Search

As a result of increasing human impact on the natural environment, environmental problems such as natural degradation, climate change, destruction of vegetation, land misuse, and erosion have emerged. The gradual increase in these problems has brought the problem of determining and measuring the human impact on the environment. The Human Influence Index, developed for this purpose, aims to measure the anthropogenic impact on space by using multiple parameters in a defined area. In this study, the intensity and spatial distribution of anthropogenic impact in Denizli province were analyzed by using the Human Influence Index. Analytic Hierarchy Process (AHP), one of the Multi-Criteria Decision Making (MCDM) methods, was used to determine the human impact. As a result of the analysis, 0.3% of Denizli province is under very high, 2% is under high and 4% is under moderate human impact. On the other hand, it was determined that 35% of the province's surface area is under low and 59% is under very low human influence. Consequently, although human influence in Denizli is intense, it has operated in a defined and restricted area.

IntroductionThere have been some significant changes regarding healthcare utilization during the COVID-19 pandemic. Majority of the reports about the impact of the COVID-19 pandemic on diabetes care are from the first wave of the pandemic. We aim to evaluate the potential effects of the COVID-19 pandemic on the severity of diabetic ketoacidosis (DKA) and new onset Type 1 diabetes presenting with DKA, and also evaluate children with DKA and acute COVID-19 infection.MethodsThis is a retrospective multi-center study among 997 children and adolescents with type 1 diabetes who were admitted with DKA to 27 pediatric intensive care units in Turkey between the first year of pandemic and pre-pandemic year.ResultsThe percentage of children with new-onset Type 1 diabetes presenting with DKA was higher during the COVID-19 pandemic (p < 0.0001). The incidence of severe DKA was also higher during the COVID-19 pandemic (p < 0.0001) and also higher among children with new onset Type 1 diabetes (p < 0.0001). HbA1c levels, duration of insulin infusion, and length of PICU stay were significantly higher/longer during the pandemic period. Eleven patients tested positive for SARS-CoV-2, eight were positive for new onset Type 1 diabetes, and nine tested positive for severe DKA at admission.DiscussionThe frequency of new onset of Type 1 diabetes and severe cases among children with DKA during the first year of the COVID-19 pandemic. Furthermore, the cause of the increased severe presentation might be related to restrictions related to the pandemic; however, need to evaluate the potential effects of SARS-CoV-2 on the increased percentage of new onset Type 1 diabetes.

Introduction:Transfusion of blood components, including red blood cells (RBC), platelets and plasma, is the fastest way to improve the haematological state of critically ill patients and can be a lifesaving intervention. Although transfusion is a common practice in paediatric intensive care units (PICUs), its adverse effects are not clearly defined. Therefore, we aimed to evaluate the incidence and predictors of RBC transfusion in mechanically ventilated children.Methods:Data of patients admitted to PICUs from January 2014 to April 2019 were extracted. The mechanically ventilated patients were categorised into two groups, and the relationship between transfusion and prognostic factors was examined.Results:This study investigated 373 children with a mean age of 3.86±4.86 years. Among these children, 239 received RBC transfusion (64.1%). Inotropic drug usage, acute kidney injury, continuous renal replacement therapy (CRRT), mortality, length of mechanical ventilation, and duration of PICU stay were associated with RBC transfusion. Logistic regression analysis revealed that inotropic drug usage increased the RBC transfusion requirement by 5.168 fold, and CRRT increased it by 2.804 fold.Conclusion:Blood transfusion is an associated adversity in critically ill children. Therefore, patients who need transfusion should be followed up more carefully and closely.

Background and objective: Severe sepsis and septic shock are life-threatening organ dysfunctions and causes of death in critically ill patients. The therapeutic goal of the management of sepsis is restoring balance to the immune system and fluid balance. Continuous renal replacement therapy (CRRT) is recommended in septic patients, and it may improve outcomes in patients with severe sepsis or septic shock. Therapeutic plasma exchange (TPE) is another extracorporeal procedure that can improve organ function by decreasing inflammatory and anti-fibrinolytic mediators and correcting haemostasis by replenishing anticoagulant proteins. However, research about sepsis and CRRT and TPE in children has been insufficient and incomplete. Therefore, we investigated the reliability and efficacy of extracorporeal therapies in paediatric patients with severe sepsis or septic shock. Materials and methods: We performed a multicentre retrospective study using data from all patients aged Results: Between January 2014 and April 2019, 168 septic patients were enrolled in the present study. Of them, 47 (27.9%) patients underwent CRRT and 24 underwent TPE. In patients with severe sepsis, the requirement for CRRT was statistically associated with mortality (p < 0.001). In contrast, the requirement for TPE was not associated with mortality (p = 0.124). Conclusion: Our findings revealed that the requirement for CRRT in patients with severe sepsis is predictive of increased mortality. CRRT and TPE can be useful techniques in critically ill children with severe sepsis. However, our results did not show a decrease of mortality with CRRT and TPE.

Children in paediatric intensive care units (PICUs) are vulnerable to infections because invasive devices are frequently used during their admission. We aimed to determine the prevalence, associated factors, and prognosis of infections in our PICU. This retrospective study evaluated culture results from 477 paediatric patients who were treated in the PICU between January 2014 and March 2019. Ninety patients (18.9%) had bacterial infections, with gram-negative bacteria being the predominant infectious agents. Culture-positive patients were younger than culture-negative patients, and age was related to mortality and various clinical factors. Culture-positive bacterial infections in the PICU were associated with increased use of invasive mechanical ventilation (odds ratio(OR); 2.254), red blood cell (RBC) transfusions (OR:2.624), and inotropic drugs (OR:2.262). Carbapenem resistance was found in approximately one-third of gram-negative bacteria, and was most common in tracheal aspirate specimens and cases involving Klebsiella spp. Total parenteral nutrition was a significant risk factor (OR:5.870). Positive blood culture results were associated with poorer patient survival than other culture results. These findings indicate that infections, especially those involving carbapenem-resistant bacteria, are an important issue when treating critically ill children.

