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Objective: To develop a standardized steroid dosing regimen (SSR) for physicians treating childhood-onset systemic lupus erythematosus (SLE) complicated by lupus nephritis (LN), using consensus formation methodology. Methods: Parameters influencing corticosteroid (CS) dosing were identified (step 1). Data from children with proliferative LN were used to generate patient profiles (step 2). Physicians rated changes in renal and extrarenal childhood-onset SLE activity between 2 consecutive visits and proposed CS dosing (step 3). The SSR was developed using patient profile ratings (step 4), with refinements achieved in a physician focus group (step 5). A second type of patient profile describing the course of childhood-onset SLE for ≥4 months since kidney biopsy was rated to validate the SSR-recommended oral and intravenous (IV) CS dosages (step 6). Patient profile adjudication was based on majority ratings for both renal and extrarenal disease courses, and consensus level was set at 80%. Results: Degree of proteinuria, estimated glomerular filtration rate, changes in renal and extrarenal disease activity, and time since kidney biopsy influenced CS dosing (steps 1 and 2). Considering these parameters in 5,056 patient profile ratings from 103 raters, and renal and extrarenal course definitions, CS dosing rules of the SSR were developed (steps 3–5). Validation of the SSR for up to 6 months post–kidney biopsy was achieved with 1,838 patient profile ratings from 60 raters who achieved consensus for oral and IV CS dosage in accordance with the SSR (step 6). Conclusion: The SSR represents an international consensus on CS dosing for use in patients with childhood-onset SLE and proliferative LN. The SSR is anticipated to be used for clinical care and to standardize CS dosage during clinical trials. © 2021, American College of Rheumatology

Objective: To investigate the pharmacokinetics, effectiveness, and safety of subcutaneous (SC) abatacept treatment over 24 months in patients with polyarticular-course juvenile idiopathic arthritis (JIA). Methods: In this phase III, open-label, international, multicenter, single-arm study, patients with polyarticular JIA (cohort 1, ages 6–17 years and cohort 2, ages 2–5 years) in whom treatment with ≥1 disease-modifying antirheumatic drug was unsuccessful received weight-tiered SC abatacept weekly: 10 to

ObjectivesTo investigate whether MTX should be combined with an additional DMARD and bridging glucocorticoids as initial treatment for patients with early RA to induce an effective long-term response.MethodsThe Care in early RA study is a two-year investigator-initiated pragmatic multicentre randomized trial. Early RA patients, naïve to DMARDs and glucocorticoids, were stratified based on prognostic factors. High-risk patients were randomized to COBRA-Classic (n = 98): MTX, sulfasalazine, prednisone step-down from 60 mg; COBRA-Slim (n = 98): MTX, prednisone step-down from 30 mg; or COBRA-Avant-Garde (n = 93): MTX, leflunomide, prednisone step-down from 30 mg. Low-risk patients were randomized to COBRA-Slim (n = 43); or Tight Step Up (TSU) (n = 47): MTX without prednisone. Clinical/radiological outcomes at year 2, sustainability of response, safety and treatment adaptations were assessed.ResultsIn the high-risk group 71/98 (72%) patients achieved a DAS28-CRP < 2.6 with COBRA-Slim compared with 64/98 (65%) with COBRA-Classic and 69/93 (74%) with COBRA-Avant-Garde (P = 1.00). Other clinical/radiological outcomes and sustainability of response were similar. COBRA-Slim treatment resulted in less therapy-related adverse events compared with COBRA-Classic (P = 0.02) or COBRA-Avant-Garde (P = 0.005). In the low-risk group, 29/43 (67%) patients on COBRA-Slim and 34/47 (72%) on TSU achieved a DAS28-CRP < 2.6 (P = 1.00). On COBRA-Slim, low-risk patients had lower longitudinal DAS28-CRP scores over 2 years, a lower need for glucocorticoid injections and a comparable safety profile compared with TSU.ConclusionAll regimens combining DMARDs with glucocorticoids were effective for patients with early RA up to 2 years. The COBRA-Slim regimen, MTX monotherapy with glucocorticoid bridging, provided the best balance between efficacy and safety, irrespective of patients’ prognosis.Trial registrationClinicalTrials.gov, http://www.clinicaltrials.gov, NCT01172639.

