studies. The only previous Turkish study reported a rather high prevalence of spondyloarthritis (SpA) in patients with IBD. We aimed to determine the frequency of SpA and ankylosing spondylitis (AS) in patients with IBD attending a gastroenterology clinic from a referral centre. The study was conducted in 122 patients with established diagnosis of IBD [28 with Crohn&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s disease (CD) and 94 with ulcerative colitis (UC)]. A detailed medical history was obtained and a complete physical examination was performed in all the patients. Standard pelvic X-rays for examination of the sacroiliac joints were performed only when clinically indicated. The X-rays were read blindly by an experienced rheumatologist and reported according to the established grading system. The modified New York criteria were used to classify AS, and the European Spondyloarthropathy Study Group criteria for SpA. The prevalence of AS and SpA in patients with IBD was 8.2 and 28.7%, respectively. SpA was found to be significantly more common in the patients with CD compared to patients with UC, but the frequency of AS was not different between these two groups. There was no correlation between localisation or extent of the intestinal inflammation and presence of AS and SpA. A higher frequency of women was observed in patients diagnosed as SpA. Almost half of the patients with SpA (45.7%) had not been diagnosed before the study, although they had a history of IBP and/or peripheral arthritis. This study suggests that the prevalences of SpA and AS in Turkish patients with IBD are similar to those in many other populations. There may be a significant female predominance of SpA among patients with IBD.
The aim of this study was to examine the prevalence of hyperuricemia and its associated factors i... more The aim of this study was to examine the prevalence of hyperuricemia and its associated factors in an urban area of Izmir, located in western Turkey. Our study group was selected by computerized sampling from the participants of a larger population-based study searching for the prevalence of rheumatoid arthritis in Balcova and Narlidere districts of Izmir. A total of 132 subjects (69 women and 63 men) were included in this study. Serum uric acid, glucose, creatinine and lipid levels were studied. Body composition along with body fat percentage was determined anthropometrically. A total of 16 subjects had hyperuricemia (4 women and 12 men). The overall prevalence of hyperuricemia was 12.1% and the mean uric acid level was 4.9 +/- 1.3 mg/dl. Males had significantly higher uric acid levels than females (P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.05; 5.5 +/- 1.3 vs. 4.3 +/- 1.1 mg/dl, respectively). The prevalence of hypertension, diabetes, obesity and metabolic syndrome was 24.4, 5.3, 28 and 26.5%, respectively. There was no gouty subject. Sum of skinfold thickness (SFT) measurements and creatinine levels were the independent predictors of hyperuricemia (beta = 0.45, 0.47, respectively). Uric acid measurement is important not only for inflammatory rheumatic disorders but also for predicting metabolic syndrome and related coronary artery disease. There is sex difference in uric acid levels in favor of women most probably explained by gonadal hormones. Hyperuricemia is significantly predicted by anthropometric measure of SFT which is a simple clinical screening method along with creatinine levels.
The objective is to report a case of atypical acute infectious mononucleosis in a juvenile ankylo... more The objective is to report a case of atypical acute infectious mononucleosis in a juvenile ankylosing spondylitis patient who was treated with infliximab. A 20-year-old man was hospitalized for the evaluation of lymphadenopathy and systemic symptoms. His symptoms developed at the eighth week of the infliximab treatment and he required hospitalization. Lymph node biopsy was performed and he was diagnosed as atypical infectious mononucleosis (absence of fever, pharyngitis, lymphocytosis and negative atypical lymphocytosis on blood smear). Infections have become major concerns in patients treated with TNF-blocking agents. In theoretical base, it is not surprising as TNF-alpha has a crucial role in the body&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s defense against both bacterial and viral invasion. Blocking the action of TNF may also change the course of the disease and could lead to a delay in the diagnosis. TNF-alpha-blocking treatment may mask the typical symptoms of infectious mononucleosis and atypical cases should be included in the differential diagnosis of lymphadenopathy in patients receiving anti-TNF-alpha agents.
