doi: 10.3899/jrheum.161351, 62. MeSH Der in wöchentlichen Sitzungen stattfindende interdisziplinäre Austausch ermöglicht eine ganzheitliche Betreuung durch Berücksichtigung medizinischer, sozialer und psychologischer Faktoren. Other tools such as Simple Measure of Impact of Lupus Erythematosus in Youngsters (SMILEY), which is not available in German, and European Consensus Lupus Activity Measurement (ECLAM) (10% each) and British Isles Lupus Assessment Group (BILAG) index (5%) were considered less relevant. KV drafted the manuscript. Suggested corticosteroid induction therapies for proliferative lupus nephritis class III or IV (adapted from Refs. Open-ended comments were analyzed. doi: 10.1191/0961203305lu2076oa, 52. (2002) 46:2121–31. Other suggested additional immunosuppressants (multiple answers possible, see Supplementary Appendix for dosing regimens) were cyclophosphamide (CP) [n = 7 (17%), with no clear preference for the preferred dosage], azathioprine (AZA), and rituximab (RTX) (n = 3 each). Arthritis Rheum. In up to 20% of all SLE patients, the onset of the disease occurs in childhood or adolescence (2). Consensus protocols for the diagnosis and management of the hereditary autoinflammatory syndromes CAPS, TRAPS and MKD/HIDS: a German PRO-KIND initiative. doi: 10.1177/0961203318801526, 27. (2006) 15:198–206. 49. Finally, the role of adherence and therapeutic drug monitoring to optimize treatment with MMF, and the use of “multitarget therapy” (i.e., MMF in combination with a calcineurin inhibitor) for induction of LN were not included in this survey, as these measures have only recently gained attention. Eur J Rheumatol. A 6-year prospective study in a cohort of 228 patients with lupus nephritis. The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. There was general agreement that patients should receive HCQ as a concomitant therapy for LN (Table 7). However, normalization of urine sediment and normalization of serum complement C3 were also considered important decision tools (see Table 5). Arthritis Care Res. Ann Rheum Dis. The use of high-dose intravenous CP (0.5–0.75 g/m2 monthly for 6 months) was recommended to be reserved for adult patients with proliferative LN class III/IV showing unfavorable clinical (nephritic urine sediment and impaired renal function with an eGFR between 25 and 80 ml/min/1.73 m2), or histologic (crescents or necrosis in >25% of glomeruli) prognostic factors (21). All authors critically revised the manuscript and approved the final draft. Haffner D, Hoyer PF, Zimmerhackl LB, Tönshoff B, Ehrich JHH, Gahr M, et al. (2011) 7:227–41. (2020) 18:17. doi: 10.1186/s12969-020-0409-3, 11. Their task is to develop T2T protocols for the most prevalent pediatric rheumatic diseases in Germany, some of which have been published (9–15). 55% of respondents would suggest consulting a pediatric rheumatologist, which suggests that, depending on the presentation of the patient, pediatric rheumatologists are less frequently involved in the initial diagnosis and treatment planning. Conclusion: The majority of pediatric rheumatologists and nephrologists in Germany and Austria propose the use of corticosteroids, most often in combination with either MMF or CP, for treatment of proliferative LN in children. (2019) 28:613–20. Am J Nephrol. Would you like email updates of new search results? Houssiau FA, Vasconcelos C, D’Cruz D, Sebastiani GD, Garrido Ed Ede R, Danieli MG, et al. eCollection 2023. ∘ Indication for kidney biopsy in SLE patients. Objectives To determine the influence of HLA-B27 positivity on risk of developing chronic nonbacterial osteomyelitis (CNO). 