Serdal Ugurlu

Istanbul University, İstanbul, Istanbul, Turkey

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Publications (98)428.75 Total impact

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    ABSTRACT: This open-label pilot study aimed to investigate the efficacy of canakinumab in colchicine-resistant familial Mediterranean fever (FMF) patients. Patients with one or more attacks in a month in the preceding 3 months despite colchicine were eligible to enter a 30-day run-in period. Patients who had an attack during the first run-in period advanced to a second 30-day period. At the first attack, patients started to receive three canakinumab 150 mg subcutaneous injections at 4-week intervals, and were then followed for an additional 2 months. Primary efficacy outcome measure was the proportion of patients with 50 % or more reduction in attack frequency. Secondary outcome measures included time to next attack following last canakinumab dose and changes in quality of life assessed by SF-36. Thirteen patients were enrolled in the run-in period and 9 advanced to the treatment period. All 9 patients achieved a 50 % or more reduction in attack frequency, and only one patient had an attack during the treatment period. C-reactive protein and serum amyloid A protein levels remained low throughout the treatment period. Significant improvement was observed in both physical and mental component scores of the Short Form-36 at Day 8. Five patients had an attack during the 2-month follow-up, occurring median 71 (range, 31 to 78) days after the last dose. Adverse events were similar to those observed in the previous canakinumab trials. Canakinumab was effective at controlling the attack recurrence in patients with FMF resistant to colchicine. Further investigations are warranted to explore canakinumab's potential in the treatment of patients with colchicine resistant FMF. ClinicalTrials.gov NCT01088880 . Registered 16 March 2010.
    Full-text · Article · Dec 2015 · Arthritis Research & Therapy
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    ABSTRACT: Objectives: Fatigue is an important problem in inflammatory diseases and affects the quality of life (QoL). We aimed to evaluate the severity and impact of fatigue in Behçet's syndrome (BS) and to determine its association with type of organ involvement and gender. Methods: One hundred and fifty-two BS, 51 rheumatoid arthritis (RA), 51 systemic lupus erythematosus (SLE), 51 ankylosing spondylitis (AS) patients and 65 healthy controls were evaluated by the fatigue severity scale, fatigue impact scale, fibromyalgia impact questionnaire (FIQ), RAPID3, SF-36 and Behçet's syndrome activity scale (the latter only in BS patients). We also analysed subgroups of BS patients with predominantly eye, vascular, joint and mucocutaneous involvement and did an additional gender analysis. Results: Fatigue severity and fatigue impact scores were similar among BS, RA, SLE and AS patients and significantly higher than that in healthy controls (F4df =8.51; p<0.001 and F4df = 8.67; p<0.001, respectively). The fatigue severity and fatigue impact scores were similarly high in BS subgroups with different types of organ involvement, and in both genders. Conclusions: Fatigue is an important problem in BS, as it is in other inflammatory conditions. It is similarly severe in subgroups of patients with eye, vascular, joint and mucocutaneous involvement and in either gender. Fatigue is a candidate outcome measure for clinical trials, to assess the life impact of Behçet's syndrome.