resolution of the acute-phase response followed. All pharmacological treatments were withdrawn an... more resolution of the acute-phase response followed. All pharmacological treatments were withdrawn and the patient remains well 9 months following surgery. CD is a rare and heterogeneous group of disorders that can mimic systemic CTDs [3]. In childhood, it is usually of the localized type [4]. Patients are frequently asymptomatic but can occasionally present with systemic symptoms [5]. Treatment of unicentric disease of either histological type involves resection, resulting in cure. One caveat to this may be malignant transformation of unicentric CD [6]. Multicentric CD is usually of the plasma cell type and commonly presents with lymphadenopathy, systemic symptoms, organomegaly and acute-phase response [1]. The prognosis of this variant in adults is poor. Moreover, some cases are associated with Kaposi sarcoma and lymphoma [5, 7]. Such malignancies are less commonly associated with CD of the young. Therapy of this form of CD includes immune modulators such as corticosteroids, IFN-and cytotoxic immunosuppression with or without radiotherapy [8]. Monoclonal antibody therapy including anti-CD20 or anti-Il-6 has been used with anecdotal efficacy [1]. A number of recent observations have cast light on the pathogenesis of CD. The levels of IL-6 and other pro-inflammatory cytokines are raised [1]. There also appears to be an association of multicentric CD and HHV-8 infection [1]. This is one of the few cases of paediatric CD with renal involvement described. Previous reports in adult patients describe various forms of glomerular and interstitial pathology and a few cases of nephrotic syndrome caused by renal amyloidosis [9]. In adult patients, there is an emerging experience of 18 F-FDG PET for the diagnosis and subsequent follow-up of CD [10], but no such data in children. In our patient, initial abdominal lymph node biopsy identified only reactive inflammatory changes. Using 18 F-FDG PET/CT we were able to precisely identify the most metabolically active node with confidence, thus guiding the successful surgical management of this case. We conclude with the message that CD should be considered in children presenting with a lupus-like phenotype recalcitrant to cytotoxic immunosuppression.
Objectives: Trichinellosis is a cosmopolitan parasite infection caused by Trichinella nematodes t... more Objectives: Trichinellosis is a cosmopolitan parasite infection caused by Trichinella nematodes that is acquired from consumption of raw meat from several animal species. Knowledge of the clinical pattern and laboratory features of the disease in childhood is limited. The purpose is to study the clinical pattern of trichinellosis caused by Trichinella britovi in children and to compare it in household adults. Methods: We evaluated all children up to 17 years of age and their adult householders exposed to the consumption of infected meat during an outbreak of trichinellosis. A questionnaire was developed to record clinical data. The blood sample was collected for blood count, muscle enzymes, serum electrolytes, albumin and serology. All exposed children were treated with mebendazole, and severe symptomatic patients received prednisolone. Clinical and laboratory presentations and outcome were recorded. To evaluate the clinical picture of trichinellosis in childhood, clinical and laboratory findings were compared between children and household adults with a confirmed diagnosis who consumed the same amount of infected meat. Results: In 47 (62%) of 76 children with suspected trichinellosis, the diagnosis was serologically confirmed. The main clinical and laboratory findings in children were fever, abdominal pain, myalgia, facial and/or eyelid edema, rash, eosinophilia and increased muscular enzymes. The incubation period was similar in children and adults, but myalgia (66% versus 96%, P Ͻ 0.01), facial and/or eyelid edema (57% versus 86%, P Ͻ 0.05), eosinophilia (52% versus 96%, P Ͻ 0.01) and increased serum creatine kinase (38% versus 79%, P Ͻ 0.01) were less common in children than in adults. Seroconversion occurred in fewer children than adults, but the difference was not statistically significant. Conclusions: T. britovi infection shows a benign course and a milder clinical picture in children than in adults who consumed the same amount of infected meat.
The aim of this study was to determine the prevalence of primary Sjogren&#39;s syndrome (pSS)... more The aim of this study was to determine the prevalence of primary Sjogren&#39;s syndrome (pSS) in a general Turkish population according to the latest proposed American-European Consensus Group (AECG) criteria and European-1 (EU-1) criteria. The study was conducted in two districts of Izmir and involved 2835 subjects 20 years of age and older. In the first stage, face-to-face interviews were performed at the registered households. In the second stage, subjects reporting symptoms of both dry eye and dry mouth were invited to the hospital for a full examination, which included Schirmer-1, sialometry and serologic tests. In the third stage, a minor salivary gland biopsy was performed as required. A total of 2887 subjects were contacted and a complete interview was obtained for 2835 (1551 female, 1284 male) subjects. A total of 159 subjects (126 female, 33 male) confirmed oral and ocular dryness, and 86 of these patients (54.1%) underwent a detailed clinical examination in the hospital. pSS was diagnosed in 10 patients (nine females) according to the EU-1 criteria, and in six patients (six females) according to the AECG criteria. We found a minimum crude prevalence of 0.21% [95% confidence interval (CI): 0.03-0.29] in the sample population and an age-sex adjusted prevalence of 0.16% (95% CI: 0.06-0.35), according to AECG criteria. According to EU-1 criteria, these prevalence rates were found to be 0.35% (95% CI: 0.10-0.45) and 0.28% (95% CI: 0.13-0.51) respectively. The pSS prevalence rates found in the Turkish population in this study were lower than the estimated prevalence rate in a general population.