In the past decade, multidrug-resistant (MDR) gram-negative bacteria have become a major problem, especially for patients in intensive care units. Recently, colistin became the last resort therapy for MDR gram-negative bacteria infections. However, nebulised colistin use was limited to adult patients. Thus, we investigated the efficacy and safety of nebulised colistin treatment against MDR microorganisms in the paediatric intensive care unit (PICU). Data of all patients admitted for various critical illnesses (January 2016 to January 2019) were reviewed. Differences between groups (with and without a history of nebulised colistin) were compared. Of 330 patients, 23 (6.97%) used nebulised colistin. Significant relationships were found between nebulised colistin usage and several prognostic factors (inotropic drug use (p = 0.009), non-invasive mechanical ventilation (p ≤ 0.001), duration in PICU (p ≤ 0.001), and C-reactive protein level (p = 0.003)). The most common microorganism in tracheal aspirate and sputum cultures was Pseudomonas aeruginosa (13 patients). The most common underlying diagnosis was cystic fibrosis, noted in 6 patients. No serious nephrotoxicity and neurotoxicity occurred. This study showed that colistin can be safely used directly in the airway of critically ill children. However, nebulised colistin use did not have a positive effect on mortality and prognosis.

Toplumun en temel birimi, yapı taşı olarak kabul edilen aile, evrensel bir kurum olmakla birlikte toplumlara, kültürlere ve dönemlere göre farklılık gösterebilmektedir. Özellikle günümüzün hızla değişen, dönüşen toplumlarında aile kurumu bu değişim sürecinden oldukça etkilenmektedir. Sosyolojik alan yazında sıklıkla ailenin toplumsal bir kurum olma özelliğine vurgu yapılmakta ve değişen, dönüşen, farklılaşan işlevleri tartışılmaktadır. Toplumsal bir kurum olarak ailenin en önemli işlevlerinden biri hiç şüphesiz topluma yeni katılan üyelere, toplumun değer ve normları öğretilerek, aktarılarak onların toplumsallaşmasını sağlamaktır. Bu süreçte aile yapısı içerisinde edinilmesi beklenen değerlerden biri de bireylerin kadın ya da erkek olmaları nedeniyle nasıl davranmaları gerektiğinin sınırlarını çizen toplumsal cinsiyet rolleridir. Alan yazında toplumsal cinsiyet rollerinin özellikle toplumsal yaşam, aile yapısı ve evlilik yaşamına ilişkin rollerde farklılıklar gösterdiğinin altı çizilmektedir. Toplumsal yaşama ilişkin tüm bu unsurlar ve değişim boyutları, toplumsal yaşamın bir yansıması olarak karşımıza çıkan edebi eserlerde sıklıkla işlenen, vurgulanan konular arasında yer almaktadır. Edebi eser türlerinden ilk akla gelen roman türünde toplumsal yaşamda bireylerin ya da ailelerin başından geçen ya da geçme ihtimali olan olaylar aktarılırken toplumsal yapıya ilişkin sosyo-kültürel değerlerin yansıtıldığı görülmektedir. Buradan hareketle çalışmada, Ayşe Kulin’in kaleme aldığı ve 1997 yılında yayımlanan “Adı: Aylin” adlı biyografik romanı, Türkiye’deki sosyo-kültürel değişmelerin özellikle aile yapısı, evlilik, kadın ve toplumsal cinsiyet rolleri bağlamında değerlendirilmesi amacıyla içerik açısından analiz edilerek, nitel bir çalışma gerçekleştirilmektedir. Romanda Aylin karakteri geleneksel sınırlar içerisinde modern bir yaşam tarzı sürdürmeye çalışırken hem özel hem de kamusal alanda görünür olma çabasıyla sosyo-kültürel değişme sürecinde özellikle kadınların farklılaşan, çeşitlenen toplumsal cinsiyet rolleri konusuna gönderme yapmaktadır.

Tocilizumab (TCZ) which is a humanized interleukin (IL)-6 receptor antibody has been increasingly widespread used in rheumatology practice. TCZ-related hypofibrinogenemia is a not well-described side effect, but awareness seems to be incresing as publications on this topic are also becoming more frequent. Our aim in this study was to determine the frequency, timing, and approach to TCZ-related hypofibrinogenemia in rheumatic diseases. We retrospectively screened our patients who received TCZ for inflammatory rheumatic diseases and studied serum fibrinogen at least once before and/or after drug administration. We recorded and analyzed demographic features (age, gender, diagnosis), comorbidities, laboratory parameters, management, and outcome data. 30 patients who received TCZ due to rheumatological diseases and had at least one fibrinogen level were included in this study. 73.3% were female and median age was found to be 65 (42-82) years, with median disease duration of 148 (36-500) months. 90% of the patients received TCZ for RA and 10% for GCA. We examined the fibrogen levels at an average of 24 months (min 1 max 108) following the start of TCZ treatment. The study group was divided into those with normal fibrinogen levels (normal fibrinogen group) and those with low fibrinogen levels (lowfibrinogen group). In total, hypofibrinogenemia was found in 14 (46.6%) patients. Median serum fibrinogen level in the low fibrinogen group after TCZ was calculated to be 151.5 mg/dl (min 92 max 171). Most patients were asymptomatic (71.4%). Ecchymoses were in seen 4 (28,6%) patients. No major bleeding were seen. TCZ was discontinued in 5 out of 14 patients (35.7%), while 9 out of 14 patients (64.3%) were closely followed. Outcomes regarding fibrinogen levels (11 out of 14) were as follows: increase in 4 and not checked in one after cessation of the drug, 3 spontaneous increases under the drug, and 3 persistently low levels on TCZ treatment. We found no difference in terms of gender (p = 0.417), platelets (p = 0.343), ESR (p = 0.448), and CRP (p = 0.660) at the time of TCZ initiation between groups. TCZ-related hypofibrinogenemia is more frequent than expected with occurence in both the early and late stages of treatment. Further research is required to determine whether to regularly measure fibrinogen levels in patients using TCZ and how to treat patients with hypofibrinogenemia., Competing Interests: Declarations. Ethical approval: This study was approved by the ethics committee of Eskisehir Osmangazi University with decision number of 06, decision dated July 13, 2021. All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Declaration of Helsinki and its later amendments or comparable ethical standards. For this type of retrospective study formal written informed consent is not required. Previous related congress abstract publication: The preliminary results of this study was submitted to EULAR 2024 Congress held in Vienna in June 2024 and accepted as an abstract ( https://doi.org/10.1136/annrheumdis-2024-eular.4093 ). Disclaimer: Neither an AI nor an editing software was employed in the process of writing and editing this manuscript. Conflict of interest: The authors declare that there is no conflict of interest., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Dermatomyositis (DM) is an idiopathic inflammatory myositis (IIM) characterized by skin manifestations and muscle involvement. Spontaneous intramuscular hemorrhage (SIH) is a fatal complication that is very rare in the course of DM, but not well known to rheumatologists. Our aim was to determine the frequency and possible risk factors of DM-related SIH. A retrospective analysis was conducted on a cohort of DM patients who were observed in the rheumatology department of the university hospital between 1998 and January 2024. The clinical, laboratory, radiological data of the patients and the treatments they received during the follow-up were analyzed. To determine possible risk factors for the development of SIH in the course of DM, our patients with DM were analyzed together with other rare SIH cases in the literature. The study included 42 of our DM patients. 32 of the patients (76.2%) were female. The median age of the patients was 53 (24-82) years, the median age of DM diagnosis of the patients was 47 (18-75) years, and the median duration of DM of the patients was 36 (2-276) months. 7.1% of patients had dysphagia, and 16.7% had intertitial lung disease (ILD). 5 (11.9%) patients were diagnosed with malignancy. The incidence rate of SIH development in our DM cohort was 0.238/100 patient years (95% CI 0.006-1.256). We tried to identify independent risk factors for SIH development by comparing our 41 DM patients without SIH with the data of patients with 23 DM-related SIH collected from the literature by adding our 1 patient (24 pts). Male sex (OR 4.97, 95% CI 1.66-14.92, p = 0.003), ILD presence (OR 9.71, 95% CI 2.99-31.47, p < 0.001), anti-MDA5 positivity (OR 16.0, 95% CI 1.60-159.3, p = 0.006), anti-Ro52 positivity (OR 11.6, 95% CI 2.93-46.34, p < 0.001), heparin use (OR 4.42, 95% CI 2.68-7.24, p < 0.001), intravenous immunoglobulin (IVIG) use (OR 11.7, 95% CI 2.26-60.54, p < 0.001), and steroid dose (OR 1.03, 95% CI 1.00-1.05, p = 0.005) were identified as risk factors for the development of SIH in the univariate analysis. The death rate due to hemorrhage was 50%. No single risk factor was found to be associated with death. As a result, SIH may occasionally arise in patients with DM. Rheumatologists should be aware that patients with dysphagia and/or ILD, who are on heparin, getting high doses of steroids, and test positive for anti-MDA5 and/or anti-Ro52 antibodies may develop SIH in the early stages of DM., (© 2024. The Author(s).)