Patients with polyarticular-course juvenile idiopathic arthritis (pJIA), receiving disease-modifying anti-rheumatic drugs with immunosuppressive effects, may be at increased risk of vaccine-preventable infections. This substudy assessed protective antibody responses to diphtheria and tetanus vaccination given prior to study enrolment in patients with pJIA.

Report the 2-year efficacy and safety of tocilizumab in patients with polyarticular-course juvenile idiopathic arthritis (pcJIA).

The anti-interleukin-6 receptor-alpha antibody tocilizumab was approved for intravenous (IV) injection in the treatment of patients with systemic juvenile idiopathic arthritis (sJIA) aged 2 to 17 years based on results of a randomized controlled phase 3 trial. Tocilizumab treatment in systemic juvenile idiopathic arthritis (sJIA) patients younger than 2 was investigated in this open-label phase 1 trial and compared with data from the previous trial in patients aged 2 to 17 years.

Novartis Pharma Background: Canakinumab is a human anti-interleukin-1 beta (IL-1 beta) monoclonal antibody neutralizing IL-1 beta-mediated pathways. We sought to characterize the molecular response to canakinumab and evaluate potential markers of response using samples from two pivotal trials in systemic juvenile idiopathic arthritis (SJIA). Methods: Gene expression was measured in patients with febrile SJIA and in matched healthy controls by Affymetrix DNA microarrays. Transcriptional response was assessed by gene expression changes from baseline to day 3 using adapted JIA American College of Rheumatology (aACR) response criteria (50 aACR JIA). Changes in pro-inflammatory cytokines IL-6 and IL-18 were assessed up to day 197. Results: Microarray analysis identified 984 probe sets differentially expressed (>= 2-fold difference P < 0.05) in patients versus controls. Over 50% of patients with >= 50 aACR JIA were recognizable by baseline expression values. Analysis of gene expression profiles from patients achieving = 50 aACR JIA response at day 15 identified 102 probe sets differentially expressed upon treatment (>= 2-fold difference P < 0.05) on day 3 versus baseline, including IL-1 beta, IL-1 receptors (IL1-R1 and IL1-R2), IL-1 receptor accessory protein (IL1-RAP), and IL-6. The strongest clinical response was observed in patients with higher baseline expression of dysregulated genes and a strong transcriptional response on day 3. IL-6 declined by day 3 (>= 8-fold decline P < 0.0001) and remained suppressed. IL-18 declined on day 57 (>= 1.5-fold decline, P

8958 Aedia funesta (Esper, 1786) Basel (BS), Hörnli, 2.7.2014 und Münchenstein (BL), Merian Gärten, 17.7.2013 / 2.7.2014. Vor 1980 galt die Art in der Region als sehr selten. Kurz nach 2000 wurden einzelne Funde bekannt. In den letzten Jahren hat sich die Art stark verbreitet und kann jetzt praktisch überall, insbesondere im Siedlungsbereich, nachgewiesen werden., Published as part of Joos, R Enato, Fritsch, Dieter, Bryner, Rudolf, Billen, Wolfgang & Wicki, Christoph, 2018, Vielfalt der nachtaktiven Gross- und Kleinschmetterlinge in zwei Parkanlagen bei Basel, pp. 57-71 in Entomo Helvetica 11 on page 63, DOI: 10.5169/seals-986024, http://zenodo.org/record/8034307

