333 research outputs found
Comparing ultraviolet light A photo(chemo)therapy with Methotrexate protocol in childhood localized scleroderma : evidence from systematic review and meta-analysis approach
No full textOBJECTIVE:
Localized scleroderma is a skin fibrosing disorder that, if untreated, may result in severe disability. The purpose of this systematic review is to compare the present evidence concerning the effectiveness of Methotrexate versus phototherapy, alone or associated with Psoralen, in childhood localized scleroderma.
METHOD:
A systematic search between January 1996 and May 2017 was performed to identify studies investigating the efficacy of Methotrexate (MTX) or phototherapy (UVA) for treating localized scleroderma with onset ≤18 years. Due to a lack of validated clinical criteria, four clinical response criteria were used to assess the treatment efficacy as primary outcome. We determined a combined estimate of the proportion of children responding to MTX and UVA.
RESULTS:
A total of 19 studies was included (8 MTX; 11 UVA). In the methotrexate group, 193 children were included in the analysis; in the phototherapy group, a total of 48 treated children. For both groups age, disease subtype, glucocorticoids (GCs) use, and side effects of treatment were also analyzed. The meta-analysis suggested that UVA and MTX protocols have both a favorable effect in active lesions of childhood localized scleroderma. However, MTX resulted significantly superior to UVA, with or without Psoralen.
CONCLUSION:
Our study supports the combination of MTX and GCs in patients with a high risk of complication. Phototherapy with UVA1 could represent a therapeutic option in patients with limited scleroderma, where lesions do not cross joints and they do not lead to potential cosmetic changes
A comparison between nailfold capillaroscopy patterns in adulthood in juvenile and adult-onset systemic sclerosis: A EUSTAR exploratory study
No full textOBJECTIVE Qualitative capillaroscopy patterns in juvenile- and adult-onset systemic sclerosis (SSc) were studied in adulthood using data from the EULAR Scleroderma Trials and Research (EUSTAR) database.
METHODS Data collected between June 2004 and April 2013 were examined with focus on capillaroscopy. In this retrospective exploratory study, series of patients with juvenile-onset SSc were matched with series of adult-onset SSc having the same gender and autoantibody profile.
RESULTS 30 of 123 patients with juvenile-onset and 2108 of 7133 with adult-onset SSc had data on capillaroscopy. Juvenile-onset SSc showed scleroderma pattern more frequently than adult-onset SSc (93.3% and 88%). The OR was 2.44 and 95% CI 0.57-10.41. An active scleroderma pattern was present in 58% of juvenile- and 61% of adult-onset SSc. The OR was 0.91 and 95% CI 0.28-2.93. The late scleroderma pattern was present in 61% of juvenile- and 55.5% of adult-onset SSc. The OR was 1.06 and 95% CI 0.34-3.56.
CONCLUSION This is the first exploratory study on the comparison of capillaroscopy between juvenile- and adult-onset SSc in adulthood. Juvenile-onset SSc had an increase prevalence of scleroderma pattern, but a similar distribution of the three patterns was suggested. Further studies are needed to define this issue
Nailfold Capillaroscopy Patterns in Adulthood are Similar in Patients with Juvenile and Adult-Onset Systemic Sclerosis : A Eustar Case-Control Study : OP0221
No full textBackground: Nailfold capillaroscopy is a useful investigation to identify patients with Raynaud's phenomenon secondary to systemic sclerosis (SSc) because typical capillary changes can be clearly demonstrated in adults as well as in children. However, in juvenile-onset SSc the overall capillaroscopic pattern is called “scleroderma pattern”, the classification in “early”, “active” and “late” pattern (1) has never been applied, and differences in microvascular abnormalities between juvenile- and adult-onset SSc have never been explored.
Objectives: Microvascular abnormalities described as qualitative overall patterns by nailfold capillaroscopy in patients with juvenile- and adult-onset systemic sclerosis (SSc) were studied in adulthood using data from the EULAR Scleroderma Trials and Research (EUSTAR) database.
Methods: Data collected between June 2004 and April 2013 in the EUSTAR registry were examined with focus on capillaroscopy. In this case-control study, adult patients with juvenile-onset SSc with available data on capillaroscopy were matched with corresponding patients with adult-onset SSc having the same gender and autoantibody profile. Patients aged ≥65 year-old at disease onset were excluded.
