High Levels of Circulating Endothelial Progenitor Cells Are Associated with Acrotism in Patients with Takayasu Arteritis

Objectives: To investigate the association between endothelial progenitor cells (EPCs) and Takayasu arteritis (TA). Subjects and Methods: A total of 39 subjects were included in this study: 12 subjects had been diagnosed with active TA, 11 had active Behcet disease (BD), and 16 were healthy controls. The EPCs, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP) levels of all the subjects were measured. MedCalc 15.8 software (MedCalc, Belgium) was used for all statistical analyses. Results: The level of EPCs was higher in TA patients (4.25 +/- 2.56) than in the BD group (2.27 +/- 2.0) and the healthy controls (2.12 +/- 1.2) (p = 0.015). TA patients with acrotism (n = 4) had higher levels of EPCs compared to TA patients without acrotism (n = 8) (6.50 +/- 1.73 vs. 3.12 +/- 2.16, p = 0.02). A positive correlation was found between EPCs and the ESR (r = 0.723, p = 0.0079) and between EPCs and CRP in patients with TA (r = 0.769, p < 0.0034). Conclusion: High levels of circulating EPCs were correlated with the CRP level and the ESR in patients with TA. These cells could be a marker for acrotism and inflammation in patients with TA. (C) 2016 S. Karger AG, Base

Anti-Tumor Necrosis Factor Alpha Treatment and Tuberculin Skin Test

Purpose: The use of anti-tumor necrosis factor alpha (anti-TNF) drugs has been a milestone in the treatment of rheumatic diseases. Despite their strong efficacy, there are some factors restricting the use of anti-TNF agents. We must be careful especially for the granulomatous diseases which can be seen endemic in our country such as tuberculosis and leishmaniasis. In our country according to the RAED 2005 Consensus Meeting Reports, patients candidate for anti-TNF treatment are evaluated for both active and inactive tuberculosis before treatment and prophylaxis with isoniazid (INH) has been performed where indicated. Material and Methods: Tuberculin skin tests (TST) of 43 patients followed up in the Rheumatology Clinic and receiving anti-TNF therapy were repeated under treatment. Patients' pretreatment first TST results, drugs they used, INH prophylaxis state, smoking status and the duration of anti-TNF treatment were evaluated. Results: 14 patients (32.6%) were women, while 29 (67.4%) were men. The mean of first TST values were 11.72 +/- 90.3 mm (0-30) and the mean of second TST values were 12.06 +/- 12.4 mm (0-45). 48.8% of the patients were smoking and 74.4% of the patients had received INH prophylaxis for 9 months. The mean total duration of anti-TNF drug use was found as 22.67 +/- 19.11 (5-68) months. No statistically significant difference (p=0.888) was observed between the first pretreatment and second under treatment TST results of the patients. Discussion: Tuberculosis remains to be a serious public health problem for both our country and the whole world. For this reason in our country, a detailed assessment is performed for all patients before anti-TNF treatment. In our study patients who are planned to start anti-TNF therapy were assessed with their first TST values and INH prophylaxis were given to 32 patients (74.4%) before treatment. No statistically significant difference was observed between pre and post-treatment TST values when control TST were performed with the earliest after five months of treatment. These findings may suggest that there is no evident increase in the risk of tuberculosis for patients receiving anti-TNF treatment with appropriate INH prophylaxis

Granulomatous polyangitis (Wegener granulomatosis): Clinical findings and results of long-term follow-up

Introduction: To evaluate long-term outcome of patients with granulomatous polyangitis (GPA) followed up in a tertiary university hospital. Patients and Methods: We reviewed medical records of 22 patients with GPA diagnosis confirmed by tissue biopsies between 2004 and 2014. Results: The mean time from the onset of symptoms to diagnosis was 7.8 +/- 12.3 months [interquartile range (IR)= 4.0]. The most commonly involved organs were the upper respiratory tract (URT) (72.7%), lower respiratory tract (81.8%) and kidneys (72.7%). URT involvement indicated good prognosis (p= 0.046). Survival in the patients with and without URT involvement was 124.6 +/- 6.9 months and 59.7 +/- 22.9 months, respectively. End-stage renal failure (ESRF) requiring dilaysis and cardiac involvement were associated with mortality (p= 0.022 and p= 0.026, respectively). Of the 12 dialysis-dependent patients at diagnosis, 11 survived > 3 months and seven regained renal function permanently. Dialysis dependency was significantly lower in patients who received plasmapheresis (p= 0.047). Overall mortality rate was 18% (4/22). Mean survival was 55.9 +/- 42.8 months (IR= 84.0). Conclusion: Diagnosis of GPA may be delayed by the nonspecific nature of its symptoms. URT involvement was associated with good prognosis, whereas cardiac involvement and ESRF requiring dialysis were associated with poor outcome. Plasmapheresis may increase the rate of renal recovery in the patients with ESRF requiring dialysis

If Neurologists Establish The Diagnosis of Primary Sjogren's Syndrome?