6684b Diplopseustis perieresalis (Walker, 1859), Abb. 3h Münchenstein (BL), Merian Gärten, 28.10.2015. Nach Graubünden (Schmid 2007) ist dies erst der zweite Nachweis für die Schweiz. Die ursprünglich in Australien und Neuseeland verbreitete Art wurde vermutlich mit Zierpflanzenimporten (möglicherweise Palmen) in die westpaläarktische Region verschleppt und breitet sich nun aus. Nachweise liegen inzwischen auch aus Grossbritannien, Holland, Belgien, Frankreich, Deutschland, Österreich, Spanien und Portugal vor. Grossschmetterlinge, Published as part of Joos, R Enato, Fritsch, Dieter, Bryner, Rudolf, Billen, Wolfgang & Wicki, Christoph, 2018, Vielfalt der nachtaktiven Gross- und Kleinschmetterlinge in zwei Parkanlagen bei Basel, pp. 57-71 in Entomo Helvetica 11 on page 62, DOI: 10.5169/seals-986024, http://zenodo.org/record/8034307, {"references":["Schmid J. 2007. Schweizer Erstfunde von Kleinschmetterlingen aus Graubunden (Lepidoptera: Nepticulidae, Tineidae, Bucculatricidae, Agonoxenidae, Blastobasidae, Gelechiidae, Tortricidae, Pyralidae). Mitteilungen der Entomologischen Gesellschaft Basel 57 (2 - 4): 124 - 131."]}

9651 Aporophyla nigra (Haworth, 1809) Münchenstein (BL), Merian Gärten, 28.10.2015. Die Art ist stark wärmeliebend. In der Region sind nur ganz wenige Nachweise bekannt., Published as part of Joos, R Enato, Fritsch, Dieter, Bryner, Rudolf, Billen, Wolfgang & Wicki, Christoph, 2018, Vielfalt der nachtaktiven Gross- und Kleinschmetterlinge in zwei Parkanlagen bei Basel, pp. 57-71 in Entomo Helvetica 11 on page 63, DOI: 10.5169/seals-986024, http://zenodo.org/record/8034307

Joos, R Enato, Fritsch, Dieter, Bryner, Rudolf, Billen, Wolfgang, Wicki, Christoph (2018): Vielfalt der nachtaktiven Gross- und Kleinschmetterlinge in zwei Parkanlagen bei Basel. Entomo Helvetica 11: 57-71, DOI: 10.5169/seals-986024

2060 Spuleria flavicaput (Haworth, 1828), Abb. 3f Münchenstein (BL), Merian Gärten, 27.5.2015. Von dieser Art liegen nur ganz wenige Beobachtungen aus der Schweiz vor. Die meisten stammen aus der ersten Hälfte des letzten Jahrhunderts. Neben dem jetzigen Fund in Basel sind neuere Nachweise aus Graubünden und aus dem Berner Jura bekannt. Die Raupen entwickeln sich im Innern von Weissdorn-Zweigen Crataegus sp., Published as part of Joos, R Enato, Fritsch, Dieter, Bryner, Rudolf, Billen, Wolfgang & Wicki, Christoph, 2018, Vielfalt der nachtaktiven Gross- und Kleinschmetterlinge in zwei Parkanlagen bei Basel, pp. 57-71 in Entomo Helvetica 11 on page 62, DOI: 10.5169/seals-986024, http://zenodo.org/record/8034307

9841 Gortyna flavago (Denis & Schiffermüller, 1775) Münchenstein (BL), Merian Gärten, 15.7.2015. Die Art erlitt in den letzten Jahren grosse Arealverluste. Seit 2000 ist aus der Region nur ein Nachweis bekannt., Published as part of Joos, R Enato, Fritsch, Dieter, Bryner, Rudolf, Billen, Wolfgang & Wicki, Christoph, 2018, Vielfalt der nachtaktiven Gross- und Kleinschmetterlinge in zwei Parkanlagen bei Basel, pp. 57-71 in Entomo Helvetica 11 on page 63, DOI: 10.5169/seals-986024, http://zenodo.org/record/8034307

