In this study, we examined the antibody responses after recombinant hepatitis B vaccine in juvenile SLE patients and whether antibody levels were affected by immunosuppressive therapy.
This study ...consisted of 64 juvenile SLE patients and 24 healthy controls. We evaluated HBsAg, Anti-HBs and Anti-HbcIgG titers in SLE patients. 24 patients (37%) were non-immunised, 39 patients were immunised (61%) and 1 patient (1.5%) was chronic hepatitis B carrier. Of the 24 non-immunised patients, 3 had active disease (SLEDAI>10) and 1 was being treated for tuberculosis infection so they were not included in the vaccination program. Twenty non-immunised SLE patients were given 3 dose recombinant hepatitis B vaccine doses at 0,1,6 months. AntiHBs antibody titer >10 IU/ml one month after the last dose of vaccine was accepted as seroconversion.
After 3 doses of vaccination, 16 (80%) of SLE patients and all of the healthy controls had seroconversion. Since two patients had SLEDAI score >10 after the first 2 doses of vaccine and one patient had SLEDAI score >10 after the first dose, these patients were given only two doses of hepatitis B vaccine. These patients had already seroconverted. One patient had exacerbation of the disease one month after the third dose of the vaccine. Protective antibody responses were statistically insignificant between the two groups (p=0.49). Geometric mean antibody titers of SLE patients were lower than those of the healthy controls. Adequate antibody response was not affected by immunosuppressive treatment as prednisone, azathioprine, and hydroxychloroquine.
Juvenile SLE patients could reach an adequate antibody response after recombinant hepatitis B vaccination and this response is not affected by immunosuppressive treatment.
To analyse the demographics, main clinical and laboratory features and subtype distribution of juvenile idiopathic arthritis (JIA) in an eastern Mediterranean country, based on a multicentre ...registry.
Between March 2008 and February 2009 with this cross-sectional study, consecutive patients seen with JIA in selected centres were registered through a web-based registry. All patients were classified according to the International League of Associations for Rheumatology (ILAR) criteria.
There were 634 patients with a mean age of 11.84 ± 4.66 years and the female/male ratio was 1.2. The distributions of JIA patients according to onset of disease were as follows: systemic 92 (14.5%), oligoarticular extended 26 (4.1%), oligoarticular persistent 234 (36.9%), rheumatoid factor (RF) positive polyarthritis 20 (3.2%), RF negative polyarthritis 129 (20.3%), enthesitis-related 120 (18.9%), psoriatic 13(2.1%). The frequency of uveitis was 15.7% among all of the oligoarthritis patients. Anti-nuclear antibody (ANA) was positive mainly among the oligoarticular onset patients. Twenty-one patients also had Familial Mediterranean fever (FMF). Among systemic JIA patients, the frequency of macrophage activation syndrome (MAS) was 15.2% (n=14). At the end of the mean follow-up of 7.6 ± 4.4 years, 305 (48.1%) patients were defined to have inactive disease on medication, and 106 (16.7%) were completely free of any disease symptoms without medication.
Enthesitis related arthritis had a high frequency whereas psoriatic arthritis was very rare compared to other series. We suggest that there are certain differences in the characteristics of JIA in our eastern Mediterranean population. Thus, genetic studies need to be assessed in these populations separately and findings of genome wide association studies need to be confirmed in different populations.