The objective of this study was to assess the pregnancy outcomes in a cohort of patients who experienced pregnancies before and/or after being diagnosed with Takayasu's arteritis (TA). The present investigation encompassed a total of 88 pregnancies seen in a cohort of 35 patients who met the criteria outlined by the American College of Rheumatology in 1990 for the classification of Takayasu arteritis (TA). Pregnancies were classified into two categories. 1. Pregnancies that occurred before the diagnosis (pre-d or pre-TA) 2. Pregnancies that happened following a diagnosis (post-d or post-TA). Fifty-nine pregnancies (67.0%) occurred in 21 TA patients before the diagnosis with and a complication rate of 15.2%, and twenty-nine pregnancies (33.0%) occurred in 14 patients concomitant with or after TA diagnosis and complication rate 100%. Although the hypertension rate was higher in the pre-d group than in the post-d group, it was not significant (32.2% vs. 10.3%, p = 0.160). However, preeclampsia (20.6% vs. 0%, p = 0.001), low birth weight (27.5% vs. 1.6%, p = 0.001), and prematurity (24.1% vs. 1.6%, p = 0.035) were observed more frequently in the post-d group compared to the pre-d group. The frequency of abortions and in-utero deaths were similar in both groups (p > 0.05). Patients with hypertension had significantly higher rates of preeclampsia (p = 0.003), preterm birth (p = 0.036), low birth weight (p = 0.250), abortion (p = 0.018), in utero death (p = 0.128), and cesarean section (p = 0.005) than those without hypertension. Renal artery involvement was detected in 15 (42.8%) patients. All patients with renal artery involvement had hypertension, and they had significantly more pregnancy complications than the other group (p = 0.001). TA negatively affects pregnancy outcomes. A good control of arterial hypertension before conception and during pregnancy is critical to improve both maternal and fetal outcomes. In addition, detecting renal artery stenosis before pregnancy is important in reducing possible negative pregnancy outcomes., (© 2024. The Author(s).)

Pulmonary hypertension (PH) is a clinical condition characterized by increased pulmonary arterial pressure arising from a heterogeneous range of diseases that has a deteriorating effect on the quality of life and may cause early mortality if left untreated. Connective tissue disorders (CTD)-associated PH is the second most common cause of pulmonary arterial hypertension (PAH), after the idiopathic form, categorized as group I. Systemic scleroderma (SSc) accounts for 75% of CTD-associated PH cases. Although SSc ranks first place for CTD-associated PH, SSc is followed by systemic lupus erythematosus (SLE) and mixed connective tissue disease (MCTD), having a lesser frequency of PH occurrence, while it occurs as a rare complication in cases with rheumatoid arthritis (RA) and inflammatory myositis. PH may also occur during non-SSc CTDs and even other rheumatic diseases, including Behcet's disease and adult-onset Still's disease, albeit to a lesser extent. The prognosis of CTD-associated PH is worse than the other forms of PH. Although, as in idiopathic pulmonary arterial hypertension (IPAH), the mechanism of CTD-related PH is associated with an increase in vasoconstrictors like endothelin-1 and a decrease in vasodilators like prostacyclin and nitric oxide production, inflammation, and autoimmune mechanisms also play a role in the development and progression of PH. This may lead to the involvement of more than one mechanism in CTD-associated PH. Knowing which mechanism is dominant is very important in determining the treatment option. This review will primarily focus on the epidemiology, risk factors, and prognosis of PH that develops during rheumatic diseases; the pathogenesis and treatment will be briefly mentioned in light of the newly published guidelines. Key Points • Pulmonary arterial hypertension (PAH) associated with connective tissue disease (CTD) in Western countries is the second most common type of PAH after idiopathic PAH (IPAH). • CTD-PH can be seen most often in systemic scleroderma (SSc), less in systemic lupus erythematosus (SLE), mixed CTD (MCTD), and rarely in other CTDs. • While current guidelines recommend annual transthoracic echocardiography as a screening test for asymptomatic SSc patients, screening for PH is not advised in the absence of symptoms suggestive of PH in other CTDs. • CTD-PH treatment can be divided into specific vasodilator PH treatments and immunosuppressive therapy. Current treatment guidelines recommend the same treatment algorithm for patients with CTD-associated PH as for patients with IPAH. Several case series have shown the beneficial effect of immunosuppressive agents in patients with SLE-PH and MCTD-PH., (© 2022. International League of Associations for Rheumatology (ILAR).)

