Background Juvenile-onset systemic lupus erythematosus (JSLE) is an autoimmune disorder with multisystem involvement, leading to inflammatory damage to the joints, kidney, central nervous system, and ...hematopoietic system affecting children and adolescents. According to these multisystem deteriorations lead reduction in health-related quality of life (HRQL) in patients with JSLE. Objectives The aim of this study was to assess the relationship between HRQL and pain in patients with JSLE. Methods 19 patients (female=16, male=3) with JSLE and their parents were enrolled in the study. The subjects were recruited in a pediatric rheumatology clinic. They were diagnosed with JSLE by a pediatric rheumatologist based on the clinical diagnostic criteria. Patients with recent diagnoses of JSLE and those with mental deficits were excluded. Visual Analog Scale (VAS) was used to evaluate pain (during rest, activity and exercise) and well being. Functional ability was evaluated with Childhood Health Assessment Questionaire (CHAQ). Also, the Turkish version of PedsQL 3.0 Arthritis Module was used to evaluate HRQL. Results The mean age was 14.16±2.66 years (range 12–18 years). The mean disease duration was 3.42±2.76 years (range 0.08–15 years). The mean of number of affected joint was 2.16±2.92. Significant relationships were found between score of VAS-pain (during activity) and number of affected joint (r=0.801 p=0,000) and score of PedsQL-patient's report (r=-0.655 p=0,002) and score of PedsQL-parent's report (r=-0.670 p=0,002) and score of VAS-well being (r=0.759 p=0,000). Conclusions This study demonstrated that there is significant relationship between increased pain and reduction health-related quality of life in patients with JSLE. Due to number of affected joint increasing pain may results impairment daily functions and reduction health-related quality of life in patients with JSLE. Therefore, we concluded that reduction of pain has substantial role on improving of health-related quality of life in treatment of patients with JSLE. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.6056
BackgroundJuvenile 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.ObjectivesTo 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.Methods107 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.ResultsWhile 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.ConclusionsInitial 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.ReferencesTse SML, Laxer RM. New advances in juvenile spondyloarthritis. Nat Rev Rheumatol 2012;8:269-79Kasapcopur O, Demirli N, Ozdogan H et al. Evaluation of classification criteria for Jüvenile-onset spondyloarthropathies. Rheumatol Int. 2005;25:414–18Disclosure of InterestNone declared
BackgroundFatigue is a common symptom in pediatric health conditions and is associated with poorer health related quality of life. (1). The Pediatric Quality of Life Inventory PedsQL™ ...Multidimensional Fatigue Scale (MFS) was designed by Varni and colleagues to measure fatigue in children. PedsQL-MFS is a widely used instrument to measure the disease-specific health-related quality of life summary measures in children and adolescents with arthritis (1-3).ObjectivesThe aim of this study was to describe the cultural adaptation, validity, and reliability of a Turkish version of the PedsQL-MFS in children and adolescents with arthritis.MethodsA total of 66 children (8-12 years) and adolescents (13-18 years) with arthritis and their parents were enrolled in the study. Children (n=32) and adolescents (n=34) were diagnosed with Juvenile Idiopathic Arthritis by a pediatric rheumatologist based on the ILAR diagnostic criteria. The 10 cm Visual Analog Scale (VAS)-fatigue was used to evaluate the validity of related domains in the the PedsQL-MFS. The participants rated how often a particular problem occurred in the past month, using a 5-point Likert Scale and for the young child self-report a 3-point scale. Each item is reverse-scored and rescaled to 0–100 scale, so that higher scores indicate fewer symptoms of fatigue. Both the PedsQL-MFS and 10 cm Visual Analog Scale (VAS)-fatigue were filled out by children and adolescents over 8 years of age and their parents.ResultsThe means of ages were 9.66±1.38 for children, 15.53±1.6 for adolescents. Internal reliability was good to excellent (Cronbach's alpha coefficients 0.86-0.91 for self-reporting and 0.83-0.91 for parent reporting). Parent–child concordance for all scores was moderate (0.49-0.57) (Table 1). The PedsQL-MFS and VAS-fatigue were highly