BackgroundFamilial Mediterranean Fever (FMF) is the most common periodic fever syndrome, characterized by recurrent fever and serositis attacks. It has been shown that there might be an ongoing ...subclinical inflammation between attacks. Adrenomedullin (ADM) is synthesized in endothelium, and has been shown to have high levels in patients with inflammation such as FMF. Colchicine is the treatment of choice and given once or twice daily depending on expert opinion.ObjectivesIn this study, it was aimed to investigate ADM as a marker for inflammation in pediatric patients with FMF who are using colchicine in different dosage schemaMethodsPediatric patients with FMF diagnosed clinically and genetically confirmed were included in the study. The colchicine was started in one or two doses randomly. The clinical and laboratory parameters were assessed on six clinical visits made every two months. After the third visit the dosing schema was changed to twice or once depending on the schema at the beginning.ResultsA total of 37 patients (female/male ratio: 0.94) were included in the study. Mean age of patients, age at disease onset, and age at diagnosis were 7.78±2.00, 5.05±3.04, and 7.51±2.66 years, respectively. Twenty patients received colchicine in once daily dosage while 17 patients had in twice-daily dosage at the beginning of the study. There were 10 patients with heterozygote and 27 with homozygote MEFV mutations. After the treatment was started all patients demonstrated improvement in clinical and laboratory findings such as erythrocyte sedimentation rate and C-reactive protein. However, ADM levels did not show any correlation with ESR and CRP levels. Mean ADM levels in six consecutive visits were as follows, first 322.19±161.92 ng/L; second 330.50±189.63 ng/L; third 339.54±168.03 ng/L; forth 378.11±177.63 ng/L; fifth 328.91±172.30 ng/L and sixth 326.25±165.87 ng/L. ADM levels were similar in all visits (p=0.954) and did not show any difference between the first and second three visits i.e. before and after changing the dosage schema (p=0.593).ConclusionsThe results indicated that patients using colchicine in once or twice daily doses did not show any significant difference according to the clinical and laboratory findings and had similar effects in controlling disease manifestations. ADM levels did not demonstrate any alterations in all visits that may suggest the continuation of subclinical inflammation in these patients.Disclosure of InterestNone declared
BackgroundJuvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in childhood, affecting the joints and lasting at least 6 weeks, with the age of disease onset under 16 ...years. The classification of the childhood chronic arthritis hasn't been solved.ObjectivesWe aimed that analysing the demographics, clinical and laboratory features, disease status, and subtype distributions of JIA according to the International League of Associations for Rheumatology (ILAR) criteria in Turkey.MethodsBetween March 2010 and February 2014 with this cross-sectional study, consecutive patients seen with JIA in selected 5 major centres. All patients were classified according to the ILAR criteria. Related to the disease status assessment we evaluate acute phase reactants, physician-patient-parent global assessment of overall disease activity (PGA)(21circle VAS), pain scale (10cm VAS), disease status, and Juvenile Arthritis Damage Index (JADI). We checked the correlations between physician-patient-parent global assessment (PGA) of overall disease activity, and disease status scores.ResultsIn this study, 208 (58.1%) females and 150 (41.9%) males were evaluated (Female/Male:1.39). The mean age of patients was 11.15±4.47 years. The distributions of JIA patients according to onset of disease were as follows: systemic 50 (13.9%), oligoarticular extended 35 (9.4%), oligoarticular persistent 104 (29%), rheumatoid factor (RF) positive polyarthritis 5 (1.2%), RF negative polyarthritis 89 (24.9%), enthesitis-related 51 (13.8%), psoriatic 15 (3.8%), unclassified group 9 (2.6%). The frequency of uveitis was 21 (15.1%) among all of the oligoarticular patients and 12 (5.5%) among in the other group. ANA was positive mainly among the oligoarticular onset patients. 16 patients also had FMF. Among systemic JIA patients, the frequency of macrophage activation syndrome was 24% (n=12).Related to the disease status of the subtypes of JIA revealed that while the RF positive polyarthritis was found to have the highest, and the unclassified group has the lowest activity score via using Articular JADI. The RF (-) polyarthritis was found to has the highest, and the unclassified group has the lowest activity score according to the Extra-articular JADI. Based on the last week's parent-patient-pain score, while the unclassified group has the highest, the psoriatic arthritis has the lowest activity score.Assessment of the disease status by the PGA was resulted the fact that there was statistically significant positive correlation between parent-patient (r=0.770), moderate positive correlation between physician-parent (r=0.456) and physician-patient (r=0.512). There was also statistically positive correlation between the patient and parent pain score (r=0.857).ConclusionsWe assessed the main clinical and laboratory features, and the disease activity status of the Turkish patients with childhood chronic arthritis. We evaluated the diagnoses and the subtype distributions according to ILAR classification.Disclosure of InterestNone declared