10 Center for Pediatric Rheumatology, Olgahospital, Stuttgart, Germany 11 German Rheumatism Research Center, Leibniz Institute, Berlin, and Charité Universitätsmedizin Berlin, Pediatric Pneumology, Immunology and Critical Care Medicine and SPZ (Center for Chronically Sick Children), Berlin, Germany The Joint European League Against Rheumatism and European Renal Association-European Dialysis and Transplant Association (EULAR/ERA-EDTA) recommendations define proteinuria as <0.5 g/24 h and normal or near normal (within 10% of normal eGFR if previously abnormal) eGFR as complete response (CR), and this definition has also been adopted by the SHARE Initiative (16, 27). This result may reflect the historically lower prevalence of these ethnicities in Germany and Austria and the lack of awareness of this risk factor. Er unterstützt als Förderverein die Arbeit der Kinderrheumatologie am Olgahospital Stuttgart und will durch Erfahrungsaustausch die Situation der kranken Kinder und Jugendlichen und ihren Eltern erleichtern. This site needs JavaScript to work properly. Results: Increased urinary protein excretion, either assessed by 24 h urine collection or spot urine (protein-creatinine ratio), and reduced estimated glomerular filtration rate were specified as important parameters for indication of kidney biopsy to diagnose proliferative LN and monitoring of therapy. Lupus. Table 6. Moroni G, Radice A, Giammarresi G, Quaglini S, Gallelli B, Leoni A, et al. It was shown that low-dose ASA may be beneficial in the primary prophylaxis of cardiovascular (CV) events in SLE patients (60, 61). Overall, there was a considerable heterogeneity regarding the suggested diagnostics and management of juvenile proliferative LN. The small number of cases distributed over several centers hampers a standardized procedure for this difficult-to-treat disease. ALOX5AP is an Indicator for High CD8 Lymphocyte Infiltration and "Hot" Tumor Microenvironment in Osteosarcoma: A Bioinformatic Study. PMC Suhlrie A, Hennies I, Gellermann J, Büscher A, Hoyer P, Waldegger S, et al. J Pediatr. Data collection was closed in January 2017. An official website of the United States government. Um negative Auswirkungen einer langfristigen stationären Behandlung auf die psychosoziale Entwicklung von Kindern und Jugendlichen mit Rheuma zu vermeiden, verfolgen wir am Olgahospital das Konzept einer primär ambulanten Betreuung. The Supplementary Material for this article can be found online at: https://www.frontiersin.org/articles/10.3389/fped.2022.851998/full#supplementary-material. Clin Exp Rheumatol. doi: 10.1177/0961203313483376, 48. Nach der Diagnose einer rheumatischen Erkrankung bei Kindern und Jugendlichen erfolgt die Behandlung durch das Rheumateam. Time acceptable to achieve remission was selected by participants. Twelve-month outcome in juvenile proliferative lupus nephritis: results of the German registry study. Arthritis Rheumatol. doi: 10.1055/a-1516-0459, 10. Epub 2014 Sep 18. Front. doi: 10.1002/art.34410, 7. In case of LN class III, 71% (n = 29) of participants considered an interval of 8–12 weeks until remission, following induction therapy, as acceptable. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). In the overall cohort, presence of PF correlated significantly with tumor site, histologic subtype, relative tumor size, and primary metastases, but not with body mass index or local surgical remission. This timeline may be rather optimistic, as recent registry data showed that 25 and 17% of German patients with juvenile LN class III/IV receiving induction treatment with corticosteroids in combination with either MMF of CP showed persistent proteinuria after 3 and 6 months, respectively (7). doi: 10.1371/journal.pone.0199003, 34. The latter was more frequently proposed by pediatric nephrologists and is in line with recent guidelines for treatment of proliferative LN in adults (21). (2013) 52:847–55. Systemic lupus erythematosus (SLE) in childhood: analysis of clinical and immunological findings in 34 patients and comparison with SLE characteristics in adults. Accessibility In summary, targets in the management of LN need to be defined and should be the subject of consensus findings. Smith EMD, Lythgoe H, Midgley A, Beresford