Thyrotoxic hypokalemic periodic paralysis (THPP) is a rare complication of hyperthyroidism and an... more Thyrotoxic hypokalemic periodic paralysis (THPP) is a rare complication of hyperthyroidism and an uncommon form of hypokalemic periodic paralysis. Its differentiation of more common forms of periodic paralysis is important because aggressive treatment can place the patient at risk for rebound hyperkalemia. Treatment of the underlying thyroid dysfunction cures the muscle symptoms. Here we describe a 37-year-old Turkish male with THPP whose paralysis attack recurred soon after administration of radioactive iodine.
Systemic inflammation plays an important role in the development of atherosclerosis (AS). The aim... more Systemic inflammation plays an important role in the development of atherosclerosis (AS). The aim of this study was to evaluate the presence of early AS in patients with familial Mediterranean fever (FMF) that is characterized by recurrent inflammatory attacks of serositis. Sixtyone FMF patients (30 Male/31 Female; 31.5 [18-54] years) and 31 healthy controls (16 Male/15 Female; 31 [22-58] years) were studied. All FMF patients were on regular daily colchicine treatment and during attack-free periods. Both the FMF patients and controls with a history of diabetes mellitus (DM), hypertension, and hyperlipidemia were excluded. Body mass index (BMI) was calculated. Serum lipids, C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR) were assessed. Two-hour oral glucose tolerance test was performed to rule out DM and glucose intolerance. To investigate early AS "endotheliumdependent flow-mediated dilatation (FMD%)," "nitroglyc-erin-induced endothelium-independent peripheral vasodilatation (NTG%)," and intima-media thickness (IMT) of common carotid arteries (CCA) were measured by ultrasonograpy. The median disease duration for FMF patients was 16 (1-45) years. Age, sex, BMI, smoking status, and serum lipids were comparable in patients and controls (p>0.05). However, ESR and standard CRP were significantly higher in the patients group (p<0.05). There were no differences in the measurements of right, left, and averaged IMT of CCA between patients and controls ([0.49 vs 0.5], [0.51 vs 0.52] and [0.5 vs 0.51]; p>0.05, respectively). None of the subjects had carotid artery plaques. FMD% and NTG% were also similar in patients and controls group ([18.2 vs 20.6] and [24.2 vs 22.5]; p>0.05, respectively). This study suggests that the markers of early AS are not impaired in FMF patients on regular daily colchicine treatment. Keywords Arteriosclerosis. Familial mediterranean fever. Ultrasonography Abbreviations AS atherosclerosis BAD brachial artery diameter BMI body mass index CCA common carotid artery CRP C-reactive protein CV cardiovascular DM diabetes mellitus ESR erythrocyte sedimentation rate FMD flow-mediated dilatation FMF familial Mediterranean fever HDL high density lipoprotein HT hypertension IMT intima-media thickness
It is well established that there are people with higher risk of developing acute rheumatic fever... more It is well established that there are people with higher risk of developing acute rheumatic fever (ARF) and rheumatic heart disease (RHD). Mediterranean fever (MEFV) gene mutations might be one of the genetic predisposition factors in the development of ARF/RHD since defect in familial Mediterranean fever (FMF) patients is proposed to be heightened inflammatory response to certain stimuli. Previous clinical observations suggested a relationship between FMF and ARF/RHD. The aim of this study was to investigate the role of the MEFV gene mutations in the susceptibility to RHD in Turkish patients. A total of 100 patients with RHD and 100 healthy controls were included in the study. Diagnosis of RHD was based on echocardiographic findings in which a predominant mitral stenosis was used as an inclusion criterion. Genetic analysis was carried out by sequence analysis investigating two hot spots (exons 2 and 10) for MEFV mutations. Mutation analysis showed that 22 RHD patients (22%) and 24 healthy controls (24%) carried at least one mutated allele. MEFV mutations were identified in 22 of 200 (11%) chromosomes in RHD patients while 26 of the 200 (13%) chromosomes of healthy controls were found to carry a mutated allele. No difference was found in allele frequencies and their distribution between the patients and healthy controls (p = 0.54). MEFV mutations are not associated with a predisposition to develop RHD in adult Turkish patients.