707 Monopis imella (Hübner, 1813), Abb. 3b Neben wenigen Angaben aus der ersten Hälfte des letzten Jahrhunderts liegen jetzt wieder einzelne aktuelle Beobachtungen vor, darunter zwei im Rahmen dieser Untersuchung aus Münchenstein (BL), Merian Gärten, 30.4.2014 und 20.5.2015. Die Raupe lebt an Detritus, Kot von Tieren, auch in Vogelnestern., Published as part of Joos, R Enato, Fritsch, Dieter, Bryner, Rudolf, Billen, Wolfgang & Wicki, Christoph, 2018, Vielfalt der nachtaktiven Gross- und Kleinschmetterlinge in zwei Parkanlagen bei Basel, pp. 57-71 in Entomo Helvetica 11 on page 60, DOI: 10.5169/seals-986024, http://zenodo.org/record/8034307

10010 Mythimna obsoleta (Hübner, 1803) Münchenstein (BL), Merian Gärten, 25.6.2014. In der Region gibt es nur wenige Nachweise. Die Art wandert und wird meist nur anhand einzelner Falter nachgewiesen., Published as part of Joos, R Enato, Fritsch, Dieter, Bryner, Rudolf, Billen, Wolfgang & Wicki, Christoph, 2018, Vielfalt der nachtaktiven Gross- und Kleinschmetterlinge in zwei Parkanlagen bei Basel, pp. 57-71 in Entomo Helvetica 11 on page 63, DOI: 10.5169/seals-986024, http://zenodo.org/record/8034307

9490 Mormo maura (Linnaeus, 1758) Münchenstein (BL), Merian Gärten, 4.9.2013 / 10.9.2014. Diese grosse Eulenfalterart lebt bevorzugt in eher feuchten Gebieten mit Gehölzen entlang von Bächen und Gewässern. Die Art wird nicht häufig nachgewiesen, da die Falter kaum Lichtquellen anfliegen., Published as part of Joos, R Enato, Fritsch, Dieter, Bryner, Rudolf, Billen, Wolfgang & Wicki, Christoph, 2018, Vielfalt der nachtaktiven Gross- und Kleinschmetterlinge in zwei Parkanlagen bei Basel, pp. 57-71 in Entomo Helvetica 11 on page 63, DOI: 10.5169/seals-986024, http://zenodo.org/record/8034307

8747 Gluphisia crenata (Esper, 1785) Münchenstein (BL), Merian Gärten, 18.6.2014. Seit 1980 erlitt die Art in der Region grosse Arealverluste. Nach 2000 sind nur wenige Nachweise bekannt., Published as part of Joos, R Enato, Fritsch, Dieter, Bryner, Rudolf, Billen, Wolfgang & Wicki, Christoph, 2018, Vielfalt der nachtaktiven Gross- und Kleinschmetterlinge in zwei Parkanlagen bei Basel, pp. 57-71 in Entomo Helvetica 11 on page 62, DOI: 10.5169/seals-986024, http://zenodo.org/record/8034307

7998 Thalera fimbrialis (Scopoli, 1763) Basel (BS), Hörnli, 8.7.2015. Die Art kam ursprünglich vor allem in Halbtrockenrasen vor. In neuerer Zeit wurde sie aber nur noch in der Ebene in tiefen Lagen nachgewiesen., Published as part of Joos, R Enato, Fritsch, Dieter, Bryner, Rudolf, Billen, Wolfgang & Wicki, Christoph, 2018, Vielfalt der nachtaktiven Gross- und Kleinschmetterlinge in zwei Parkanlagen bei Basel, pp. 57-71 in Entomo Helvetica 11 on page 62, DOI: 10.5169/seals-986024, http://zenodo.org/record/8034307