Introduction: Neurological involvements were shown in 20% of patients with Primary Sjogren's Syndrome (pSS). Neurological symptoms may be the first signs of pSS in 57% of the cases. In addition, early diagnosis and treatment of neurological disorders may save or improve the quality of life of these cases. There have been reports about the neurologic manifestations of pSS but little is known about the details of neurologically presented cases. Method: In this study, we described 11 pSS patients who presented with neurological manifestations. Results: Central nervous system (CNS) involvement was recorded in 7 (63.7%) and peripheric nervous system (PNS) involvement in 4 cases (36.4%). Conclusion: Our findings regarding the cases with neurological manifestations leading to the diagnosis of pSS suggest that 1) The frequency of CNS involvement was higher than that of PNS, and the most frequent clinical pictures of CNS involvement are Multiple Sclerosis (MS)-like illnesses and optic neuritis, 2) Guillain Barre Syndrome (GBS) was the most frequent disease of PNS involvement; 3) Mononeuropathy multiplex (MM) might be the first sign of pSS; 41 Neurologists should consider pSS in the differential diagnosis of cases with MS, optic neuritis, GBS and neuropathies of unknown causes including MM; 51 There is an urgent need of therapeutical guidelines for the cases with neurological involvement associated with pSS

Spondylodiscitis and endocarditis caused by S. vestibularis

Streptococcus vestibularis is a recently described member of the viridans group that was first isolated from the vestibular mucosa of the human oral cavity and described as a new species in 1988. It has been rarely associated with human infections. In few papers, it has been reported as a causal agent of systemic infection in immunosupressed adults and in those with other severe underlying diseases, like coronary valve diseases. A 65-year-old woman was admitted to the hospital with complaints of fever for three months, general malaise, effort dyspnea, weight loss, back pain and myalgia. Both native aortic valve endocarditis and spondylodiscitis due to Streptococcus vestibularis were detected. The patient was successfully treated with intravenous potassium penicillin G and gentamicin for six weeks, followed by oral amoxicillin for three months, in addition to aortic valve replacement. In all patients with spondylodiscitis, infective endocarditis should be considered, particularly in patients with heart valve disease history, since spondylodiscitis may be the presenting sign of an infective endocarditis. Cardiac valve replacement surgery should be performed if the course of fever and inflammatory syndrome is unfavorable after appropriate antibiotic treatment. We report the first case with both native aortic valve endocarditis and spondylodiscitis due to Streptococcus vestibularis

The Psoriatic Arthritis Registry of Turkey: results of a multicentre registry on 1081 patients

WOS: 000397050600018PubMed ID: 27794533Objective. The aim was to assess the characteristics of PsA, find out how well the disease is controlled in real life, demonstrate the treatments and identify the unmet needs. Methods. The PsA registry of Turkey is a multicentre Web-based registry established in 2014 and including 32 rheumatology centres. Detailed data regarding demographics for skin and joint disease, disease activity assessments and treatment choices were collected. Results. One thousand and eighty-one patients (64.7% women) with a mean (S.D.) PsA duration of 5.8 (6.7) years were enrolled. The most frequent type of PsA was polyarticular [437 (40.5%)], followed by oligoarticular [407 (37.7%)] and axial disease [372 (34.4%)]. The mean (S.D.) swollen and tender joint counts were 1.7 (3) and 3.6 (4.8), respectively. Of these patients, 38.6% were on conventional synthetic DMARD monotherapy, 7.1% were on anti-TNF monotherapy, and 22.5% were using anti-TNF plus conventional synthetic DMARD combinations. According to DAS28, 86 (12.4%) patients had high and 105 (15.2%) had moderate disease activity. Low disease activity was achieved in 317 (45.7%) patients, and 185 (26.7%) were in remission. Minimal disease activity data could be calculated in 247 patients, 105 of whom (42.5%) had minimal disease activity. The major differences among sexes were that women were older and had less frequent axial disease, more fatigue, higher HAQ scores and less remission. Conclusion. The PsA registry of Turkey had similarities with previously published registries, supporting its external validity. The finding that women had more fatigue and worse functioning as well as the high percentage of active disease state highlight the unmet need in treatment of PsA

Exon 2: Is it the good police in familial mediterranean fever?

WOS: 000463722100007PubMed ID: 30489254Objective: Familial Mediterranean fever (FMF) is the most common autoinflammatory disease. Most of the identified disease-causing mutations are located on exon 10. As the number of studies about the effect of the exonal location of the mutation and its phenotypic expression is limited, we aimed to investigate whether the exonic location of the Mediterranean fever (MEFV) mutation has an effect on the clinical manifestation in patients with FMF. Methods: Study population was derived from the main FMF registry that included 2246 patients from 15 different rheumatology clinics. We categorized the mutations according to their exon locations and retrieved the clinical and demographic information from the database. Results: Patients having the MEFV mutations on exon 2 or 10 (n: 1526) were divided into three subgroups according to the location of the MEFV mutations: Group 1 (exon 2 mutations), Group 2 (exon 10 mutations), and Group 3 (both exon 2 and exon 10 mutations). Group 2 patients were of a significantly younger age at onset, and erysipel-like erythema, arthritis, amyloidosis, and a family history of FMF were more common in this group. Conclusion: Patients with FMF and exon 10 mutations show more severe clinical symptoms and outcome. Exon 2 mutations tend to have a better outcome