Objectives: We aimed to compare the spectrum and severity of COVID-19 and vaccine breakthrough infections (BIs) among patients with IIMs, other systemic autoimmune and inflammatory diseases (SAIDs), and healthy controls (HCs)., Methods: This is a cross-sectional study with data from the COVAD study, a self-reported online global survey that collected demographics, COVID-19 history, and vaccination details from April to September 2021. Adult patients with at least one COVID-19 vaccine dose were included. BIs were defined as infections occurring > 2 weeks after any dose of vaccine. Characteristics associated with BI were analyzed with a multivariate regression analysis., Results: Among 10,900 respondents [42 (30-55) years, 74%-females, 45%-Caucasians] HCs were (47%), SAIDs (42%) and IIMs (11%). Patients with IIMs reported fewer COVID-19 cases before vaccination (6.2%-IIM vs 10.5%-SAIDs vs 14.6%-HC; OR = 0.6, 95% CI 0.4-0.8, and OR = 0.3, 95% CI 0.2-0.5, respectively). BIs were uncommon (1.4%-IIM; 1.9%-SAIDs; 3.2%-HC) and occurred in 17 IIM patients, 13 of whom were on immunosuppressants, and 3(18%) required hospitalization. All-cause hospitalization was higher in patients with IIM compared to HCs [23 (30%) vs 59 (8%), OR = 2.5, 95% CI 1.2-5.1 before vaccination, and 3 (18%) vs 9 (5%), OR = 2.6, 95% CI 1.3-5.3 in BI]. In a multivariate regression analysis, age 30-60 years was associated with a lower odds of BI (OR = 0.7, 95% CI 0.5-1.0), while the use of immunosuppressants had a higher odds of BI (OR = 1.6, 95% CI 1.1-2.7)., Conclusions: Patients with IIMs reported fewer COVID-19 cases than HCs and other SAIDs, but had higher odds of all-cause hospitalization from COVID-19 than HCs. BIs were associated with the use of immunosuppressants and were uncommon in IIMs., (© 2022. The Author(s).)

Background: It is important to determine the correlation of the CO-RADS classification and computed tomography (CT) patterns of the lung with laboratory data. To investigate the relationship of CO-RADS categories and CT patterns with laboratory data in patients with a positive RT-PCR test. We also developed a structured total CT scoring system and investigated its correlation with the total CT scoring system., Method: The CT examinations of the patients were evaluated in terms of the CO-RADS classification, pattern groups and total CT score. Structured total CT score values were obtained by including the total CT score values and pattern values in a regression analysis. The CT data were compared according to the laboratory data., Results: A total of 198 patients were evaluated. There were significant differences between the CO-RADS groups in terms of age, ICU transfer, oxygen saturation, creatinine, LDH, D-dimer, high-sensitivity cardiac troponin-T (hs-TnT), CRP, structured total CT score values, and total CT score values. A significant difference was also observed between the CT pattern groups and oxygen saturation, creatinine and CRP values. When the structured total CT score values and total CT score values were compared they were observed to be correlated., Conclusions: Creatinine can be considered as an important marker for the CO-RADS and pattern classifications in lung involvement. LDH can be considered as an important marker of parenchymal involvement, especially bilateral and diffuse involvement. The structured total CT scoring system is a new system that can be used as an alternative., (© 2022. The Author(s).)

Familial Mediterranean fever (FMF) is a monogenic autoinflammatory disease characterized by recurrent episodes of fever and serositis. Colchicine (Col) has a crucial role in the prevention of amyloidosis and FMF attacks. The effect of Col on innate immune cells is based on the inhibition of the microtubule system. The microtubule system is also very important for neurosecretory functions. The inhibitory effect of Col on neurosecretory functions is an overlooked issue. Considering that the neuroimmune cross-talk process plays a role in the development of inflammatory diseases, the effect of Col on the neuronal system becomes important. FMF attacks are related to emotional stress. Therefore, the effect of Col on stress mediators is taken into consideration. In this hypothetical review, we discuss the possible effects of Col on the central nervous systems (CNS) and peripheral nervous systems (PNS) in light of mostly experimental study findings using animal models. Studies to be carried out on this subject will shed light on the pathogenesis of FMF attacks and the other possible mechanisms of action of Col apart from the anti-inflammatory features., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Korkmaz, Cansu and Cansu.)

Rheumatoid arthritis (RA) is a chronic autoimmune disease characterized by articular and extra-articular manifestations. Among extra-articular involvement, rheumatoid meningitis (RM) is a rare condition, which may exhibit variable symptoms including headache, focal and/or generalized neurologic deficits. It may develop as the preceding manifestation of RA or occur at any time of the disease course. Some drugs used for the treatment of RA may give rise to aseptic meningitis or create a tendency to infectious meningitis due to their immunosuppressive effect. All these possibilities may lead to difficulties in the differential diagnosis. Achieving a diagnosis in a short time is crucial in terms of prognosis. Here, we would like to report a case with longstanding RA manifested by left-sided weakness and seizure shortly after initiating etanercept (ETA) therapy. ETA-induced meningitis was confirmed with appropriate diagnostic tools. Our aim with this case-based review is to attract the attention of this rare condition and discuss diagnostic challenges., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH, DE part of Springer Nature.)

Competing Interests: The authors declare no conflict of interest.

Amyloidosis is described by the deposition of misfolded proteins in the tissues. Amyloidoses are classified into two as systemic and localized. Out of the systemic forms, AL (light chain) amyloidosis is the most prevalent type; however, amyloid A (AA) amyloidosis is more frequently encountered in the rheumatology practice. AA amyloidosis stands out as a major complication of familial Mediterranean fever (FMF). Splenic and renal involvement is more likely in FMF-associated systemic amyloidosis. The involvement of thyroid and adrenal glands has also been described, although infrequently. Amyloidoses have a heterogeneous plethora of clinical manifestations, with certain phenotypes associated with specific amyloid forms. Gynecological amyloidosis is a rare condition. Uterine involvement may occur in a localized fashion or may also arise as a part of systemic involvement, albeit at a lesser ratio. Several cases of uterine AL amyloidosis have been documented so far as an organ involvement in systemic AL amyloidosis. On the other hand, uterine amyloidosis associated with AA amyloidosis has been described merely in one case with rheumatoid arthritis (RA). Here, we presented a 40-year-old female patient with FMF known for 38 years who underwent splenectomy and hysterectomy due to massive splenomegaly, deep anemia, and persistent menometrorrhagia. Histological examinations of materials revealed uterine and splenic AA amyloidosis. This case report is first-of-its-kind to describe FMF-associated uterine AA amyloidosis and also provides a discussion of possible mechanisms of amyloidosis-induced uterine bleeding.