    Full-text · Article · Oct 2015 · Clinical and experimental rheumatology
  • H Ozdogan · S Ugurlu · B Ergezen

    No preview · Article · Sep 2015 · Pediatric Rheumatology
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    H Ozdogan · S Ugurlu · G Hatemi · E Ozgun · G Can · G Ozgon · B Ergezen

    Preview · Article · Sep 2015 · Pediatric Rheumatology
  • A Gul · H Özdogan · O Kasapcopur · B Erer · S Ugurlu · S Sevgi · S Turgay

    No preview · Article · Sep 2015 · Pediatric Rheumatology
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    Full-text · Article · Sep 2015 · Pediatric Rheumatology

  • No preview · Article · Sep 2015 · Pediatric Rheumatology
  • S Ugurlu · E Seyahi · G Hatemi · A Hacioglu · H Ozcan · FN Akkoc · H Ozdogan
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    ABSTRACT: Background In a recent pilot study, it was reported that Canakinumab reduced the frequency of attacks in 9 patients with Familial Mediterranean Fever (FMF) resistant to colchicine with no apparent side effects (1). Objectives We present our experience with Canakinumab in FMF patients resistant or intolerant to colchicine. Methods The charts of the patients with FMF who were on Canacinumab were evaluated retrospectively with regard to response and safety. Results There were 24 patients with FMF (14 F/10 M) who were receiving canakinumab for various indications. Here we report 19 (11 F/8 M) who had at least 3 injections. Four patients had concomitant diseases such as psoriasis, ankylosing spondylitis (2), polyarteritis nodosa. The indications for canakinumab (150mg/mo) were insufficient response to colchicine in 10 (>1 attack/month), amyloidosis in 6 and injection site reaction to anakinra in3 patients. The mean age of the patients was 31,10±13,04 years, while the disease duration was 24,84±10,29 years. The mean colchicine dose was 2,28±0,38 mg/day. The median injection number with canakinumab was 5 (range 3-14). The mean duration of canakinumab therapy was 9,68±5,26 months. Eleven of the patients had no attacks after canakinumab, while in five patients attack frequency was reduced more than %50. In 6 cases with FMF amyloidosis, proteinuria decreased in 2 (from 15020 mg/dl to 2226 mg/dl, and from 6135 mg/dl to 4000 mg/dl), increased in the other 2 (from 1700mg/dl to 4700mg/dl and from 5001 mg/dl to 7061 mg/dl), and did not change in the remaining 2. Ten of the 24 patients complaining of severe myalgia and calf pain unresponsive to colchicine treatment, improved significantly on CAN. According to patient global assessment 18 patients reported significant improvement while only one, reported no change. CAN was stopped because of remission (no attacks at least for 3 months) in 3 and for pregnancy demand in one patient. Attacks recurred after 4, 6, 12 months from discontinuation of CAN in 3 patients and 4th patient is still attack-free for the last 7months. Canakinumab was well tolerated in general. None of the patients had injection site reactions. The patient with psoriasis reported a flare in psoriatic plaques, which responded to local treatment. Therapy was discontinued temporarily in one patient who developed mild leucopenia, which did not recur on a 2-monthly regimen. Treatment was switched to another biological agent in 2 patients with amyloidosis because of increasing proteinuria. One other patient with amyloidosis whose proteinuria was stable, developed lichen planus lesions and CAN treatment had to be stopped. Conclusions Canacinumab is effective in controlling the attacks in patients with inadequate response to colchicine. In a selected group of FMF patients, Canacunimab may serve as a treatment alternative with a favorable side effect profile. For better understanding the drug's efficacy and safety in the long term there is a need for controlled trials. References Disclosure of Interest : None declared DOI 10.1136/annrheumdis-2014-eular.5654
    No preview · Article · Sep 2015 · Pediatric Rheumatology

  • No preview · Article · Sep 2015 · European Respiratory Journal
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    Full-text · Article · Sep 2015 · Pediatric Rheumatology
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    ABSTRACT: Anti-TNF agents are being increasingly used in patients with Behçet׳s syndrome (BS) when conventional immunosuppressives fail. However, experience with anti-TNF treatment on pulmonary artery involvement (PAI) of BS is limited. A chart review revealed 13 patients with PAI (all men) treated with anti-TNF agents (12 infliximab and 1 adalimumab) following an inadequate response to immunosuppressives for 12.2 ± 9.5 SD months and 2 male patients who developed PAI while receiving infliximab for large vein thrombosis for 10 months and for parenchymal central nervous system involvement for 2 years, respectively. The first patient developing PAI while receiving infliximab responded to cyclophosphamide and prednisolone but the second died with hemoptysis within 1 month. At the end of the survey, 6 of the 13 patients with PAI were continuing these agents for 25.5 ± 16.2 SD months with good response, 4 stopped anti-TNF treatment after a mean of 23 ± 9.8 SD months after achieving clinical and radiologic response and 1 patient with good response went to another center after receiving infliximab for 10 months and the remaining 2 experienced serious infections (lung tuberculosis and aspergillosis) necessitating early withdrawal. Two patients relapsed within 3 years after stopping anti-TNF agents and concomitant azathioprine. One developed mesenteric vein thrombosis necessitating bowel resection and the second developed new PAI that was controlled with cyclophosphamide and prednisolone after short courses of infliximab, adalimumab, and canakinumab. Anti-TNF treatment seems to be effective for refractory PAI of BS but may not prevent its development. Relapses can be seen after withdrawal. Caution is required for their serious adverse effects. Copyright © 2015 Elsevier Inc. All rights reserved.