correlated, with coefficients from -0.38 to -0.45, indicating concurrent validity.Table 1.Parent–child concordance for PedsQL-MFS8–12 years (α)13–18 years (α)Child self-report Total fatigue0.890.86 General fatigue0.900.87 Sleep/rest fatigue0.910.86 Cognitive fatigue0.900.87Parent proxy-report Total fatigue0.890.83 General fatigue0.900.83 Sleep/rest fatigue0.910.84 Cognitive fatigue0.910.86ConclusionsWe demonstrated the reliability and validity of fatigue measurement using the Turkish version of the PedsQL-MFS in our sociocultural context. the PedsQL-MFS can be utilized as a tool for the evaluation of fatigue in children and adolescents with JIA aged 8–18 years.ReferencesPaulo L.T., Len C.A., Hilario M.O., Pedroso S.A., Vitalle M.S., Terreri M.T. Fatigue in children with juvenile idiopathic arthritis: reliability of the “Pediatric Quality of Life Inventory-Multidimensional Fatigue Scale”. Rheumatol Int. 2015 Jan;35(1):133-9.Varni, J. W., Burwinkle, T. M., Limbers, C. A., & Szer, I. S. (2007). The PedsQL as a patient-reported outcome in children and adolescents with fibromyalgia: An analysis of OMERACT domains. Health and Quality of Life Outcomes, 5(9), 9.Varni, J. W., Burwinkle, T. M., & Szer, I. S. (2004). The PedsQL Multidimensional Fatigue Scale in pediatric rheumatology: Reliability and validity. Journal of Rheumatology, 31(12), 2494–2500.Disclosure of InterestNone declared
BackgroundMyocardial dysfunction due to coronary arterial lesions is an important complication after Kawasaki disease in childhood (1,2). Speckle-tracking echocardiography (STE) is a recently ...developed technique for evaluation of myocardial deformation (3).ObjectivesOur objective was to examine myocardial deformation at long term follow-up of Kawasaki patients.MethodsWe assessed regional myocardial function in 32 asymptomatic Kawasaki patients and 30 age matched healthy child as control group. STE examination were recorded and the acquired raw data were saved for offline analysis The inferoseptal, anterolateral, inferior, anterior, and inferolateral (posterior) walls were investigated for longitudinal systolic peak values for strain (LV-SR) imaging.ResultsMean age was 21±12 months, mean follow up time 61 months, Left ventricular ejection fraction was 57,373±8,338 in patients and 61,100±3,804 in control group. LV-SR of Kawasaki patients at basal-inferoseptal (20,000±6,046 vs 25,200±5,074), basal-anterolateral (19,595±5,713 vs 22,733±3,494), apical-septal (23,378±5,609 vs 26,267±4,334), apical-inferior (24,054±3,696 vs 25,200±2,396) segments had lower values than control group.ConclusionsWe demonstrated subclinical systolic impairment of function especially at left ascending artery territories with STE. STE has the potential to detect these subtle abnormalities, and help management during long term follow up.ReferencesPrintz BF, et al. Noncoronary cardiac abnormalities are associated with coronary artery dilation and with laboratory inflammatory markers in acute Kawasaki disease. Journal of the American College of Cardiology 2011;57(1): 86–92.Xu QQ, et al. Evaluation of left ventricular systolic strain in children with Kawasaki disease. Pediatric cardiology 2014;35(7): 1191–1197.Marwick TH, et al. Myocardial strain measurement with 2 dimensional speckle-tracking echocardiography: definition of normal range. JACC: Cardiovascular Imaging 2009;2(1): 80–84.Disclosure of InterestNone declared
BackgroundSystemic lupus erythematosus (SLE) is an autoimmune disease characterized by vasculitis and inflammation in various organs. Cardiovascular involvement, although not frequent in ...juvenile-onset SLE (j-SLE), if present, is a significant cause of morbidity and mortality (1). Particularly, involvement of the myocardium layer may lead to ventricular dysfunction since its progression is insidious (1,2). Speckle tracking echocardiography (STE) could demonstrate subclinical myocardial deformations (strain) that globally seems normal in conventional echocardiography (2).ObjectivesThe aim of this study is early detection of subclinical systolic dysfunctions in j-SLE with STE and if present, then to investigate whether this is disease-related or it is a result of other predisposing conditions.Methods35 patients with j-SLE and 30 healthy children as a control group were evaluated between January and August 2015 at outpatient clinics of Cerrahpasa Medical Faculty. STE was performed on all patients and controls. Medical records that are including age at diagnosis, duration of the disease, diagnostic criteria, laboratory tests and