Objectives: Immunoglobulin (Ig) A vasculitis affects children more commonly than adults and previous literature lacks any formal damage assessment. Our aim in this study is to investigate the disease course, relapse rates and prognostic factors in adult patients with IgA vasculitis and to evaluate the disease-related damage., Methods: We assembled a retrospective cohort of adult IgA vasculitis from six tertiary Rheumatology Centres in Turkey. The demographics, clinical characteristics, treatment and outcomes of patients were abstracted from medical records., Results: The study included 130 (male/female: 85/45) patients and the mean age was 42.2±17 years. Cutaneous manifestations and arthritis/arthralgia were the most common clinical manifestations. One hundred thirteen patients (86.9%) were treated with oral glucocorticoids (GC). As additional immunosuppressive (IS) agents, azathioprine was given to 44 (34.9%) and pulse cyclophosphamide to 18 (12.6%) patients. Seventy-nine patients (60%) had follow-up of median 15 (IQR 7-40) months. Twelve (15%) patients relapsed during follow-up. The mean VDI score was 0.4 in the last visit. Nineteen (24.7%) patients had at least one damage item at the end of follow-up. Most frequent damage items were renal 11 (42%), ocular 4 (15%) and cardiovascular 4 (15%)., Conclusions: In this cohort the most frequent damage item was renal and was related to the disease itself. Damage score was higher in patients with more severe disease and treated more aggressively. Our results suggest that more effective treatment options are needed in a subgroup of patients with IgA vasculitis to prevent the damage related with the vasculitis, especially with more severe disease.

Objectives: To evaluate differences between the patients with familial Mediterranean fever (FMF) with homozygous (Hom), heterozygous (Het) and compound heterozygous (cHet) MEFV mutations in terms of clinical features and severity of the disease, as well as frequency of concomitant disorders, without focusing on Exon 10 mutations., Methods: The patients with FMF were diagnosed using the Tel-Hashomer diagnostic criteria. The presence of MEFV mutations was investigated in exons 2,3,5 and 10 by multiplex-PCR reverse hybridisation method. All the patients were questioned for the presence of concurrent disorders, and the medical records of these patients were revised retrospectively., Results: 259 unrelated patients (female: 143, male: 116; mean age: 33.5±12 years) were included in this study. Hom and Het mutations were found in 79 (31.9%) and 88 (35.6%) patients with FMF, respectively. cHet mutations were found in 68 (27.5%) FMF patients. Early onset and early diagnosis of FMF were found in Hom group compared to Het and compound Het groups. The number of the patients with a higher severity score was significantly higher in Hom group (n=40, 50.6%) than Het (n=12, 13.6%) and cHet groups (n=10, 14.7%), (p<0.0001). No significant differences were found between the groups in terms of clinical features, except for erysipelas like erythema (ELE) (Hom group: 69.6% vs. Het group 37.5%, p<0.0001). Amyloidosis and concomitant disorders were found in 22 FMF patients with Hom MEFV mutations, 16 FMF patients with heterozygous mutations, 7 FMF patients with cHet mutations., Conclusions: While the presence of homozygous mutations creates tendency for a severe disease phenotype, the development of concomitant disorders seems to be independent of homozygous mutations.

Hematologic involvement or hematologic malignancies are frequently encountered during the course of rheumatic diseases. Bone marrow (BM) aspiration and/or biopsy examinations may have a diagnostic role in explaining hematologic findings detected in rheumatology clinical practice. Our aim was to describe the indications for BM examinations and to share our BM aspiration/biopsy results. We analyzed 140 BM aspiration/biopsy results of patients conducted at the Department of Rheumatology from 2010 to 2018. Demographics, complete blood count (CBC), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) values, serum biochemistry test results including lactate dehydrogenase (LDH), organomegaly, indications for BM examinations and BM examination results for each patient, and mortality rates were recorded. Out of the 140 patients, 63.6% were female, and the median (Q1-Q3) age was 53 (39.5-65) years. One hundred fifteen (82.1%) patients were diagnosed as having primary rheumatic disease, and 25 (17.8%) were admitted due to musculoskeletal symptoms. Rheumatoid arthritis (RA) (n = 34, [29.5%]), and systemic lupus erythematosus (SLE) (n = 21, [18.2%]) were the most common rheumatic diseases. Cytopenia was the most common indication for BM aspiration/biopsy (n = 83, [59.3%]). Thirty-nine (47%) of 83 patients had drug-induced cytopenia. A pathology was detected in 40 (28.5%) of the 140 BM examinations. Patients with pathologic BM results had either a hematologic malignancy (n = 38, [95%]) or metastasis to a solid organ (n = 2, [5%]). The group of patients with pathologic BM biopsy results had significantly higher rates of lymphadenopathy, splenomegaly, and monoclonal gammopathy compared with the group with non-pathologic results (p = 0.001, p = 0.011, and p = 0.023, respectively). Likewise, LDH concentrations of those with pathologic results were higher than in patients with non-pathologic results [737 (range 577-1420) IU/L vs. 541 (range 306-840) IU/L, p = 0.019]. In this study, cytopenia or CBC abnormalities accompanied by elevated LDH values or anemia along with increased ESR were the most common indications for BM aspiration/biopsy. Further prospective studies are needed to determine the indications of BM aspiration/biopsy and establish the parameters that predict abnormal BM results in rheumatology practice.