    No preview · Article · Jun 2015 · Seminars in arthritis and rheumatism
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    ABSTRACT: Objectives Behçet's syndrome (BS) is most active during young adulthood and may cause severe disability. We had previously observed a high frequency of work disability among our Behçet's patients in a cross-sectional study. This time we aimed to evaluate the sustainability of work among our patients who did have a job 5 years ago. Methods We had surveyed work disability among 300 consecutive Behçet's syndrome patients who attended our clinic in 2009. We had observed that after excluding students, homemakers and retired patients, among the 149 work eligible patients 29 (21%) were unemployed and 120 were employed. We now evaluated those patients who did have a job 5 years ago regarding work loss and reasons for work loss, using a standard questionnaire. We also checked their hospital charts for new types of organ involvement that developed during these 5 years, and any new medications that were started. Results Among the 120 patients who did have a job in 2009 (87 men, 33 women, mean age 36±8.6, disease duration 9.7±7.1 years, 48% with major organ involvement), we were able to contact 97 patients. Sixteen patients (16%) had lost their jobs during the previous 5 years. Nine of these (11%) were related with Behçet's syndrome and the rest were due to other causes (5 had retired, 1 had a baby, 1 was doing his military service). Among the 81 patients who were still working, 10 (10%) had to change their work place during the last 5 years and were unemployed for a mean of 6.7±5.3 months between these jobs. Conclusions Work disability is an important problem among Behçet's syndrome patients. During a 5 year follow-up, 11% of patients lost their jobs due to their disease, and a further 11% had to interrupt work and change their work places. We were only able to evaluate work disability in this study, and work productivity is another important issue that needs to be studied among patients with Behçet's syndrome. Disclosure of Interest None declared
    No preview · Article · Jun 2015 · Annals of the Rheumatic Diseases
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    ABSTRACT: Background BCG vaccination is thought to cause false positive PPD and concomitant medications to cause false negative PPD results when screening patients before starting TNF-alpha antagonists. Moreover it is assumed that INH is difficult to tolerate in this patient group. However there is a long time between BCG vaccination and TNF-alpha antagonist use in most of the rheumatology patients; there is no consistent data to show that medications cause negative PPD results; and INH is usually well tolerated by most of our patients. Objectives To determine whether BCG vaccination causes false positive PPD, concomitant medications cause false negative PPD and whether INH is difficult to tolerate in these patients. Methods We included adult patients who were prescribed a TNF-alpha antagonist for the first time between January 2010 and December 2012 in our clinic. Patients who had a history of active tuberculosis were excluded. BCG vaccination was determined by checking for BCG scars. We used logistic regression to analyse the determinants of a positive PPD (≥5 mm). The variables were having a BCG scar, each medication, age, gender, diagnosis and disease duration. We also evaluated the frequency of being able to complete 9 months of INH treatment and the reasons for discontinuation. Results A TNF-alpha antagonist was started in 1229 patients (613 men, 616 women, mean age 39.53±13.82 years, disease duration 6.49±6.87 years). We excluded 136 patients who had previously used a TNF-alpha antagonist, 21 patients younger than age 18 and 41 patients who had previous tuberculosis treatment. Among the remaining 1031, an initial PPD test was available in 873, and QTF in 215 patients. At least one BCG scar was present in 757 patients. Multivariate regression analysis showed that BCG vaccination and male sex were associated with PPD positivity (OR=3.21, 95%CI 1.87-5.52, p<0.001; and OR=2.51, 95%CI 1.78-3.53, p<0.001 respectively), while azathioprine use was associated with a negative PPD (OR=0.50, 95%CI 0.27-0.93, p=0.029). 482/565 (85.30%) patients completed 9 months of treatment, 30 with interruptions and 34 with mild transaminase elevations not requiring interruption. 69 (12.21%) had to stop INH after 3.43±2.27 months. The reasons were hepatotoxicity in 31, non-willingness in 13, allergic dermal reactions in 6, nausea in 2, dizziness in 2, pregnancy in 1, shortness of breath in 1 and pancreatitis in 1 patient. Twelve patients stopped taking INH after their TNF-alpha therapy was stopped. Among the 31 who had to stop INH for transaminase elevation 7 were using concomitant methotrexate. None of the patients developed tuberculosis during our follow-up of up to 3 years. Conclusions BCG vaccination may still be a cause of false positive PPD in candidates for treatment with TNF-alpha antagonists. Azathioprine seems to be associated with negative PPD. INH prophylaxis is generally well tolerated despite concomitant methotrexate. Disclosure of Interest None declared