BackgroundJuvenile systemic sclerosis (jSSc) is an orphan autoimmune disease. Several adult publications looked at the differences between male and female patients with Systemic Sclerosis. There is ...rarity of data regarding this topic in pediatric jSSc. The juvenile scleroderma inception cohort (www.juvenilescleroderma.com) is a prospective standardized register for patients with jSSc.Objectivescomparison of patients characteristic at the time of inclusion in the registry who are male or female.MethodsPatients with jSSc were included worldwide to the juvenile scleroderma inception cohort. We compered the demographics and clinical characteristics of the male and female patients.ResultsUp till now 74 patients were enrolled, 54 with djSSc (76%) and 18 with ljSSc (24%). 14 (19%) of the patients were male (M) and 60 female (F) (81%). The mean disease duration at the time of inclusion was 9.3 in M and 9.2 in F patients. 74.4% of the M and 76.7% of the F had diffuse subset. The mean age of the onset of Raynaud symptomatic was 9.3 in M and 9.2 years in the F patients and the non-Raynaud symptomatic with 9.1 in M and 9.9 in F patients. At the time of the inclusion the mean modified Rodnan Skin Score was 20 in M and 15.1 in F patients. Anti-Scl 70 positivity was found in 42.9% in M and 32.1% in F patients. Anticentromere positivity occurred in 16.7% in M and 3.3% in F patients (p=0.027). Capillary changes were present in 50% of the M and 60% of F patients, but 50% in M and F had already history of ulcerations, but 28.6% in M and 15.5% in F had active ulceration. 57.1% of the M and 50% of the F patients had cardiopulmonary involvement. Six patients had pulmonary hypertension, they were all F. 75% of M and 46.7% of F patients had signs of interstitial lung disease on imaging. Renal involvement was around 7% in both sexes. 21.4% in M and 38.3% in F patients had gastrointestinal involvement. 92.9% of M and 55.9% in M patients had musculoskeletal involvement.ConclusionsWe present the data on the first 74 patients with jSSc included in our cohort. Patients with male sex have a more severe disease similar to adult male patients.Disclosure of InterestNone declared
BackgroundJuvenile systemic sclerosis (jSSc) is an orphan autoimmune disease. Several adult publications looked at the differences between limited (ljSSc) and diffuse subtype (djSSc). There is rarity ...of data regarding this topic in pediatric jSSc. The juvenile scleroderma inception cohort is a prospective standardized register for patients with jSSc.Objectivescomparison of patients characteristic at the time of inclusion with ljSSc and djSScMethodsWe compered the demographics and clinical characteristics of the ljSSc and djSSc.ResultsUp till now 74 patients were enrolled, 54 (76%) with djSSc and 18 with ljSSc (24%). 9% in djSSc and 25% in ljSSc showed overlap features. Disease duration at time of inclusion in the cohort was 3.7 years in the djSSc and 3.3 years in ljSSc. 82% in the djSSc and 78% in the ljSSc group were female. The mean age of the onset of Raynaud symptomatic was 9.0 years in the jdSSc and 9.9 years in ljSSc group and onset of the non-Raynaud symptomatic with 9.4 in djSSc and 10.6 ljSSc. At the time of inclusion the mean modified Rodnan Skin Score was 18.5 in the djSSc and 8,4 in ljSSc (p=0.0001). Anti-Scl 70 positivity was found in 31.5% of djSSc and 30.8% in ljSSc. Only 2 patient in the djSSc group and one in the ljSSc group was anticentromere positive. Capillary changes occurred in 60.7% in the djSSc and 50% in ljSSc, but 58.2% in djSSc and only 23.5% in ljSSc had already history of ulcerations (p=0.013) and 21.8% had active ulceration in the djSSc and 5.9% in the ljSSc. 33.3% of djSSc and 72.7% of ljSSc had cardiac involvement (p=0.027). pulmonary hypertension occurred in 57.1% djSSc and 18.2% in ljSSc. 63% in djSSc and 27.3% in ljSSc group had signs of interstitial lung disease on imaging (p=0.046). Renal involvement occurred in 7.1% djSSc and 5.6% in ljSSc. 39.3% of djSSc and 22.2% of ljSSc had gastrointestinal involvement. 56.4% in djSSc and 83.35 in ljSSc had musculoskeletal involvement (p=0.04).ConclusionsPatients with djSSc have younger age at onset, have more often capillary changes and active ulcerations, pulmonary hypertension and less gastorintestinal and joint involvement and more disease damage. The characteristics of the pediatric subtypes differs from adults with SSc, especially the high proportion of patients with diffuse subtype.Disclosure of InterestNone declared