Objective: Corticosteroids may cause hyperglycemia and diabetes mellitus (DM). Development of DM during long-term steroid use has been well studied; however, data regarding the short-term effects of steroid therapy are scarce. In this study, we aimed to detect the actual time of short-term steroid-induced hyperglycemia in patients without previous impaired glucose metabolism, and the ideal time (which day and in relation to meals) of glucose measurement., Methods: The 7-point blood glucose (BG) measurements of patients who were commenced moderate to high-dose steroids (≥15 mg/day prednisolone or its equivalent) due to rheumatological diseases during the first 5 days of steroid therapy were recorded. Fasting BG ≥ 7 mmol/L (126 mg/dL) or random BG ≥ 11.1 mmol/L (200 mg/dL) were considered as overt DM in accordance with the 2016 American Diabetes Association guideline, and post-meal BG ≥10 mmol/L (180 mg/dL) was considered as steroid-induced hyperglycemia., Results: Fifteen males (mean age: 44 ± 16 years) and 35 females (mean age: 41 ± 12 years) were recruited to the study. One thousand seven hundred fifty fasting, pre-meal, and 2-hours post-meal BG concentrations were analyzed. Twenty-one (42%) patients developed steroid-induced DM and 39 (78%) developed steroid-induced hyperglycemia. The highest glucose concentrations were detected on the 3rd day of steroid therapy and 2-h after meals (p < .0001)., Conclusion: Intermediate to high-dose steroid therapy causes hyperglycemia after lunch and dinner on the 3rd day of treatment. This time period should be taken into consideration in the detection and treatment of steroid-induced hyperglycemia., (Copyright © 2019 The Canadian Society of Clinical Chemists. Published by Elsevier Inc. All rights reserved.)

Low-dose methotrexate (ld-MTX) that is administered during rheumatoid arthritis (RA) treatment has hematological adverse effects such as pancytopenia, although rare. Although well-established and widely used for hematological adverse effects caused by high-dose MTX, leucovorin (folinic acid) treatment does not have an agreed-upon administration for ld-MTX-induced pancytopenia. Here, we aimed to figure out whether there was any difference in response time between the regimens with and without folinic acid prescribed to our patients who developed pancytopenia while on MTX therapy, and to identify risk factors for its development. Our cases were collectively assessed together with other rare cases available in the literature that were reported in a similar manner with an explicitly indicated response time, in days. Thereupon, we looked for any difference in response time between the regimens with and without folinic acid. In total, ten of our patients experienced pancytopenia while on ld-MTX treatment. Mean day on which hematological response was achieved was as follows: 7 days in one patient on folic acid monotherapy, 6 days in three patients on granulocyte-colony stimulating factor (G-CSF) monotherapy, 4.5 days in two patients on leucovorin monotherapy, and 4 days in the remaining three patients who were treated with G-CSF + folinic acid/leucovorin. When we collectively evaluated our patients and the patients with an explicitly stated response duration in the literature (15 patients) and compared regimens including folinic acid to those without folinic acid, duration until response/recovery from pancytopenia was significantly shorter in folinic acid group than that in the group without folinic acid (5.47 ± 2.9 days vs 10 ± 3.77 days, p = 0.002). Treatment modalities including folinic acid (leucovorin) either with or without G-CSF result in a shorter recovery/response time compared to other agents. Leucovorin should definitely be considered and applied in rescue therapy for ld-MTX-associated side effects.

Objectives: To investigate whether there is a difference between male and female patients with Behçet's disease (BD) in terms of hypercoagulability by using modified rotational thromboelastograhic (ROTEM) analysis., Methods: 126 BD patients (71 male, 55 female; mean age: 41±9 yrs) who met ISSG criteria for BD were included into the study. 23 patients with vasculitis (16 female, 7 male; mean age 49±16 yrs), and 25 healthy individuals (11 female, 14 male; mean age: 37±10 yrs) were included to the study as disease and healthy control (HC) group, respectively. Clotting time (CT), clot formation time (CFT) and maximum clot firmness (MCF) were determined by INTEM and EXTEM analyses. As a marker of vascular endothelial injury, along with inflammatory markers, vWFag levels were investigated in patients and HC group., Results: Extem-CFT was shorter in only vasculitic group compared to HC group. Intem-CFT was found to be shorter in BD patients and vasculitis group compared to HC. Intem-MCF was significantly longer in male BD patients than female BD patients. Extem-CFT was found to be shorter in male BD patients compared to female BD patients. Extem-MCF was statistically longer in male BD patients. In inactive male BD patients, while Intem-CFT was shorter than HC individuals, Intem-MCF and Extem-MCF were statistically longer than HC (p<0.02, p<0.03), respectively. However, no significant differences were found between inactive female BD patients and HC in terms of all ROTEM parameters., Conclusions: These results support that male BD patients have a hypercoagulable state compared to female BD patients, which may explain why male patients are prone to thrombotic complications.

The higher incidence of arterial and venous events is well established in patients with rheumatoid arthritis (RA). Our aim here was to investigate whether there is a prothrombotic state in RA patients by using rotational thromboelastometry (ROTEM) method and to demonstrate whether the disease variables play a role in this process. A total of 85 patients who met the 2010 RA classification criteria were consecutively included in the study. The patients with RA who have been using antiaggregant, anticoagulant, or nonsteroidal anti-inflammatory drugs (NSAIDs) and had a history of arterial or venous thromboembolism were excluded from the study. Their complete blood count (CBC), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), fibrinogen, D-dimer, and lipid profiles were measured, DAS-28 disease activation scores were calculated, and simultaneous ROTEM analysis was performed to determine the predisposition to thrombosis. Of the ROTEM parameters, clotting time (CT, seconds (s)), clot formation time (CFT, s), and maximum clot firmness (MCF) were evaluated. Having a shorter CT and/or CFT in intrinsic (I) or extrinsic (E) pathway and/or a longer MCF compared to the healthy controls was considered as "predisposition to hypercoagulability". The mean age of the 85 RA patients were 54.12 ± 13 years, and 77.6% of the patients were female (n = 66). Of the patients, 52.9% (n = 45) were using methotrexate (MTX) ± hydroxychloroquine (HCQ) ± corticosteroid (CS), while 43.5% (n = 37) were using anti-tumor necrosis factor (TNF) ± MTX. Active steroid usage was ongoing in 64.7% of the patients (n = 55). When evaluated according to DAS-28, in those with higher disease activity, a shorter I-CFT and greater I-MCF were determined (p = 0.020 and p = 0.033, respectively). In those with higher disease activity based on the correlation analysis, I-CFT and E-CFT were shorter and I-MCF and E-MCF were longer, indicating a higher predisposition to thrombosis. Using linear regression, variables with a major effect on ROTEM parameters were identified as DAS-28, CRP, and platelet count. As the first study in the literature, we identified that disease activation is the most important risk factor for prothrombotic state in RA patients irrespective of the drugs used. ROTEM can be used in clinical practice to predict thrombotic events in RA patients.