    No preview · Article · Jun 2015 · Annals of the Rheumatic Diseases
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    ABSTRACT: Background Women with auto-inflammatory disease on long-term IL-1 blockade increasingly ask about the safety and outcomes of pregnancy but little data is available. Objectives To analyse the outcomes of pregnancy in women on long-term anti-IL-1 therapy. Methods We obtained the clinical details and pregnancy outcomes of women exposed to anti-IL-1 agents in 7 countries. Results We identified 13 women aged 16 – 38 years, with a total of 15 pregnancies exposed to IL-1 blockade. 9 women had CAPS, 2 Adult Onset Stills Disease, 1 TRAPS, 1 FMF and 1 Idiopathic Pericarditis. 4 received canakinumab, 2 stopped treatment after conception, 2 switched to anakinra. 13 foetuses were exposed to anakinra. 3 women started anakinra during pregnancy, 3 stopped treatment following conception. All pregnancies were completed. The shortest term was 35 weeks. APGAR score was 9-10 at 10 mins in all cases. Birth weight range 2.02-3.94 kilos. 5 were breast fed for 3 – 40 weeks. Age at last contact was from 1 week – 8 years. Only 1 child had developmental abnormalities; growth hormone deficiency due to ectopic neurohypophysis and unilateral renal agenesis. We also identified 11 children with paternal exposure to anti-IL-1 therapies, all developmentally normal to date. Conclusions These successful pregnancies significantly extend our knowledge of pregnancy outcomes in parents receiving anti-IL-1 therapies. A co-author has previously reported the intrauterine death of a foetus (of a twin pregnancy) with bilateral renal agenesis exposed to anakinra. The surviving twin is developmentally normal. Although numbers are small the data provide reassurance to physicians caring for these patients. The relationship between IL-1 inhibition and renal agenesis requires further study. Each pregnancy should be assessed and risks and benefits of maintaining therapy individually discussed with potential parents. Disclosure of Interest None declared
    No preview · Article · Jun 2015 · Annals of the Rheumatic Diseases
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    ABSTRACT: Background There is still controversy about the association of TNF-alpha antagonists with malignancies. Although recent meta-analyses suggest that the risk of malignancy with these agents is not increased, the suspicion still exists, at least as reflected by the current labeling information. Objectives To survey the incidence of malignancy in patients treated with TNF-alpha antagonists for up to 13 years in a single center and to compare the results with the incidence of malignancy in the general population. Methods The charts of the first consecutive 376 rheumatoid arthritis (RA) patients (307 women; 69 men; mean age 55.5±12.7 SD years) who were prescribed TNF-alpha antagonists between 2001 and 2009 were reviewed retrospectively. Information regarding the demographic and clinical features, duration of use each TNF-alpha antagonist, development time and type of malignancy and the outcomes of patients were recovered from patient charts. Patients were invited to the clinic if their current status was not known. Age and sex standardized cancer incidence rates in the general population used to calculate standardized cancer rates (SIRs) were obtained from the Turkish Ministry of Health, Public Health Institution, Department of Cancer. Results The mean duration of follow-up was 67.6±17.7 SD months from the start of the first TNF-alpha antagonist to the end of 2013. During this time, 30 (7.9%) patients had died, 132 (35.1%) were still on TNF-alpha antagonists, 67 (17.8%) were using another biologic, 138 (36.7%) were no longer using any biologics and 10 (2.6%) were lost to follow-up. The main reasons of death were cardiovascular causes in 7 patients, sepsis in 