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  • article 2 Citação(ões) na Scopus
    International cohort of 382 children with lupus nephritis - presentation, treatment and outcome at 24 months
    (2023) MUTIIS, Chiara De; WENDERFER, Scott E.; BASU, Biswanath; BAGGA, Arvind; ORJUELA, Alvaro; SAR, Tanmoy; AGGARWAL, Amita; JAIN, Avinash; YAP, Hui-Kim; TEO, Sharon; ITO, Shuichi; OHNISHI, Ai; IWATA, Naomi; KASAPCOPUR, Ozgur; YILDIZ, Mehmet; LAURENT, Audrey; MASTRANGELO, Antonio; OGURA, Masao; SHIMA, Yuko; RIANTHAVORN, Pornpimol; SILVA, Clovis A.; TRINDADE, Vitor; GIANVITI, Alessandra; AKINORI, Miyazono; HAMADA, Riku; FUJIMURA, Junya; MINAMIKAWA, Shogo; KAMIYOSHI, Naohiro; KAITO, Hiroshi; ISHIMORI, Shingo; IANNUZZELLA, Francesco; TULLUS, Kjell
    Background Children with lupus have a higher chance of nephritis and worse kidney outcome than adult patients. Methods We retrospectively analyzed clinical presentation, treatment and 24-month kidney outcome in a cohort of 382 patients (<= 18 years old) with lupus nephritis (LN) class >= III diagnosed and treated in the last 10 years in 23 international centers. Results The mean age at onset was 11 years 9 months and 72.8% were females. Fifty-seven percent and 34% achieved complete and partial remission at 24-month follow-up, respectively. Patients with LN class III achieved complete remission more often than those with classes IV or V (mixed and pure). Only 89 of 351 patients maintained stable complete kidney remission from the 6(th) to 24(th) months of follow-up. eGFR >= 90 ml/min/1.73 m(2) at diagnosis and biopsy class III were predictive of stable kidney remission. The youngest and the oldest age quartiles (2y-9y, 5m) (14y, 2m-18y,2m) showed lower rates of stable remission (17% and 20.7%, respectively) compared to the two other age groups (29.9% and 33.7%), while there was no difference in gender. No difference in achieving stable remission was found between children who received mycophenolate or cyclophosphamide as induction treatment. Conclusion Our data show that the rate of complete remission in patients with LN is still not high enough. Severe kidney involvement at diagnosis was the most important risk factor for not achieving stable remission while different induction treatments did not impact outcome. Randomized treatment trials involving children and adolescents with LN are needed to improve outcome for these children.
  • conferenceObject
    The Systemic Lupus Erythematosus International Collaborating Clinics (SLICC), American College of Rheumatology (ACR), and Lupus Foundation of America (LFA) Damage Index Revision - Item Generation Phase
    (2022) KUNDAKCI, Burak; BARBER, Megan R. W.; CLARKE, Ann E.; JOHNSON, Sindhu R.; BRUNNER, Hermine; CHO, Jiacai; COSTEDOAT-CHALUMEAU, Nathalie; GINZLER, Ellen M.; HANLY, John; HASAN, Abida; INANC, Murat; KABANI, Naureen; LEGGE, Alexandra; LIMA, Kaitlin; LINDOSO, Livia; MAK, Anselm; RAMSEY-GOLDMAN, Rosalind; RUIZ-IRASTORZA, Guillermo; SILVA, Clovis A.; TAMIROU, Farah; TRINDADE, Vitor C.; VINET, Evelyne; BRUCE, Ian N.
  • article 9 Citação(ões) na Scopus
    Defining renal remission in an international cohort of 248 children and adolescents with lupus nephritis
    (2022) MUTIIS, Chiara De; WENDERFER, Scott E.; ORJUELA, Alvaro; BAGGA, Arvind; BASU, Biswanath; SAR, Tanmoy; AGGARWAL, Amita; JAIN, Avinash; YAP, Hui-Kim; ITO, Shuichi; OHNISHI, Ai; IWATA, Naomi; KASAPCOPUR, Ozgur; LAURENT, Audrey; MASTRANGELO, Antonio; OGURA, Masao; SHIMA, Yuko; RIANTHAVORN, Pornpimol; SILVA, Clovis A.; TRINDADE, Vitor; DORMI, Ada; TULLUS, Kjell
    Objective We studied the rate of remission of LN in an international cohort of 248 children and adolescents with biopsy-proven LN. Five different definitions from scientific studies and the definitions recommended by the ACR and Kidney Disease: Improving Global Outcomes were used. Methods Anonymized clinical data in patients with biopsy-proven LN class >= III (International Society of Nephrology/Royal Pathology Society) diagnosed and treated in the last 10 years in 23 international centres from 10 countries were collected. We compared the rate of patients in complete and partial remission applying the different definitions. Results The mean age at diagnosis was 11 years and 4 months, and 177 were females. The number of patients in complete and partial remission varied a great deal between the different definitions. At 24 months, between 50% and 78.8% of the patients were in full remission as defined by the different criteria. The number of patients in partial remission was low, between 2.3% and 25%. No difference in achieved remission was found between boys and girls or between children and adolescents (P > 0.05). Patients with East Asian ethnicity reached remission more often than other ethnicities (P = 0.03-0.0008). Patients treated in high-income countries showed a higher percentage of complete remission at 12 and 24 months (P = 0.002-0.000001). Conclusion The rate of children and adolescents with LN achieving remission varied hugely with the definition used. Our results give important information for long-awaited treatment studies in children and young people.