Granulomatosis with polyangiitis (GPA) involves upper and lower respiratory tracts and kidneys. Lung involvement is among the most important organ involvements in GPA. GPA's lung involvement might be confused with other granulomatous conditions with lung involvement. In this report, we presented clinical features of two cases with GPA who had been diagnosed as tuberculosis (TBC) and well treated with anti-tuberculosis (anti-TBC) drugs. However, one of two cases had ear-nose-throat (ENT) manifestations before the diagnosis of TBC and her extrapulmonary findings related with GPA have added to clinical features in the following years. In the second case, the manifestations of GPA appeared after 13 months of anti-TBC treatment. We speculated that lung involvement in these cases may be due to GPA rather than TBC. Our aim was to highlight difficulties in the differential diagnosis between GPA and TBC and suggest the possible beneficial effect of anti-TBC drugs on the lung involvement due to GPA in light of the literature data.

Ankylosing spondylitis (AS) affects sacroiliac and axial joints as well as extraarticular organs, such as the eye, lung, bowel, and heart. Although examples of renal involvement in AS, such as IgA nephropathy, amyloidosis, and glomerulonephritis, have been reported, it has not been emphasized that urolithiasis is frequently formed in the clinical course of AS. Growing evidence indicates that urolithiasis may be observed in AS patients and is more frequent than other extraarticular features. In this review, we will discuss frequency and predictors of AS-related urolithiasis and summarize the possible underlying genetic and biochemical mechanisms. We believe an increased awareness of urolithiasis as a complication of AS will encourage future studies that will shed light on disease mechanisms and preventative therapies.

In rheumatology practice, anti-tumor necrosis factor (TNF) alpha agents are frequently used medications, more so in ankylosing spondylitis (AS). There are case reports, besides their adverse effects, such as infection and injection site reaction, suggesting these agents may cause solid or hematologic malignancies. Acute leukemia secondary to anti-TNF alpha agents has been rarely reported in patients with AS. In this case report, based on a patient who developed acute leukemia while on the treatment with etanercept, we will discuss whether it is possible to predict acute leukemia by monitoring the mean corpuscular volume in light of the literature.

Objective: Systemic lupus erythematosus (SLE) may affect a number of systems, with the hematological system being one of the most common. Our aim is to determine the existence of cytopenia at diagnosis or during follow-up of our SLE patients as well as the associated factors., Material and Methods: A cohort of SLE patients that had been followed-up in the Department of Rheumotology from 1998 to 2015 was retrospectively assessed. Clinical and laboratory findings about the patients were recorded., Results: Out of 221 patients composing the cohort, cytopenia was already present in 83.3% (n=184) at the time of diagnosis. Anemia was detected in 56.1% (n=124), leukopenia in 28.9% (n=64), lymphopenia in 76% (n=168), neutropenia in 4.5% (n=10), and thrombocytopenia in 17.2% (n=38) of patients. The proportion of patients with cumulative cytopenia was 90% (n=199). Cumulative cytopenia was disease-related in 83.4% (n=166) and medication-related in 16.6% (n=33) of the patients. In cases of drug-induced cytopenia, azathioprine was the most frequently prescribed drug. In patients with cytopenia at the time of diagnosis, erythrocyte sedimentation rates (ESR) were higher, C3 and C4 hypocomplementemia was more prevalent, and they were positive for anti-ds-DNA at a greater proportion (p<0.001, p=0.015, p=0.028, and p=0.019, respectively). Moreover, photosensitivity, renal involvement, and antiphospholipid syndrome (APS) were detected more frequently in patients with cytopenia at the time of diagnosis. There was no difference between the two patient sets in terms of other organ involvement (p>0.05)., Conclusion: The most common hematological disorders in SLE patients are lymphopenia and anemia, and patients must be further examined for APS and renal involvement if they suffer cytopenia., Competing Interests: Conflict of Interest: No conflict of interest was declared by the authors.

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized with immune complex formation and renal involvement of lupus and may include several kinds of pathological conditions, but mostly, it is associated with immune complex-induced glomerular disease. Pauci-immune lupus nephritis is a very rare condition. We describe a 45-year-old female patient with pauci-immune crescentic necrotizing lupus nephritis and briefly discuss the possible mechanism and pathogenesis., Competing Interests: Conflict of Interest: No conflict of interest was declared by the authors.

Hematological abnormalities are very common in the course of systemic lupus erythematosus (SLE). Myelofibrosis is a bone marrow disorder in which there is excessive fibrous tissue formation in the bone marrow. Various benign and malignant disorders can cause or be associated with a diffuse increase in the bone marrow reticular tissue. Some diseases such as infections, neoplasms, and autoimmune diseases may also induce bone marrow fibrosis (secondary myelofibrosis). Cytopenia from autoimmune myelofibrosis (AIMF) in SLE is a rare condition. Here we present a case of AIMF associated with SLE and aim to emphasize on the other cause of cytopenia in SLE., Competing Interests: Conflict of Interest: No conflict of interest was declared by the authors.

Objective: Behçet's disease (BD) is a systemic vasculitis with a significantly varying clinical course following relapses and remissions, which may involve a number of organs such as the skin, joints, lungs, and blood vessels as well as systems such as the central nervous system and gastrointestinal system. Its prognosis is known to be worse in males. There are several studies in the literature on the clinical features and gender distribution of BD. The aim of the present study was to determine the clinical characteristics of BD and the presence of a relation with gender and to investigate the correlation of our results with the current literature., Material and Methods: We retrospectively reviewed 329 patient files. The demographic features of the patients, their symptoms and findings of BD, the results of pathergy tests, the presence of any individuals in the family with BD, and HLA-B51 antigen positivity were recorded., Results: The most frequent findings were oral aphtous ulcers (100%), genital ulcers (84%), papulopustular lesions (69.9%), and joint involvement (57.4%). Vascular involvement and ocular involvement were significantly higher in males, whereas joint involvement and headache were more common in females (p<0.001, p=0.014, p<0.001, and p<0.001, respectively). Contrary to the literature, we found that the disease had an earlier onset in individuals with a positive familial history of an oral aphthous ulcer or BD (p=0.03 and p=0.02, respectively) and that joint involvement was more common in patients with erythema nodosum (p=0.02)., Conclusion: The clinical features of BD may vary between the genders. Variations exist in the results depending on the population size, the department where the study was conducted, the patient inclusion criteria, and the region where the patients live., Competing Interests: No conflict of interest was declared by the authors.