5 and malignancies in 4. In 10 (2.6%) patients cause of death was unknown. A total of 8 patients had developed malignancies (2.12% [95% CI 0.66-3.58]). These were solid cancers in 6 (breast =1, lung =1, gastric =1, rectum =1, colon =1, unknown primary=1), hematologic cancers in 2 (acute leukemia=2). Since there were patients who were lost to follow-up and those who died of unknown causes, we calculated SIRs according to three different scenarios. If we assume that none of the patients who were lost to follow-up or whose cause of death was unknown, had a malignancy, the SIR would be 0.78 (95% CI 0.34-1.42). If we assume that all of the patients who were lost to follow-up (n=10) had a malignancy, but none of the patients whose cause of death was unknown had a malignancy, the SIR would be 1.7 (95% CI 1.04-2.0). Finally if we assume all patients who were lost to follow-up or whose reason of death was unknown (n=20) had a malignancy the SIR would be 2.7 (95% CI 1.8-3.8). Conclusions Our data indicate that an increased incidence of cancer, as compared to the general population, cannot be ruled out among RA patients enrolled in a TNF – alpha antagonist registry. This uncertainty is compounded by the fact that the comparisons we make here are between the incidence in a cohort of practically cancer free patients at the time of cohort entry and a general population incidence in which no such restriction is present. Disclosure of Interest None declared
    No preview · Article · Jun 2015 · Annals of the Rheumatic Diseases
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    ABSTRACT: Background Hemoptysis is the most frequent symptom of the feared pulmonary artery involvement (PAI) in Behçet's syndrome (BS). Some patients with PAI experience recurrent hemoptysis despite clinical response to treatment and regression or even resolution of abnormal radiologic findings. Enlargement of bronchial arteries and collateral formation may be the source of recurring hemoptysis in these patients. Objectives To evaluate the cause of hemoptysis in BS patients with PAI despite achievement of clinical and radiologic treatment response. Methods Retrospective chart review of 6 BS patients (all men) having repeated hemoptysis with no overt radiologic findings of active PAI. Results The mean age of the patients and their mean disease duration at the diagnosis of PAI was 28.8±5.8 SD years and 2±1.5 SD years, respectively. All patients had hemoptysis as the initial symptom of PAI that had been in the form of pulmonary artery aneurysms (PAA) in 5 patients and pulmonary artery thrombosis (PAT) in one. All patients received treatment consisting of monthly cyclophosphamide pulses and corticosteroids (1.7±1.2 mean ± SD years) that was followed by azathioprine. This treatment was successful in ameliorating hemoptysis in all patients except 1 PAA patient who experienced gross hemoptysis necessitating pulmonary artery embolization 2 years later. Hemoptysis recurred after a mean interval of 5.8±6.2 SD years (range 1 – 17 years). Thorax CT evaluations did not show any new development or worsening of existing PAI. Rather, PAA was resolved in 4 patients and regressed in 1 and PAT was also regressed in 1 patient. On the other hand, enlargement of bronchial arteries and collaterals were detected in 5 patients. Four of them underwent transcatheter bronchial artery embolization that was complicated by hemiparesis in 1 patient. This procedure was successful in controlling hemoptysis but was repeated in 1 patient 2 years later because of newly enlarged bronchial arteries. This patient still continues to experience occasional and small hemoptysis 3 years after the last procedure. The other 3 patients are currently free of hemoptysis ranging for between 3 months and 8 years after the procedure. The fifth patient died of massive hemoptysis in the emergency unit in another center before undergoing embolization. His last CT did not show any evidence of PAI. In the sixth patient, the source of bleeding could not be demonstrated despite repeated bronchoscopies and pulmonary angiography. This patient is now completely symptom free after 4 years and does not take any medications. Conclusions Enlargement of bronchial arteries and formation of collaterals can complicate the course of PAI during remission and may result in recurrent and potentially fatal hemoptysis in BS patients. Whether this enlargement is only due to the compromised pulmonary circulation or there is also accompanying vasculitis remains to be studied. Transcatheter embolization appears to be successful in controlling hemoptysis in such patients. Disclosure of Interest None declared
