Prevalence and outcomes of a pilot definition of severe refractory systemic lupus erythematosus: observations from a multinational Asia-Pacific cohort
Abstract Background Emerging therapies have the potential to be used in patients with severe refractory systemic lupus erythematosus (srSLE), but no agreed definition of srSLE exists. We evaluated a pilot srSLE definition to assess whether a set of disease activity and treatment thresholds could ide...
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2025-07-01
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| Online Access: | https://doi.org/10.1186/s13075-025-03622-8 |
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| author | Rangi Kandane-Rathnayake Worawit Louthrenoo CS Lau Laniyati Hamijoyo Jiacai Cho Aisha Lateef Shue Fen Luo Yeong-Jian Wu Sandra Navarra Leonid Zamora Zhanguo Li Yi-Hsing Chen Shereen Oon Madelynn Chan Sargunan Sockalingam Yanjie Hao Zhuoli Zhang Sang-Cheol Bae Jun Kikuchi Tsutomu Takeuchi Yasuhiro Katsumata BMDB Basnayake Fiona Goldblatt Sean O’Neill Kristine Pek Ling Ng Nicola Tugnet Mark Sapsford Yih Jia Poh Cherica Tee Michael Tee Naoaki Ohkubo Adrienne O’Donnell Lefeber Tamas Shisha Yoshiya Tanaka Vera Golder Mandana Nikpour Alberta Hoi Peter Gergely Eric Morand |
| author_facet | Rangi Kandane-Rathnayake Worawit Louthrenoo CS Lau Laniyati Hamijoyo Jiacai Cho Aisha Lateef Shue Fen Luo Yeong-Jian Wu Sandra Navarra Leonid Zamora Zhanguo Li Yi-Hsing Chen Shereen Oon Madelynn Chan Sargunan Sockalingam Yanjie Hao Zhuoli Zhang Sang-Cheol Bae Jun Kikuchi Tsutomu Takeuchi Yasuhiro Katsumata BMDB Basnayake Fiona Goldblatt Sean O’Neill Kristine Pek Ling Ng Nicola Tugnet Mark Sapsford Yih Jia Poh Cherica Tee Michael Tee Naoaki Ohkubo Adrienne O’Donnell Lefeber Tamas Shisha Yoshiya Tanaka Vera Golder Mandana Nikpour Alberta Hoi Peter Gergely Eric Morand |
| author_sort | Rangi Kandane-Rathnayake |
| collection | DOAJ |
| description | Abstract Background Emerging therapies have the potential to be used in patients with severe refractory systemic lupus erythematosus (srSLE), but no agreed definition of srSLE exists. We evaluated a pilot srSLE definition to assess whether a set of disease activity and treatment thresholds could identify patients with poor outcomes. Methods Data from a 13-country longitudinal SLE cohort, collected prospectively between 2013 and 2020 were analysed. srSLE was defined if a patient was in high disease activity (SLEDAI-2K ≥ 10) despite combination use of at least glucocorticoids (GC) and immunosuppressants (IS) at both the index and preceding visit. Synchronised to the index srSLE visit, we assessed disease activity, medication use and treat-to-target (T2T) endpoint attainment over 12 months (m). Results Of 3,744 patients studied, 578 (14%) had srSLE, in 1,810 visits. The median [IQR] SLEDAI-2K at the srSLE index visit was 12 [10, 14], which decreased to 6 [4, 10] at 6m and 12m. Most patients remained on combination anti-malarial, GC, and IS at all follow-up time points. The median [IQR] GC dose at the index visit was 10 [5, 20] mg/day; this reduced to 8 mg [5.0, 12.9] at 6m and 5 mg [5.0, 10.0] at 12m. Less than a quarter of patients attained LLDAS and only 1% attained GC-free remission over 12 months. Conclusions A draft definition of srSLE was clearly associated with poor outcomes. Work to evaluate multiple thresholds with which to define srSLE, and their outcomes, is warranted. |
| format | Article |
| id | doaj-art-0dcffcfe289f4f0ab7d60040a12e856e |
| institution | Kabale University |
| issn | 1478-6362 |
| language | English |
| publishDate | 2025-07-01 |
| publisher | BMC |