Results: 30 adult patients with juvenile-onset SSc and 2108 patients with adult-onset SSc were included in the analysis. The mean age at the visit of patients with adult- and juvenile-onset SSc was 52.91±12.6 and 29.56±10.71 year-old respectively. Mean age at the onset of Raynaud's phenomenon was 38.60±12.23 year-old in adult-onset and 11.36±5.12 year-old in juvenile-onset SSc. Similar distribution of modified Rodnan skin score between adult- and juvenile-onset SSc was observed (8.85±7.88 and 10.25±9.74 respectively). The majority of patients had a scleroderma pattern and it was equally distributed among early, active and late pattern in juvenile-onset SSc and adult-onset SSc. Results of univariate analysis showed that in patients with juvenile-onset SSc showed the presence of scleroderma pattern more frequently than adult-onset SSc (OR=2.44, p=0.17), even if not significant. No difference was observed in the distribution of early, active and late pattern between the two groups (OR estimates are near 1.0). Juvenile-onset SSc and adult-onset SSc shared similar capillaroscopy patterns and organ involvement, except for a slightly decreased frequency of oesophageal involvement (46.66% vs 66.22%) and increased lung fibrosis assessed by HRCT (18.13% vs 42.85%) in juvenile-onset SSc (OR=0.46 p=0.04 and OR 2.97, p=0.009 respectively).
Conclusions: This study is the largest series of adult patients with juvenile-onset SSc in which capillaroscopy has been performed and classified using the three qualitative patterns usually applied in adult-onset SSc. We showed that the microvascular involvement documented by nailfold capillaroscopy patterns in adulthood was similar in patients with juvenile- and adult-onset SSc. The possibility to use the same classification in three patterns applied in adults may be useful to standardise this examination.
References:
Cutolo M, Pizzorni C, Tuccio M, Burroni A, Craviotto C, Basso M, et al. Nailfold videocapillaroscopic patterns and serum autoantibodies in systemic sclerosis. Rheumatology (Oxford). 2004;43(6):719-26
Occurrenceof arthritis is in 37%of the patients without overlying skin involvementinjuvenile localized scleroderma. Summary of the extracutaneous involvement in a monocentric cohort
No full textA cross-sectional, international survey on non-invasive techniques to assess the microcirculation in patients with Raynaud's phenomenon (Sunshine Survey)
No full textBackground: Microcirculatory impairment in patients with Raynaud's phenomenon (RP) may be assessed by different techniques, but real-life data concerning their roles and current usage are not available.
Objectives: To obtain an overview of the specific techniques which may be used for the assessment of adult patients with RP in clinical and research settings: nailfold videocapillaroscopy (NVC), dermoscopy, stereomicroscopy, and digital USB microscopy, laser Doppler flowmetry, imaging, and anemometry/velocimetry, laser Speckle Contrast Analysis (LASCA), thermographic imaging, upper limb arterial Doppler ultrasound.
Methods: This survey was conducted online between October and December 2015 on behalf of EULAR study group on Microcirculation in Rheumatic Diseases (SG_MC/RD). Emails with a link to the survey were sent to physicians from the European Scleroderma Trials and Research group (EUSTAR) and SG_MC/RD mailing lists. Of those e-mailed, 418 were physicians looking after adult patients, and this group was considered in the following descriptive analysis.
Results: Of the 418 eligible physicians, 107 completed the survey, giving an overall response rate of 25.6%. Among the respondents 89 (83.2%) were rheumatologists, 74 (69.2%) European; 87 (81.3%) were practising for more than 10 years and 50% looked after between 31 and 60 patients per year with primary and/or secondary RP. The most routinely performed technique was NVC (63/107, 58.9%) both by rheumatologists and non-rheumatologists (54/89, 60.7% and 9/18, 50.0%). NVC was reported as the most available technique (93/107, 86.9%), and available in the place of work in 78/107 (72.9%) among both rheumatologists and non-rheumatologists. Nailfold capillaroscopy was the most frequently performed by the physician him/herself by using different types of equipment relating to availability: NVC 64/94 (68.0%), dermoscopy 38/63 (60.3%), stereomicroscopy 31/42 (73.8%), and digital USB microscopy 34/39 (87.1%). Most rheumatologists reported high levels of “appropriateness” for NVC in both clinical and research settings for global assessment (86/88, 97.7% clinical setting, 87/88, 98.9% research setting), and differential diagnosis of primary and secondary RP (clinical and research setting both 84/87 96.5%). In clinical setting NVC showed the highest percentage of appropriateness for monitoring primary RP (84/88, 95.4%), RP secondary to connective tissue diseases other than systemic sclerosis (82/87, 94.2%) and to systemic sclerosis (87/87, 100%). All techniques other than capillaroscopy reached a consensus lower than 2/3 of respondents based on their knowledge/experience. In research setting, all techniques were judged as potentially useful with a consensus more than 2/3 of respondents.