    No preview · Article · Jun 2015 · Annals of the Rheumatic Diseases
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    S. Ugurlu · B. Ergezen · H. Ozdogan
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    ABSTRACT: Background Approximately 5 to10% of FMF patients do not respond to colchicine treatment and/or intolerant to colchicine because of side effects. Several case reports and case series have pointed out the efficacy of IL-1 blockade in colchicine resistant FMFsubgroup. Objectives To review the patients followed in our center with FMF who received anakinra, an anti IL-1 receptor antagonist, because of insufficient colchicine response. Methods FMF patients who were treated with anakinra were retrospectively reviewed with regard to indication, effect on disease activity and acute phase response, adverse events. Patient global assessment was recorded before and after anakinra treatment. Results There were 30 FMF patients with FMF who were treated with anakinra for various indications (amyloidosis in 7, colchicine resistant recurrent febrile attacks in 19, colchicine related side effects in 4). The mean age of this group was 36.34±11.64 years. The mean duration of the disease was 23.93±12 years. There were various co-existing pathologies among this study group like multiple sclerosis (1), ankylosing spondylitis (1), SLE (1), Behçet's disease (1), low grade lymphoma (1) and PAN (2). Four patients were pregnant. The mean colchicine dose was 2,08±0,5 mg/d. The mean duration of anakinra treatment was 13,5±12,93 months. Twenty three patients reported no attacks after anakinra treatment whereas 5 patients reported at least 50% decrease in the attack frequency. Mean patient global assessment decreased from 8,60±2,3 to 1,92±2,7 under anakinra treatment (p=0.001). Among the 7 patients with amyloidosis, anakinra was stopped in 2 patients because of increased proteinuria. However, a significant decrease in proteinuria was detected in 3 patients. One patient developed severe injection site reaction and therefore the drug was stopped. Overall, 4 of our patients with amyloidosis are still on Anakinra treatment. Three patients had a severe allergic reaction (severe disseminated rash in 1 patient and severe injection site reaction in 2 patients) and therefore the drug was stopped. Two patients had infections (one had genital warts and urinary tract infection, the other had sinusitis and folliculitis) and the treatment was terminated. One of our patients reported that her psoriatic lesions got worse on anakinra. Twenty two patients reported no adverse events during the treatment. Conclusions Anakinra was effective in controlling the symptoms in colchicine-resistant FMF cases. It was also effective in FMF related amyloidosis. The major cause of treatment termination was injection site reactions. Anakinra seems to be an effective alternative in patients who have insufficient response to colchicine. Disclosure of Interest None declared
    Preview · Article · Jun 2015 · Annals of the Rheumatic Diseases
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    ABSTRACT: Background Juvenile spondyloarthropathies (JSpA) are a group of chronic childhood rheumatic diseases which emerge especially before 16 and after 6 years-old. While enthesitis and oligoarthritis are the major signs of the early period, axial skeletal involvement and sacroiliitis are the late period signs. Although the diagnosis of the spondyloarthropathy is easy in adults, it is quite difficult in children. Objectives To determine clinical and demographical features of the JSpA cases that is followed-up in our department, and to evaluate treatment modalities and long term complications of disease together with the side effects of the drugs. Methods 107 children (21 female, 86 male) that admitted to our clinic with the diagnosis of JSpA between January 2005-December 2014 were involved in our study. Enthesitis related arthritis and Juvenile Ankylosing Spondylitis were included under the topic of JSpA. Clinical and laboratory variables were obtained from patient records. Results While the mean age of the disease onset and diagnosis were 11,4±3 years (range 3 -17 years) and 12,4±2,8 years (range 5-18 years) respectively, the mean duration of follow-up period was 2,7±2,5 years (range 2 months-12 years). Lower extremity arthritis (n=83, 77,6%), hip pain (n=63, 58,9%) and inflammatory low back pain (n=47, 43,9%) were