Objective: This study aimed to investigate the frequency in which familial Mediterranean fever (FMF) coexists with other diseases and determine whether Mediterranean fever (MEFV) gene mutations are involved in such coexistence., Material and Methods: In total, 142 consecutive patients with FMF investigated for MEFV mutation were enrolled in this study [Female: 87; Male: 55, mean age 32±12 years (11-62)]. All the patients were questioned for the presence of concurrent disorders, and the medical records of these patients were revised retrospectively. A previous diagnosis of inflammatory disorder other than FMF was considered true if it met the relevant criteria. MEFV mutations were divided into 2 groups, namely M694V and its subgroup (homozygous or heterozygous) (Group I) and others (Group II). Compound heterozygosity for M694V mutation was included in Group II to form a homogeneous group for Group I. Group I and Group II were compared according to phenotypical features. The presence of MEFV mutation was investigated in exons 2, 3, 5, and 10 by the multiplex-PCR reverse hybridization method., Results: Concomitant disorders were found in 17 of 73 patients with FMF (23%) in Group I and 5 of 56 patients (8.9%) in Group II (p=0.04). Concomitant disorders in Group I were as follows: 7 cases of amyloidosis, 2 cases of Behcet's disease (BD), 4 cases of ankylosing spondylitis (AS), 1 case of antiphospholipid syndrome, 1 case of Henoch-Schonlein purpura (HSP), 1 case of combination of psoriatic arthritis, HSP, and membranoproliferative glomerulonephritis, and 1 case of AS and amyloidosis. In Group II, the following disorders were found: 1 case of amyloidosis, 1 case of BD, 1 case of AS, 1 case of ulcerative colitis, and 1 case of vitiligo., Conclusion: The presence of M694V mutation may predispose patients with FMF to developing other inflammatory disorders., Competing Interests: No conflict of interest was declared by the authors.

Aim: To evaluate the long-term effects of anti-tumor necrosis factor-alpha (TNF-α) therapy on patients with chronic hepatitis B and C infections., Methods: Rheumatoid arthritis, ankylosing spondylitis and Crohn's disease patients administered anti-TNF-α therapy for at least 36 months were retrospectively reviewed for hepatitis B or C serology, liver function tests, viral load, genotype and liver biopsy results, if performed. Nine relevant cases receiving anti-TNF-α were evaluated: six patients had chronic hepatitis C, one had chronic dual hepatitis B and C and two had chronic hepatitis B infection., Results: The patient with dual infection exhibited virologic breakthrough for hepatitis C and required treatment. Two patients with occult hepatitis B infection developed hepatitis B surface antigen (HBsAg) reversion and low-level viremia at the end of the study., Conclusion: Long-term use of anti-TNF-α treatments may result in viral replication that requires anti-viral therapy. Before determining the safety of anti-TNF drugs in the treatment of autoimmune diseases in patients with hepatitis C infection, studies with large homogeneous patient groups must be performed, and the exact group of hepatitis C virus infected patients for whom anti-TNF treatment would be deemed safe should be identified. Prior to anti-TNF-α treatment, it seems logical to screen all patients for HBsAg and anti-HB core immunoglobulin G status, especially in endemic regions. These patients must be followed periodically by means of alanine aminotransferase, HBsAg and hepatitis B virus DNA to identify HBsAg reversion and active viral replication that might require anti-viral prophylaxis or treatment., (© 2014 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd.)

Objective: Some rheumatic diseases are being influenced by weather conditions. We examined the relationship between oral ulcers and weather/season in Behçet's disease (BD) patients and evaluated the oral ulcers' characteristics by the patients' perspectives., Material and Methods: Patients with BD were evaluated using a self-questionnaire, including patients' clinical and demographical characteristics and detailed characteristics of oral ulcers and the association with season., Results: The most common site of oral ulcers was the tongue (89%). Of 90 patients, there were predisposing factors in oral ulcers. Among patients with BD, 30 believed that seasonal variation aggravated their oral ulcers, especially in winter and autumn., Conclusion: The exacerbation of oral ulcers in patients with BD may be related to the seasons. The weather changes in our study are based on the patients' impression; these should be confirmed by objective findings and clinical assessment in further studies.

Objective: The aim of this study is to determine the incidence of isoniazid (INH)-related hepatotoxicity in patients with rheumatologic diseases receiving tumor necrosis factor-α (TNF-α) antagonists along with a disease-modifying antirheumatic drug (DMARD)., Material and Methods: We have retrospectively evaluated 87 patients receiving anti-TNFα agents who were followed up between June 2005 and February 2010 at our rheumatology department. Sixty-one of 87 patients have received INH prophylaxis for 9 months for latent tuberculosis infection., Results: A total of 61 (70.1%) of 87 patients used INH prophylaxis (Group I), while the remaining 26 (29.9%) (Group II) had not; 53 patients had used any DMARD in Group I, while 21 patients had used in Group II. No significant differences were found among Group I and II with respect to clinical features. When two groups were compared, in Group I, elevations of liver enzymes were detected in five patients (8.1%) who had normal baseline values. Among these patients, hepatotoxicity developed in two patients. Hepatotoxicity developed one patient in Group II (p=0.85)., Conclusion: INH chemoprophylaxis was well tolerated in patients using anti-TNF-α agent and a DMARD. It seems not to be a strong risk factor for hepatotoxicity. However, comorbidities and other drugs used may be additional factors in the elevation of transaminases.

This retrospective study aims to seek a relationship between the number of thrombotic events and presence of thrombophilic factors in Behcet's disease (BD). Any patient with a vascular problem evaluated for at least one thrombophilic factor was deemed eligible for the present study. Upon an evaluation of their medical records, 96 (23.9%) of the 402 patients diagnosed with BD were determined to have a history of thrombosis. Of these 96 patients, 72 (75%) had only one thrombotic attack, while the remaining 24 (25%) had two or more thrombotic attacks. Thrombophilic parameters were evaluated in only 36 of the 72 patients (50%) who had one thrombotic event (Group I) and in 18 of the 24 patients (75%) who had more than one thrombotic event (Group II). The patients with recurrent thrombotic events had a significantly higher incidence of combined thrombophilias than those patients with only one thrombotic event (8/18, 44%; 4/36, 11%; p < 0.01). The number of the patients free from thrombophilic risk factors was significantly higher in Group I than in Group II (21/36, 58%; 4/18, 22%; p < 0.02). Group II had an increased frequency of combined thrombophilic risk factors (OR 6.4; 95% CI 1.5-25.8) when compared to Group I. No significant differences were found between Groups I and II with respect to clinical features of BD. In conclusion, combined thrombophilias in patients with BD may have a role in the development of recurrent thrombotic events.