| record_format | Article |
| series | Arthritis Research & Therapy |
| spelling | doaj-art-0dcffcfe289f4f0ab7d60040a12e856e2025-08-20T03:43:01ZengBMCArthritis Research & Therapy1478-63622025-07-012711810.1186/s13075-025-03622-8Prevalence and outcomes of a pilot definition of severe refractory systemic lupus erythematosus: observations from a multinational Asia-Pacific cohortRangi Kandane-Rathnayake0Worawit Louthrenoo1CS Lau2Laniyati Hamijoyo3Jiacai Cho4Aisha Lateef5Shue Fen Luo6Yeong-Jian Wu7Sandra Navarra8Leonid Zamora9Zhanguo Li10Yi-Hsing Chen11Shereen Oon12Madelynn Chan13Sargunan Sockalingam14Yanjie Hao15Zhuoli Zhang16Sang-Cheol Bae17Jun Kikuchi18Tsutomu Takeuchi19Yasuhiro Katsumata20BMDB Basnayake21Fiona Goldblatt22Sean O’Neill23Kristine Pek Ling Ng24Nicola Tugnet25Mark Sapsford26Yih Jia Poh27Cherica Tee28Michael Tee29Naoaki Ohkubo30Adrienne O’Donnell Lefeber31Tamas Shisha32Yoshiya Tanaka33Vera Golder34Mandana Nikpour35Alberta Hoi36Peter Gergely37Eric Morand38Centre for Inflammatory Diseases, School of Clinical Sciences, Monash UniversityDepartment of Internal Medicine, Faculty of Medicine, Chiang Mai UniversityDepartment of Medicine, Queen Mary Hospital, The University of Hong KongDepartment of Internal Medicine, Faculty of Medicine, Padjadjaran UniversityRheumatology Division, Department of Medicine, National University HospitalDepartment of Rheumatology, Division of Medicine, Woodlands HealthDepartment of Rheumatology, Allergy and Immunology, Chang Gung Memorial HospitalDepartment of Rheumatology, Allergy and Immunology, Chang Gung Memorial HospitalSection of Rheumatology, University of Santo Tomas HospitalSection of Rheumatology, University of Santo Tomas HospitalDepartment of Rheumatology and Immunology, People’s Hospital, Peking University Health Science CenterDivision of Allergy, Immunology and Rheumatology, Taichung Veterans General HospitalDepartment of Rheumatology, St Vincent’s Hospital MelbourneDepartment of Rheumatology, Allergy & Immunology, Tan Tock Seng HospitalDepartment of Medicine, Universiti Malaya Medical CentreDepartment of Rheumatology, St Vincent’s Hospital MelbourneRheumatology and Immunology department, Peking University First HospitalHanyang University Hospital for Rheumatic Diseases, Hanyang University Institute for Rheumatology Research and Hanyang Institute of Bioscience and BiotechnologyDepartment of Internal Medicine, Keio UniversityDepartment of Internal Medicine, Keio UniversityDepartment of Internal Medicine, Tokyo Women’s Medical UniversityDepartment of Nephrology, Teaching Hospital KandyDepartment of Rheumatology, Flinders Medical CentreRheumatology Department, Liverpool HospitalDepartment of Rheumatology, North Shore Hospital, Health New Zealand WaitemataDepartment of Rheumatology, Greenlane Clinical Centre, Health New Zealand Auckland, Te Whatu OraDepartment of Rheumatology, Middlemore Hospital, Health New Zealand Counties Manukau, Te Whatu OraDepartment of Rheumatology and Immunology, Singapore General HospitalCollege of Medicine, University of the PhilippinesCollege of Medicine, University of the PhilippinesThe First Department of Internal Medicine, University of Occupational and Environmental HealthNovartis PharmaNovartis PharmaThe First Department of Internal Medicine, University of Occupational and Environmental HealthCentre for Inflammatory Diseases, School of Clinical Sciences, Monash UniversityDepartment of Rheumatology, St Vincent’s Hospital MelbourneCentre for Inflammatory Diseases, School of Clinical Sciences, Monash UniversityNovartis PharmaCentre for Inflammatory Diseases, School of Clinical Sciences, Monash UniversityAbstract Background