cumulative clinical manifestations were evaluated. SLE disease activity was assessed using the SLE Disease Activity Index (SLEDAI). A SLEDAI score >4 was arbitrarily designated as a sign of moderate/severe disease activity.Resultsj-SLE patients had lower EF values than control subjects. Left ventricular end diastolic dimension (LVEDD) and left ventricular end systolic dimension (LVESD) were significantly greater in j-SLE patients (42,278±4,530 vs. 37,314±5,535; 28,108±3,344 vs. 24,055±3,290 p=0.001, respectively) than in the control group. There was a significant reduction in systolic parameters of longitudinal strain in the j-SLE group (p<0.05) at all segments compared to control patients.SLE patients were divided into two subgroups. Group 1 included patients having SLEDAI scores >8 at the beginning of the disease but who improved with therapy during follow up, with resulting SLEDAI scores less than or equal to 4 points. Group 2 included j-SLE patients with SLEDAI scores >8 at diagnosis but with SLEDAI scores still greater than 4 at the end of follow up.In comparisons of two groups, mid inferior and mid inferolateral LV segment STE strain measurements of Group 2 were significantly lower than those in Group 1, (15.9000±6.47130 vs. 20.0714±4.49725 mid inferior; 17.9000±7.23341 vs. 23.2308±3.87629 mid inferolateral, p=0.075, 0.055, respectively).ConclusionsWe can say that prevention of long-term cardiovascular complications in j-SLE begins with noticing the iceberg early, before irreversible changes take place. In this regard, STE is an accurate method for detecting subclinical systolic dysfunction.ReferencesApte M, McGwin G Jr, Vilá LM, Kaslow RA, Alarcόn GS, Reveille JD. Associated factors and impact of myocarditis in patients with SLE from LUMINA, a multiethnic US cohort. Rheumatology (Oxford) 2008;47: 362–367.Huang BT, Yao HM, Huang H. Left ventricular remodeling and dysfunction in systemic lupus erythematosus: A three-dimensional speckle tracking study. Echocardiography 2014; 31: 1085–1094.Disclosure of InterestNone declared
Background Juvenile idiopathic arthritis (JIA) is a common chronic illness that affects 1 in 1,000 children (1). Patients with JIA may experience significant disability due to muscular weakness, ...joint pain, contracture, and physical deconditioning. Children with arthritis have been shown to participate in less physical activities and to have low functional ability and decreased physical fitness than their peers (2-5). Objectives To investigate the effects of land-based home exercise program on pain, functional ability, and health-related quality of life in patients with JIA. Methods A randomized controlled single-blind study design including 81 patients (ages 5-17 years) with JIA was used. Socio-demographic data and clinical features were assessed. Functional ability was assessed with 6-Minute Walking Test (6-MWT) and Childhood Health Assessment Questionnaire (CHAQ). Pain was measured using a Visual Analog Scale (VAS). Quality of life was evaluated with The Pediatric Quality of Life Inventory (PedsQL). Patients were randomly assigned to the exercise or control group. Exercise group (n=43) completed 12-week individually planned land-based home exercise program once a week at hospital for 4 days per week. Control group (n=38) was included in waiting list until the end of the study. All patients continued stable dosage on medication through the study. Results In comparison of first assessment values for 6-MWT, CHAQ, VAS, PedsQL-self report and PedsQL-parent report, there was no statistically significant difference in all outcome measures between two groups. While only VAS score decreased significantly (p<0.01) in control group, statistically significant improvements (p<0.001) were found in all outcome measures in exercise group after 12 weeks. Except that VAS score (p>0.05), changes in other outcome measures (p<0.001) were significant in favour of exercise group. Conclusions The study demonstratedthat participating in 12-week individually planned land-based home exercise program may result improved physical function and quality of life in patients with JIA. References Manners PJ, Bower C. Worldwide prevalence of juvenile arthritis: why does it vary so much? J Rheumatol 2002; 29: 1520–30. Henderson CJ, Lovell DJ, Specker BL, Campaigne BN. Physical activity in children with juvenile rheumatoid arthritis: quantification and evaluation. Arthritis Care Res 1995; 8: 114–9. Tarakci E, Yeldan I, Kaya Mutlu E, Baydogan SN, Kasapcopur O. The relationship between physical activity level, anxiety, depression, and functional ability in children and adolescents with juvenile idiopathic arthritis.Clin Rheumatol 2011; 30: 1415–20. Takken T, Hemel A, Van der Net J, Helders PJM. Aerobic fitness in children with juvenile idiopathic arthritis: a systematic review. J Rheumatol 2002; 29: 2643–7. Lelieveld OT, van Brussel M, Takken T, van Weert E, van Leeuwen MA, Armbrust W. Aerobic and anaerobic exercise capacity in adolescents with juvenile idiopathic arthritis. Arthritis Rheum 2007; 57(6): 898–904. Disclosure of Interest None Declared