Conclusions: Of all the different techniques upon which opinion was sought, nailfold capillaroscopy was the one most used by physicians looking after adult patients in both clinical and research settings, the majority of whom use NVC in their everyday practice. The low proportion of clinicians using other techniques suggests that these are currently confined to specialist centres
Tumor necrosis factor-alpha blocker in treatment of juvenile idiopathic arthritis-associated uveitis refractory to second-line agents: results of a multinational survey.
No full textT Objective. Uveitis occurs in l09i»l5% of patients with juvenile idiopathic arthritis (JIA). If topical treatment fails, second—Iine agents are used to control the dismse. However, some patients need the addition of tumor necrosis factor»ot (TNF»ot) antagonist (anti-TNF). We organized a cross»sectional cohort to investigate use and efficacy of anti—TNF treatment in patients with JIA-associated uveitis. Methods. The international pediatric rheumatology community was queried about the use and efficacy of anti-TNF in treatment of HA»associated uveitis using an E-mail survey.
Results. Of the 33 responding centers following 884 patients with uveitis, only 15 centers, following 404 patients, were using anti—TNF for this indication. A total of 47 patients with JIA-related uveitis
treated with anti-TNF because of an insufficient response to previous therapy were reported. The mean age of the patients was 12.5 years The mean duration from onset of uveitis to start of anti-TNF t;reat~
ment was 45.1 months. Three different anti-TNF agents were used: etanercept in 34 cases, infliximab in 25 cases, and adalimumab in 3 cases. In 12 of the 34 patients etanercept was inefficacious and
patients were switched to inflixirnab. The final response was rated according to a composite index as 53%/ 12%/32%, and according to physician rating as 47%/ l2%/38% representing good, moderate, and
poor, respectively. in the etaneicept group; and 70%/30%/0% and 68%/24%/0% in the intliximab group. All 3 patients taking adaliniumab were responders. lntliximab was statistically significantly more efficacious for the treatment of HA-associated uveitis than etanercept (chi-square p = 0.004).
Conclusion. Anti—TNF seems to be an effective treatment for refractory .llA—associated uveitis. In this cohort infliximab was more efficacious than etanercepr
Juvenile and young adult-onset systemic sclerosis share the same organ involvement in adulthood: Data from the Eustar database
No full textObjective. The aim of the present study was to explore the long-term outcome and clinical characteristics of adult patients with juvenile onset in the EULAR Scleroderma Trials and Research (EUSTAR) cohort and compare them with adult patients with onset between 20 and 40 years of age.Methods. From the EUSTAR SSc cohort two patient groups were analysed: patients with juvenile SSc (jSSc) who are adults at present, and patients diagnosed between the age of 20 and 40 years (aSSc). Demographic data of the patients, organ involvement and outcome of the disease were examined using the Minimal Essential Data Set database system.Results. From 5000 patients in the EUSTAR cohort, 60 patients (1.2%) with jSSc and 910 patients (18%) with aSSc were selected according the inclusion criteria. In the jSSc group, the mean age of disease onset was 12.4 years (range 2-15.9 years), and in the aSSc group, the mean age was 32 years (range 20-40 years). Disease subsets were similar. The antibody profile was also comparable except for ACAs, which were positive in 5% of the jSSc group and 26.9% of the aSSc group (P < 0.005). Organ involvement (lung, kidney, joint, muscle and heart) was similar in the two groups of patients at the time of the last follow-up.Conclusion. The subset distribution in the jSSc and aSSc cohorts was found to be similar. Only the frequency of ACAs was significantly lower in the jSSc, which supports the hypothesis that the SSc patients with paediatric onset in the adult cohort may represent a distinct subgroup of the complete cohort of paediatric patients. © The Author 2012. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved
PReS-FINAL-2121: Results for 6 minute walk values in healthy German children show similar results as from Britain
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