MW, Hedrich CM. It was not a significant prognostic factor for EFS (P = .263; HR, 1.312). Groot N, de Graeff N, Avcin T, Bader-Meunier B, Brogan P, Dolezalova P, et al. Again, consensus on timelines for treatment targets need to be better defined. For SLE and specifically for LN, there is currently no T2T therapy protocol, while consensus treatment plans (CTPs) of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) and the Single Hub and Access point for pediatric Rheumatology in Europe (SHARE) initiative’s recommendations are available (16, 17). (2015) 30:1099–106. Ann Rheum Dis. (2017) 44:1032–8. doi: 10.5152/eurjrheum.2018.17096, 46. (2012) 70:199–207. This concomitant drug for SLE was not part of the selection, as it is not specifically for the treatment of LN. To date, the literature does not offer a uniform definition of complete remission in LN. official website and that any information you provide is encrypted Ravelli A, Consolaro A, Horneff G, Laxer RM, Lovell DJ, Wulffraat NM, et al. Feldman CH, Hiraki LT, Liu J, Fischer MA, Solomon DH, Alarcón GS, et al. Davies RJ, Sangle SR, Jordan NP, Aslam L, Lewis MJ, Wedgwood R, et al. As the answers on therapy schemes were a combination of Likert scale, multiple choice, and open comments, the different dosing regimens are displayed in the Supplementary Appendix. Katharinenhospital (KH)Krankenhaus Bad Cannstatt (kbc)Olgahospital (OH). Ann Rheum Dis. (2005) 9:79–84. 2. Weidenbusch M, Römmele C, Schröttle A, Anders HJ. Kanda H, Kubo K, Tateishi S, Sato K, Yonezumi A, Yamamoto K, et al. 50. In case of non-response to induction therapy, most respondents opt for a switch of medication beyond the increase of the corticosteroid dose, depending on previous therapy. Unable to load your collection due to an error, Unable to load your delegates due to an error. doi: 10.1093/rheumatology/kes348. Furthermore, normalization of serum complement C3 is also an important decision tool to define CR to the respondents, while it has been shown to have modest specificity for active LN (28). Ther apie in der Kinderrheumatologie (PRO-KIND): Enthesitis-assoziierte Arthritis Ergebnisse der Arbeitsgruppe Enthesitis-assoziierte Arthritis in der GKJR-Kommission . (2005) 146:648–53. Olgahospital Stuttgart Kriegsbergstr. Boneparth A, Radhakrishna SM, Greenbaum LA, Yen E, Okamura DM, Cooper JC, et al. Antiproteinuric effect of ARB in lupus nephritis patients with persistent proteinuria despite immunosuppressive therapy. Mok CC, To CH, Yu KL, Ho LY. According to the majority of the respondents (98%), the decision to perform a kidney biopsy is made on the basis of relevant pathological urine and kidney findings. Hydroxychloroquine was widely accepted as a concomitant measure followed by renin-angiotensin system inhibitors in cases of arterial hypertension and/or proteinuria. Bethesda, MD 20894, Web Policies For further immunosuppression in the context of maintenance therapy, most respondents (n = 24, 57%) suggested MMF (with a dosage of 1000–1200 mg/kg per day, max. Ann Rheum Dis. 2023 Mar 16;18(1):211. doi: 10.1186/s13018-023-03690-z. In our survey, respondents also rated the increase of eGFR to >90 ml/min/m2 as a very important target for remission, as well as protein excretion in 24 h urine collection < 200 mg/24 h, which is somewhat stricter than the above recommendations. This is important, as it will guide physicians toward switching to second-line treatments. Tumor size and prognosis in aggressively treated osteosarcoma. The limitations of our study are low participation/response rates (which is not unusual for an online survey distributed via mail) and which may be related to the treatment of LN being primarily in highly specialized centers. Arthritis Care Res. 8 respondents (20%) would prefer therapy with AZA, 3 (8%) with RTX (with no clear preference for the proposed dosing regimens), and 2 (5%) with CsA.
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