The aim of this study was to compare the effect of chronic inflammation on insulin resistance, se... more The aim of this study was to compare the effect of chronic inflammation on insulin resistance, serum leptin levels, and body composition (BC) in patients with ankylosing spondylitis (AS) and healthy controls. Twentyeight AS patients and 17 healthy controls were included in this study. Subjects with hypertension, diabetes, hyperlipidemia, and obesity were excluded. Acute phase reactants and serum levels of glucose, insulin, lipids, and leptin were studied. BC was determined anthropometrically and by foot-to-foot body fat analyzer (BIA, bioelectrical impedance analysis). Quantitative insulin-sensitivity check index, homeostasis model assessment for insulin resistance, and McAuley indices were calculated. Spinal mobility was assessed by the Bath Ankylosing Spondylitis Metrology Index (BASMI). Patients were also evaluated with the Bath Ankylosing Spondylitis Functional Index and the Bath Ankylosing Spondylitis Disease Activity Index. Age, sex distribution, smoking status, serum lipids, insulin concentrations, and insulin resistance indices were comparable between AS patients and controls (p>0.05). However, acute phase reactants were significantly higher and leptin levels were significantly lower in the AS patients than in controls (p<0.05). Fat percent assessed by both BIA and anthropometrical methods was lower in the male and female AS patients than in controls, and this reduced fat level reached statistical significance for men (p<0.05). There were significant correlations between percent body fat, body mass index, leptin, age, and BASMI (p<0.05; r=0.6, 0.75, 0.35, −0.41, respectively). On the other hand, body fat percent, waist-to-hip ratio, C-reactive protein, and BASMI were significantly correlated with serum leptin levels (p< 0.05; r=0.75, −0.42, −0.52, −0.47, respectively). Chronic inflammatory condition in AS may be responsible for the reduced body fat content and lower circulating leptin concentrations. Insulin levels and insulin resistance indices seem similar in patients and controls in the absence of classic vascular risk factors.
Background: Ankylosing spondylitis (AS) is a chronic inflammatory disease of spine and sacroiliac... more Background: Ankylosing spondylitis (AS) is a chronic inflammatory disease of spine and sacroiliac joints; it is characterized by new bone formation, and the disease processes can be accompanied by osteoporosis. In the present study, we investigated changes in bone mineral density (BMD) and in the levels of various bone turnover-related biomarkers and cytokines in a cohort of AS patients, with regard to clinical parameters, disease activity, and treatment regimen. Methods: 55 AS patients and 33 healthy controls included in the study. Spinal mobility was assessed by the Bath Ankylosing Spondylitis Metrology Index (BASMI), and radiologic changes were scored by the Bath Ankylosing Spondylitis Radiologic Index (BASRI). Patients were also evaluated with the Bath Ankylosing Spondylitis Functional Index (BASFI) and the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI). Bone mineral density (BMD) assessed by dual energy X-ray absorptiometry. Various biomarkers and cytokines of bone turnover including osteoprotegerin (OPG), serum band 5 tartrate-resistant acid phosphatase (TRAP-5), soluble receptor activator of nuclear factor kappa-B ligand (sRANKL), secreted frizzled-related protein 1 (sFRP-1), Dickkopf-related protein 1 (DKK-1), and sclerostin were studied. Results: The levels of TRAP-5, NTX, sRANKL, sclerostin, sFRP-1, DKK-1, and IFNγ, were similar between the patients and controls (p > 0.05), while BMD of femoral neck, and OPG levels were significantly lower in AS patients (p < 0.05). In a subgroup analysis, patients with active disease had significantly higher concentrations of OPG compared with the inactive group. Rest of the biomarkers and cytokines of bone turnover were similar between the active and inactive disease groups. Subgroup analysis of patients receiving anti-TNFα agents and conventional therapy revealed that OPG concentrations were significantly lower in the patients receiving biological drugs, while BAP and DKK-1 were significantly higher in the patients treated with conventional agents. Conclusions: In this cross-sectional study we showed that OPG levels were significantly lower in AS patients compared to healthy subjects. On the other hand, the levels of wingless (Wnt) signal pathway inhibitors seem not altered. Ectopic bone formation in AS may be related to dysfunction of these molecules at the cellular level.
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