Emerging therapies have the potential to be used in patients with severe refractory systemic lupus erythematosus (srSLE), but no agreed definition of srSLE exists. We evaluated a pilot srSLE definition to assess whether a set of disease activity and treatment thresholds could identify patients with poor outcomes. Methods Data from a 13-country longitudinal SLE cohort, collected prospectively between 2013 and 2020 were analysed. srSLE was defined if a patient was in high disease activity (SLEDAI-2K ≥ 10) despite combination use of at least glucocorticoids (GC) and immunosuppressants (IS) at both the index and preceding visit. Synchronised to the index srSLE visit, we assessed disease activity, medication use and treat-to-target (T2T) endpoint attainment over 12 months (m). Results Of 3,744 patients studied, 578 (14%) had srSLE, in 1,810 visits. The median [IQR] SLEDAI-2K at the srSLE index visit was 12 [10, 14], which decreased to 6 [4, 10] at 6m and 12m. Most patients remained on combination anti-malarial, GC, and IS at all follow-up time points. The median [IQR] GC dose at the index visit was 10 [5, 20] mg/day; this reduced to 8 mg [5.0, 12.9] at 6m and 5 mg [5.0, 10.0] at 12m. Less than a quarter of patients attained LLDAS and only 1% attained GC-free remission over 12 months. Conclusions A draft definition of srSLE was clearly associated with poor outcomes. Work to evaluate multiple thresholds with which to define srSLE, and their outcomes, is warranted.https://doi.org/10.1186/s13075-025-03622-8Systemic lupus erythematosus (SLE)Severe refractory diseasePoor outcomes |
| spellingShingle | Rangi Kandane-Rathnayake Worawit Louthrenoo CS Lau Laniyati Hamijoyo Jiacai Cho Aisha Lateef Shue Fen Luo Yeong-Jian Wu Sandra Navarra Leonid Zamora Zhanguo Li Yi-Hsing Chen Shereen Oon Madelynn Chan Sargunan Sockalingam Yanjie Hao Zhuoli Zhang Sang-Cheol Bae Jun Kikuchi Tsutomu Takeuchi Yasuhiro Katsumata BMDB Basnayake Fiona Goldblatt Sean O’Neill Kristine Pek Ling Ng Nicola Tugnet Mark Sapsford Yih Jia Poh Cherica Tee Michael Tee Naoaki Ohkubo Adrienne O’Donnell Lefeber Tamas Shisha Yoshiya Tanaka Vera Golder Mandana Nikpour Alberta Hoi Peter Gergely Eric Morand Prevalence and outcomes of a pilot definition of severe refractory systemic lupus erythematosus: observations from a multinational Asia-Pacific cohort Arthritis Research & Therapy Systemic lupus erythematosus (SLE) Severe refractory disease Poor outcomes |
| title | Prevalence and outcomes of a pilot definition of severe refractory systemic lupus erythematosus: observations from a multinational Asia-Pacific cohort |
| title_full | Prevalence and outcomes of a pilot definition of severe refractory systemic lupus erythematosus: observations from a multinational Asia-Pacific cohort |
| title_fullStr | Prevalence and outcomes of a pilot definition of severe refractory systemic lupus erythematosus: observations from a multinational Asia-Pacific cohort |
| title_full_unstemmed | Prevalence and outcomes of a pilot definition of severe refractory systemic lupus erythematosus: observations from a multinational Asia-Pacific cohort |
| title_short | Prevalence and outcomes of a pilot definition of severe refractory systemic lupus erythematosus: observations from a multinational Asia-Pacific cohort |
| title_sort | prevalence and outcomes of a pilot definition of severe refractory systemic lupus erythematosus observations from a multinational asia pacific cohort |
| topic | Systemic lupus erythematosus (SLE) Severe refractory disease Poor outcomes |
| url | https://doi.org/10.1186/s13075-025-03622-8 |
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