the major clinical findings. The most seen involvement of the lower extremity was ankle joint arthritis (n=60, 72,3%) followed by enthesitis (n=76, 71%) and tarsitis (n=30, 28%). Axial skeletal involvement was noted in 57% (n=61) of patients and mean inflammatory low back pain duration was 8,3±6,9 months (range 1-24 months). Magnetic resonance imaging could be studied in 77 patients (72%) that imaging results of 52 children (48,6%) were consistent with sacroiliitis. Lumbar movement limitation in Schober's test was seen in 42 cases (39.3%). Family history of spondylitis in 29 children (27.1%) and familial Mediterranean fever (FMF) in 4 cases. Furthermore, 5 patients diagnosed as FMF and treated with colchicine. Uveitis was seen in 11 children (10.3). Cardiological, pulmonary and renal investigations were normal of the whole study. HLA-B27 tissue antigen was positive in 73.8% (n=79) of the patients. First line therapies were methotrexate in 51 cases (47.5%) and sulphasalazine in 40 cases (37.4%). Anti-TNF alpha agents were used in 51 patients (47,7%) that were resistant to first line therapy. In the last medical visits we evaluated the patients in the terms of treatment modalities; 60.7% (n=65) of cases were noted as using drugs and in remission, 12.1% (n=13) gave up drugs and in remission, 16.8% (n=18) as minimally active and 6,5% (n=7) as active. Conclusions Initial signs of JSpA are usually lower extremity arthritis and enthesitis in children that is quite different from adults. JSpA should be strongly suspected in the case of a boy that is older than 6 years-old with lower extremity arthritis and the family history of spondyloarthropathy. Afterwards, axial skeletal evaluation must be investigated immediately and in order to prevent this complication true and efficient therapy must be started. References Disclosure of Interest None declared
    No preview · Article · Jun 2015 · Annals of the Rheumatic Diseases
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    ABSTRACT: Background Quality of life is commonly impaired in patients with chronic inflammatory diseases. The disease itself as well as the drugs used may be responsible for this impairment. Behçet's syndrome (BS) is a multisystem vasculitis with a wide variety of manifestations including oral ulcers, genital ulcers, nodular lesions, papulopustular lesions, arthritis, uveitis, venous thrombosis, arterial aneurysms, neurologic and gastrointestinal involvement. Determining the factors affecting quality of life in BS patients, would help developing effective management strategies. Objectives We aimed to determine the factors that impair quality of life (QoL) in BS. Methods Short- Form 36 (SF-36) physical component score (PCS) and mental component score (MCS) and Behçet Disease Quality of Life (BDQoL) questionnaires were filled by consecutive BS patients attending our outpatient clinic. Socioeconomic factors, each type of organ involvement during the disease course, during the last 4 weeks, disabilities caused by each, treatment modalities and overall disease activity were tested with regression analysis as possible determinants of QoL. Results 453 patients (M/F: 240/213, mean age: 38.2±10.8) were included in our study. 223 patients had eye involvement, 94 patients had vascular involvement, 102 patients had joint involvement, 28 patients had neurologic involvement, 2 patients had gastrointestinal involvement and 127 patients had only mucocutaneous involvement without organ involvement. Determinants of BDQoL in the whole group were BSAS, household income, perceived sufficiency income, neurologic damage, mucocutaneous involvement (R2: 0.38, p<0.001), among women were BSAS, neurologic and mucocutaneous involvement (R2: 0.36, p<0.001), and among men were disease related working disability, BSAS, household income, joint attack during the last 4 week, vascular and neurologic damage (R2: 0.39, p<0.001) Determinants of PCS in the whole group were BSAS, household income, eye, vascular and joint involvement during the last 4 week, disease related working disability (R2: 0.31, p<0.001), among men were BSAS, disease related working disability, household income, vascular involvement, vascular and joint involvement during the last 4 week (R2: 0.32, p<0.001), among women were BSAS, mucocutaneous involvement and perceived sufficiency income (R2: 0.32, p<0.001). Determinants of MCS in the whole group were BSAS, household income, neurologic damage (R2: 0.20, p<0.001), among men were BSAS, household income, neurologic damage, disease related working disability, mucocutaneous involvement (R2: 0.26, p<0.001), among women were BSAS and mucocutaneous involvement (R2: 0.21, p<0.001). Conclusions In addition to overall disease activity in women mucocutaneous and neurologic involvement and in men neurologic and vascular damage and joint attack seem to impair quality of life in BS. Determining the factors predicting the quality of life are important for developing management strategies and also may help determining the items that need to be addressed while developing outcome measures. Disclosure of Interest None declared