1120 Caloptilia fribergensis (Fritzsche, 1871), Abb. 3c Basel (BS), Hörnli, 14.5.2014 / 16.7.2014 und Münchenstein (BL), Merian Gärten, 15.7.2015. Es gibt bisher nur wenige Nachweise in der Schweiz. Die Raupen leben in Blattfalten von Berg-Ahorn Acer pseudoplatanus und anderen Ahorn-Arten., Published as part of Joos, R Enato, Fritsch, Dieter, Bryner, Rudolf, Billen, Wolfgang & Wicki, Christoph, 2018, Vielfalt der nachtaktiven Gross- und Kleinschmetterlinge in zwei Parkanlagen bei Basel, pp. 57-71 in Entomo Helvetica 11 on page 60, DOI: 10.5169/seals-986024, http://zenodo.org/record/8034307

7630 Apeira syringaria (Linnaeus, 1758) Basel (BS), Hörnli, und Münchenstein (BL), Merian Gärten. Die Art fliegt von Ende Juni bis Ende August und war früher häufig. Sie hat aber in den letzten Jahren grosse Arealverluste erlitten und konnte nach 2000 nur noch an wenigen Orten in der Region nachgewiesen werden., Published as part of Joos, R Enato, Fritsch, Dieter, Bryner, Rudolf, Billen, Wolfgang & Wicki, Christoph, 2018, Vielfalt der nachtaktiven Gross- und Kleinschmetterlinge in zwei Parkanlagen bei Basel, pp. 57-71 in Entomo Helvetica 11 on page 62, DOI: 10.5169/seals-986024, http://zenodo.org/record/8034307

1122 Caloptilia hemidactylella (Denis & Schiffermüller, 1775) , Abb. 3d Münchenstein (BL), Merian Gärten, 11.6.2014. Die Art wurde in der Schweiz relativ selten nachgewiesen, aber dennoch etwas zahlreicher als C. fribergensis. Ihre Raupen entwickeln sich ebenfalls in Blattfalten von Berg-Ahorn Acer pseudoplatanus., Published as part of Joos, R Enato, Fritsch, Dieter, Bryner, Rudolf, Billen, Wolfgang & Wicki, Christoph, 2018, Vielfalt der nachtaktiven Gross- und Kleinschmetterlinge in zwei Parkanlagen bei Basel, pp. 57-71 in Entomo Helvetica 11 on page 60, DOI: 10.5169/seals-986024, http://zenodo.org/record/8034307

8904 Dysgonia algira (Linnaeus, 1767) Münchenstein (BL), Merian Gärten, 25.6.2014. Die Art ist im Raum Pratteln–Birsfelden seit 1986 bekannt und galt als südlicher Zuwanderer. Heute wird sie als bodenständig betrachtet und kann regelmässig nachgewiesen werden., Published as part of Joos, R Enato, Fritsch, Dieter, Bryner, Rudolf, Billen, Wolfgang & Wicki, Christoph, 2018, Vielfalt der nachtaktiven Gross- und Kleinschmetterlinge in zwei Parkanlagen bei Basel, pp. 57-71 in Entomo Helvetica 11 on page 63, DOI: 10.5169/seals-986024, http://zenodo.org/record/8034307

4999 Epiblema costipunctana (Haworth, 1811), Abb. 3g Basel (BS), Hörnli, 10.6.2015. Bis Mitte des letzten Jahrhunderts liegen für diese seltene Art einige Funddaten aus der Schweiz vor. Seither gibt es nur noch vereinzelte Beobachtungen. Dieser neue Nachweis ist erfreulich., Published as part of Joos, R Enato, Fritsch, Dieter, Bryner, Rudolf, Billen, Wolfgang & Wicki, Christoph, 2018, Vielfalt der nachtaktiven Gross- und Kleinschmetterlinge in zwei Parkanlagen bei Basel, pp. 57-71 in Entomo Helvetica 11 on page 62, DOI: 10.5169/seals-986024, http://zenodo.org/record/8034307