Purpose The aim of this study was to describe the cultural adaptation, validity, and reliability of a Turkish version of the pediatric quality-of-life inventory (PedsQL) 3.0 Arthritis Module in a ...population with juvenile idiopathic arthritis (JIA). Methods A total of 169 patients with JIA and their parents were enrolled in the study. The Turkish version of the childhood health assessment questionnaire (CHAQ) was used to evaluate the validity of related domains in the PedsQL 3.0 Arthritis Module. Both the PedsQL 3.0 Arthritis Module and CHAQ were filled out by children over 8 years of age and by the parents of children 2–7 years of age. Results Internal reliability was poor to excellent (Cronbach's alpha coefficients 0.56–0.84 for self-reporting and 0.63–0.82 for parent reporting), and interobserver reliability varied from good to excellent (intraclass correlation coefficient (ICC) 0.79–0.91 for self-reporting and 0.80–0.88 for parent reporting) for the total scores of the PedsQL 3.0 Arthritis Module. Parent–child concordance for all scores was moderate to excellent (ICC 0.42–0.92). The PedsQL 3.0 Arthritis Module and CHAQ were highly positively correlated, with coefficients from 0.21 to 0.76, indicating concurrent validity. Conclusions We demonstrated the reliability and validity of quality-of-life measurement using the Turkish version of the PedsQL 3.0 Arthritis Module in our sociocultural context. The PedsQL 3.0 Arthritis Module can be utilized as a tool for the evaluation of quality of life in patients with JIA aged 2–18 years.
BackgroundJuvenile idiopathic arthritis (JIA) is not a single disease; it's group of all arthritis with unknown etiology, starting before 16 years of age and lasting at least 6 weeks long. JIA is the ...most common rheumatic disease in childhood. Carnitine is an important molecule in cellular energy metabolism. Secondary carnitine deficiency can be detected in chronic diseases by either renal loss or increased demand.ObjectivesWe hypothesized that secondary carnitine deficiency may be seen to increased demand in JIA patients. In the present study our aim is to determine free carnitine and acyl-carnitine levels of Turkish JIA patients.Methods114 patients with a diagnosis of juvenile idiopathic arthritis and a healthy 50 individuals served as control group, were included to the study. A fasting blood sample was taken for free carnitine and acyl-carnitine esters with tandem mass spectrometry from children in both groups.ResultsScreening of acyl-carnitine profile revealed free carnitine, C14, C16, C16-OH, C18 and C18:2-OH carnitine levels were higher (p<0,0001, p<0,0001, p<0,0001, p<0,0001 and p=0,004 respectively), while C6 carnitine levels were lower (p<0,0001) in JIA patients than the control group. Free carnitine levels were significantly higher (48.05±13.36 μmol/L) in patients under anti-inflammatory drug therapy than those who did not receive any treatment (43.18±7.96 μmol/L) (p=0,004).ConclusionsIn the present study we were not able to define secondary carnitine deficiency in JIA patients, therefore routine carnitine supplementation is not recommended in all patients with JIA.ReferencesRavelli A, Martini A. Juvenile idiopathic arthritis. Lancet. 2007 Mar 3; 369(9563):767-78.Stanley CA. Carnitine deficiency disorders in children. Ann N Y Acad. Sci.2004; 1033:42–51.Disclosure of InterestNone declared