    No preview · Article · Jun 2015 · Annals of the Rheumatic Diseases
  • H. Ozdogan · S. Ugurlu · B. Ergezen
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    ABSTRACT: Background It has been reported that anakinra, an anti IL-1 R antagonist, may be a safe alternative treatment during pregnancy in a series of patients with various autoinflammatory syndromes (1,2). Objectives To assess the safety of anakinra in pregnant FMF patients and its effect on fetal and maternal outcomes. Methods Four FMF patients who had to use anakinra during pregnancy because of recurrent febrile attacks despite prophylactic colchicine therapy in 3 and colchicine side effect in 1, were monitored closely for disease activity, side effects, fetal USG and pregnancy outcome. Results Case 1: A 33-year old FMF patient with a previous diagnosis of multiple sclerosis, developed severe myalgia and high fever at her 21st gestational week (GW) while receiving colchicine 1.5 mg/d. She didn't respond neither to 30 mg/d prednisolone nor to steroid pulses of 500 mg. Anakinra 100 mg/d was initiated. After the 3rd injection, she was symptom-free. At her 36th GW due to vaginal bleeding, she underwent C-section and gave birth to a healthy baby. After the birth she continued with regular 1,5 mg/d of colchicine and anakinra was stopped. The 1st minute APGAR score of the baby was 8. The baby was breast fed and after 30 month of follow-up the mother and the baby are healthy. This case is among the 5 patients that has been previously reported by Lachmann H in 2013 (1). Case 2: 28 year-old FMF patient on colchicine, experienced frequent febrile attacks of abdominal pain and myalgia after the 9th GW. She did not respond to 30 mg/d prednisolone. Anakinra 100 mg/day was started at the 12th GW. Her symptoms gradually decreased after the 2nd week of anakinra. Anti IL-1 treatment together with colchicine continued till delivery. The 1st minute APGAR score of the baby was 10. After the birth anakinra was stopped and she continued with colchicine (2 mg/d) without recurrence of the febrile attacks. The follow up is 18 months. The baby is breast fed and the development remains normal. Case 3: 31-year old FMF patient has been treated with anakinra since 2012 with the indication of recurrent protracted febrile myalgia unresponsive to colchicine 2 mg/d. She conceived while on anakinra treatment. Because she was symptom-free, anakinra was stopped at 29th GW and continued only with colchicine. However she flared 4 weeks later and anakinra was reintroduced. She is now at 36th GW and the control fetal USGs are normal. Case 4: 24 year-old FMF patient developed thrombocytopenia (39,000/mm3) at her 15th GW which we thought might be secondary to colchicine. Colchicine was stopped and anakinra 100 mg/d and prednisolone 10 mg/d were started at 15th GW. The initial improvement in the platelet count was lost when the steroid dose was tapered. Now she is at 37th GW and thrombocyte levels remain low but she is otherwise healthy. Conclusions Our observation in 4 FMF patients provides additional data on the safety and outcome of pregnancies exposed to anakinra. IL-1 blockade seems to be a very effective approach in the treatment of protracted febrile myalgia, non-responsive to colchicine and corticosteroid therapy, even in pregnant FMF patients. References Disclosure of Interest None declared
    No preview · Article · Jun 2015 · Annals of the Rheumatic Diseases

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428.75 Total Impact Points

Institutions

  • 2006-2015
    • Istanbul University
      • Department of Family Medicine (Cerrahpasa Faculty of Medicine)
      İstanbul, Istanbul, Turkey
  • 2007-2014
    • Istanbul Medical University
      İstanbul, Istanbul, Turkey
  • 2009-2010
    • Fatih Sultan Mehmet Training and Research Hospital
      İstanbul, Istanbul, Turkey
  • 2007-2008
    • Cumhuriyet University
      • Faculty of Medicine
      Megalopolis, Sivas, Turkey