9424 Platypergea kadenii (Freyer, 1836) Basel (BS), Hörnli, und Münchenstein (BL), Merian Gärten. Die Flugzeit war in den Jahren 2013–2015 von Ende Juni bis Mitte September. Die Art wurde in der Region im Jahre 2001 erstmals nachgewiesen. Seither hat sie ihr Areal erweitert und kann vor allem in Siedlungsgebieten gefunden werden., Published as part of Joos, R Enato, Fritsch, Dieter, Bryner, Rudolf, Billen, Wolfgang & Wicki, Christoph, 2018, Vielfalt der nachtaktiven Gross- und Kleinschmetterlinge in zwei Parkanlagen bei Basel, pp. 57-71 in Entomo Helvetica 11 on page 63, DOI: 10.5169/seals-986024, http://zenodo.org/record/8034307

Introduction Considering a lack of efficacy data in patients with early rheumatoid arthritis (eRA) presenting without classical markers of poor prognosis, we compared methotrexate (MTX) with or without step-down glucocorticoids in the CareRA trial. Methods Disease-modifying antirheumatic drug–naïve patients with eRA were stratified into a low-risk group based on prognostic markers that included non-erosiveness, anti–citrullinated protein antibodies and rheumatoid factor negativity and low disease activity (Disease Activity Score in 28 joints based on C-reactive protein (DAS28(CRP)) ≤3.2). Patients were randomized to 15 mg of MTX weekly (MTX with tight step-up (MTX-TSU)) or 15 mg of MTX weekly with prednisone bridging, starting at 30 mg and tapered to 5 mg daily from week 6 (COmbinatie therapie bij Reumatoïde Artritis (COBRA Slim)). A TSU approach was applied. Outcomes assessed were DAS28(CRP)-determined remission, cumulative disease activity, Health Assessment Questionnaire (HAQ) scores and adverse events (AEs) after 16 treatment weeks. Results We analyzed 43 COBRA Slim and 47 MTX-TSU patients and found that 65.1% in the COBRA Slim group and 46.8% in the MTX-TSU group reached remission (P = 0.081). Mean ± standard deviation area under the curve values of DAS28(CRP) were 13.84 ± 4.58 and 11.18 ± 4.25 for the MTX-TSU and COBRA Slim patients, respectively (P = 0.006). More COBRA Slim patients had an HAQ score of 0 (51.2% versus 23.4%, P = 0.006) at week 16. Therapy-related AEs between groups did not differ. Conclusion In patients with low-risk eRA, MTX with step-down glucocorticoid bridging seems more efficacious than MTX step-up monotherapy, with a comparable number of AEs observed over the first 16 treatment weeks. Trial registration EU Clinical Trials Register Identifier: EudraCT number 2008-007225-39. Registered 5 November 2008. Electronic supplementary material The online version of this article (doi:10.1186/s13075-015-0611-8) contains supplementary material, which is available to authorized users.

Objectives To determine whether baseline demographic, clinical, articular and laboratory variables predict methotrexate (MTX) poor response in polyarticular-course juvenile idiopathic arthritis. Methods Patients newly treated for 6 months with MTX enrolled in the Paediatric Rheumatology International Trials Organization (PRINTO) MTX trial. Bivariate and logistic regression analyses were used to identify baseline predictors of poor response according to the American College of Rheumatology pediatric (ACR-ped) 30 and 70 criteria. Results In all, 405/563 (71.9%) of patients were women; median age at onset and disease duration were 4.3 and 1.4 years, respectively, with anti-nuclear antibody (ANA) detected in 259/537 (48.2%) patients. With multivariate logistic regression analysis, the most important determinants of ACR-ped 70 non-responders were: disease duration >1.3 years (OR 1.93), ANA negativity (OR 1.77), Childhood Health Assessment Questionnaire (CHAQ) disability index> 1.125 (OR 1.65) and the presence of right and left wrist activity (OR 1.55). Predictors of ACR-ped 30 non-responders were: ANA negativity (OR 1.92), CHAQ disability index> 1.14 (OR 2.18) and a parent's evaluation of child's overall wellbeing