BackgroundFamilial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurring self-limited fever, abdominal pain and chest pain caused by serositis (1). FMF mainly affects ...Middle-East populations with a high prevalence in Sephardic Jews, Turkish, Arabs and Armenians (2). Carnitine is an important molecule in cellular energy metabolism. Secondary carnitine deficiency can be detected in chronic diseases by either renal loss or increased needs (3).ObjectivesOur hypothesis was that FMF patients would have lower free carnitine levels than their healthy age and gender matched controls due to increased need of carnitine because of recurrent auto-inflammation. The present study was conducted to determine the patterns of free carnitine and acyl-carnitine esters in FMF patients.MethodsThis is a cross-sectional study of 205 FMF patients who were attending the outpatient Pediatric Rheumatology clinic of Cerrahpasa Medical Faculty Children's Hospital. The patients were selected by random sampling and FMF diagnosis was confirmed by a pediatric rheumatologist according to Yalcınkaya criteria. 50 healthy subsects were enrolled to the present study. A fasting died blood sample was taken for studying free carnitine and acyl-carnitine esters with tandem mass spectrometry from children in both groups.ResultsAcyl-carnitine analyses in spot dried blood samples with ESI-MS/MS were performed in all patients and control group. Screening of acyl-carnitine profile revealed free carnitine, C16-OH and C18:2 carnitine levels were higher (p<0,0001, p<0,0001 and p=0,003 respectively), while C4-OH and C4DC carnitine levels were lower (p<0,0001) in FMF patients than the control group.ConclusionsIn the present study we were not able to define secondary carnitine deficiency in FMF patients, therefore usage of carnitine in all patients with FMF is not recommended.ReferencesOzen S, Bilginer Y. A clinical guide to autoinflammatory diseases: familial Mediterranean fever and next-of-kin. Nat Rev Rheumatol. 2014;10(3):135-47.Yilmaz E, Ozen S, Balci B, Duzova A, Topaloglu R, Besbas N et al. Mutation frequency of Familial Mediterranean Fever and evidence for a high carrier rate in the Turkish population. Eur J Hum Genet 2001;9:553–5.Stanley CA. Carnitine deficiency disorders in children. Ann N Y Acad. Sci.2004; 1033:42–51.Disclosure of InterestNone declared
BackgroundJuvenile systemic scleroderma (jSSc) is an orphan disease, with an estimated prevalence of 3 per 1000 000 children. Most jSSc patients primarily present with Raynaud phenomenon (RP). We ...investigated in our patient of the juvenile scleroderma inception cohort, how fare patients with (RP+) and without (RP-) RP differed in their clinical presentation at enrolment.MethodsThe jSSc is a prospective cohort of jSSc patients. Patients were enrolled who were diagnosed with jSSc, had a jSSc onset age under 16 years and were younger as age of 18 years at the time of inclusion. The patients are prospectively assessed every 6 months according to a standardised protocol. We reviewed the organ involvement pattern of our patients currently followed in the cohort.Results100 patients are currently followed in the cohort and 89 (89%) of them had RP. The female/male ratio was lower in the RP +group, 3.7:1 compared to 4.5:1(p=0.808). Diffuse subtype was more common in the RP +group, 72% compared to 63%. Mean age of onset of first non- Raynaud symptomatic was 10.4 years in both groups. Mean disease duration was slightly higher in the RP +group, 3.4 compared to 2.2 years. ANA positivity was higher in the RP +group, 88% compared to 70% (p=0.48). Anti-Scl70 was 34% in the RP +and 20% in the RP-group (p=0.34). Interestingly 7% of RP +but none of the RP +were anti-centromere positive. The mean modified skin score was lower in RP +group (mean of 14.8 compared to 17.0). There were significantly more nailfold capillary changes (70% compared to 18%, p=0.001) and a higher rate of history of ulceration in the RP +group (49% compared to 20%, p=0.083). Decreased DLCO and FVC <80% was higher in the RP-negative group with 45%/50% compared to 37.5%/31% respectively. Pulmonary hypertension occurred in 7% in the RP +group and there was no case in the RP- group (p=0.335). RP- group had a higher rate of urinary sediment changes 18% compared to 4.5% in the RP +group (p=0.07). No renal crisis or hypertension was reported in neither groups. Gastrointestinal involvement was similar between the two groups with around 35%. Occurrence of swollen joints was similar in both groups as the frequency of muscle weakness with around 20%. The tendon friction rub occurred around 10% in both groups. In the patient related outcomes, there was only a difference in rating of Raynauds activity.ConclusionsThe RP– group differed from RP +group in the clinical presentation at enrolment. The absence of Raynaud phenomenon was associated with a decreased rate of history of ulceration, no occurrence of pulmonary hypertension. Interestingly higher rate of urinary sedimentary changes and no anticentromere positivity was observed in RP- patients.Disclosure of InterestNone declared
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