BackgroundJuvenile systemic sclerosis (jSSc) is an orphan autoimmune disease. Currently just retrospective data exist regarding evolvement of organ involvement. In the retrospective studies ...assessment of the organ involvement is not standardized. Our project is the first one, where prospectively and with a standardized assessment data of jSSc patients are collected.Objectivesto learn about the characteristics and evolvement of jSScMethodsPatients with jSSc were recruited worldwide and were prospectively assessed, using the proposed standardized patient assessment protocol.Results26 centers from 17 countries applied to participate on the project. The assent and consent forms were translated into the local native languages. Up till now 74 patients were enrolled. Sixty (81%) of the 74 patients were female. The mean age of the onset of Raynaud symptomatic was 9.2 years (0.2 – 15.9). The mean age at the onset of the non-Raynaud symptomatic were 9,7 years (0.3 -15.9). 56 (76%) of the 74 have diffuse subtype, 10 (14%) of them have an overlap symptomatic. At the time of the inclusion the mean modified Rodnan Skin Score was 16.0. ANA positive were 55/71 (77%), 24/70 (34%) of them were anti-Scl 70 positive and 3/42 (7%) was anticentromere positive. 43/74 (58%) had already capillary changes and 36/72 (50%) inactive ulcerations, 13/72 (18%) had active ulceration at the time of the inclusion. 38/74 (51%) had cardiopulmonary involvement, 19/38 (50%) of had signs of interstitial lung disease on imaging, 18/42 (43%) had FVC <80% and 12/21 (57%) had DLCO <80%. 6/38 (16%) patients had pulmonary hypertension. 5/74 (7%) had renal involvement. 26/74 (35%) had gastrointestinal involvement, and 23/26 (88%) of them esophageal involvement. 46/73 (63%) had musculoskeletal involvement. 2/74 (3%) showed neurologic involvement. The mean CHAQ score was 0.4 (0–2.5). Patient global disease activity on VAS (0–100) was 44.9 and disease damage 41.6. Physician global of disease activity on VAS (0–100) was 39.7 and physician global of disease damage was 34.6.ConclusionsThe current recruitment data confirms that pediatric patients are different from the adult patients, there is a significantly higher proportion of diffuse subset patients with 81%. 14% of the patients have overlap features.Disclosure of InterestNone declared
Background There are a lot of effects of auto-inflammatory diseases (e.g. fear of attack, pain, fever, fatigue, problems at school) that are quite important to patients but have not been measured ...with the outcome instruments currently included in clinical trials of autoinflammatory diseases. Objectives The aim of this study is to develop and test a new multidimensional questionnaire for assessment of children with autoinflammatory disease (AID) in standard clinical care. Methods JAIMAR includes 16 parent or patient - centered measures and four dimensions that assess functional status, pain, therapeutic compliance and health-related quality of life with disease outcome. The JAIMAR is proposed for use as both a proxy-report and a patient self-report, with the suggested age range of 7-18 years for use as a self-report. The study was conducted in children with AID and their parents in seven different paediatric rheumatology center from Turkey. To validate the JAIMAR, the Outcome Measures in Rheumatology Clinical Trials (OMERACT) filter for outcome measures in rheumatology was applied. Results Completion and scoring appeared to be quick, requiring avarage15 minutes. The analysis data set included by the parents of 250 children with FMF in 351 visits and by 179 children in 187 visits. The median age of the children was 10.64±4.38 and female to male ratio was. The JAIMAR was found to be feasible and to possess face, content, criterion and construct validity. The cronbach's alpha coefficient for internal consistency for the JAIMAR dimensions were between for parents 0.677-0.998 and for children 0.507-0.986. Parents' proxy-reported and children's self-reported data were outstandingly concordant.The JAIMAR dimensions patient self-report and parent proxy-report cronbach's alpha values were between 0.770-0.989. Conclusions The development of the JAIMAR introduces a new and multi-dimensional approach in pediatric rheumatology practice. The JAIMAR provided thorough information for the study patients about recent medical history, attack status and current health status. It is valid tool for children with autoinflammatory disease and will help enhance the quality of care of in this group of patients References Kalkan G, Demirkaya E, Acikel CH, Polat A, Peru H, Karaoglu A, Sari E, Dursun I, Gok F, Ozen S; (FAVOR). Evaluation of the current disease severity scores in paediatric FMF: is it necessary to develop a new one? Rheumatology (Oxford). 2012, 51(4):743-8. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.5963
