Immune-mediated thrombotic thrombocytopenic purpura in patients with and without systemic lupus erythematosus: a retrospective study
Abstract Background Thrombotic thrombocytopenic purpura (TTP) is associated with more deleterious outcomes in patients with systemic lupus erythematosus (SLE). However, ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) levels and ADAMTS13 inhibitor were not...
| Main Authors: | , , , , , , , , |
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| Format: | Article |
| Language: | English |
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BMC
2020-08-01
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| Series: | Orphanet Journal of Rare Diseases |
| Subjects: | |
| Online Access: | http://link.springer.com/article/10.1186/s13023-020-01510-9 |
| _version_ | 1818335148162154496 |
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| author | Cai Yue Jian Su Xiaohong Fan Li Song Wei Jiang Jinghua Xia Tao Shi Xuan Zhang Xuemei Li |
| author_facet | Cai Yue Jian Su Xiaohong Fan Li Song Wei Jiang Jinghua Xia Tao Shi Xuan Zhang Xuemei Li |
| author_sort | Cai Yue |
| collection | DOAJ |
| description | Abstract Background Thrombotic thrombocytopenic purpura (TTP) is associated with more deleterious outcomes in patients with systemic lupus erythematosus (SLE). However, ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) levels and ADAMTS13 inhibitor were not routinely assayed in most previous studies. The objective of this study is to compare the characteristics and outcomes of immune-mediated TTP (iTTP) in patients with and without SLE. Methods The medical data of 28 patients with iTTP from Peking Union Medical College Hospital were analysed. ADAMTS13 activity and ADAMTS13 inhibitor were measured in all patients. Results All 28 patients had ADAMTS13 inhibitor and severe ADAMTS13 deficiency. iTTP was considered SLE-related (SLE-TTP) in 10 patients and primary (primary iTTP) in 18 patients. Renal involvement on presentation was more severe in patients with primary iTTP as determined by higher serum creatinine (162.7 ± 110.6 vs 73.3 ± 13.4 μmol/L, p < 0.01) and more prevalent acute kidney injury (72.2% vs 10.0%, p < 0.01) than in patients with SLE-TTP. More patients with SLE-TTP were treated with steroid pulse therapy (90.0% vs 16.7%, p < 0.01) and intravenous immunoglobulin (IVIG) (50.0% vs 5.6%, p = 0.01) compared to patients with primary iTTP. After adjustments for age and treatment, including steroid pulse therapy and IVIG treatment, the likelihood of clinical remission of SLE-TTP was significantly increased compared to that of primary iTTP (HR 7.6 [1.2, 50.1], p = 0.03). Mortality was also lower among patients with SLE-TTP than among patients with primary iTTP (0 vs 38.9%, p = 0.03). Conclusions Renal involvement was less severe in patients with SLE-TTP than in patients with primary iTTP. The treatment responses and outcomes of SLE-TTP were no worse and perhaps even better than those of primary iTTP. When TTP is diagnosed in SLE patients, the ADAMTS13 level and ADAMTS13 inhibitor profile should be considered in addition to clinical features. |
| first_indexed | 2024-12-13T14:18:49Z |
| format | Article |
| id | doaj.art-2e03b14dbd794c739cd430fb64d86101 |
| institution | Directory Open Access Journal |
| issn | 1750-1172 |
| language | English |
| last_indexed | 2024-12-13T14:18:49Z |
| publishDate | 2020-08-01 |
| publisher | BMC |
| record_format | Article |
| series | Orphanet Journal of Rare Diseases |
| spelling | doaj.art-2e03b14dbd794c739cd430fb64d861012022-12-21T23:42:09ZengBMCOrphanet Journal of Rare Diseases1750-11722020-08-011511910.1186/s13023-020-01510-9Immune-mediated thrombotic thrombocytopenic purpura in patients with and without systemic lupus erythematosus: a retrospective studyCai Yue0Jian Su1Xiaohong Fan2Li Song3Wei Jiang4Jinghua Xia5Tao Shi6Xuan Zhang7Xuemei Li8Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical CollegeThrombosis and Haemostasis Research Unit, Jiangsu Institute of Haematology, First Affiliated Hospital of Soochow UniversityDepartment of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical CollegeDepartment of Emergency Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical CollegeDepartment of Medical Intensive Care Unit, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical CollegeDepartment of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical CollegeDepartment of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical CollegeDepartment of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical CollegeDepartment of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical CollegeAbstract Background Thrombotic thrombocytopenic purpura (TTP) is associated with more deleterious outcomes in patients with systemic lupus erythematosus (SLE). However, ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) levels and ADAMTS13 inhibitor were not routinely assayed in most previous studies. The objective of this study is to compare the characteristics and outcomes of immune-mediated TTP (iTTP) in patients with and without SLE. Methods The medical data of 28 patients with iTTP from Peking Union Medical College Hospital were analysed. ADAMTS13 activity and ADAMTS13 inhibitor were measured in all patients. Results All 28 patients had ADAMTS13 inhibitor and severe ADAMTS13 deficiency. iTTP was considered SLE-related (SLE-TTP) in 10 patients and primary (primary iTTP) in 18 patients. Renal involvement on presentation was more severe in patients with primary iTTP as determined by higher serum creatinine (162.7 ± 110.6 vs 73.3 ± 13.4 μmol/L, p < 0.01) and more prevalent acute kidney injury (72.2% vs 10.0%, p < 0.01) than in patients with SLE-TTP. More patients with SLE-TTP were treated with steroid pulse therapy (90.0% vs 16.7%, p < 0.01) and intravenous immunoglobulin (IVIG) (50.0% vs 5.6%, p = 0.01) compared to patients with primary iTTP. After adjustments for age and treatment, including steroid pulse therapy and IVIG treatment, the likelihood of clinical remission of SLE-TTP was significantly increased compared to that of primary iTTP (HR 7.6 [1.2, 50.1], p = 0.03). Mortality was also lower among patients with SLE-TTP than among patients with primary iTTP (0 vs 38.9%, p = 0.03). Conclusions Renal involvement was less severe in patients with SLE-TTP than in patients with primary iTTP. The treatment responses and outcomes of SLE-TTP were no worse and perhaps even better than those of primary iTTP. When TTP is diagnosed in SLE patients, the ADAMTS13 level and ADAMTS13 inhibitor profile should be considered in addition to clinical features.http://link.springer.com/article/10.1186/s13023-020-01510-9ADAMTS13 proteinAcquired thrombotic thrombocytopenic purpuraSystemic lupus erythematosusThrombotic microangiopathies |
| spellingShingle | Cai Yue Jian Su Xiaohong Fan Li Song Wei Jiang Jinghua Xia Tao Shi Xuan Zhang Xuemei Li Immune-mediated thrombotic thrombocytopenic purpura in patients with and without systemic lupus erythematosus: a retrospective study Orphanet Journal of Rare Diseases ADAMTS13 protein Acquired thrombotic thrombocytopenic purpura Systemic lupus erythematosus Thrombotic microangiopathies |
| title | Immune-mediated thrombotic thrombocytopenic purpura in patients with and without systemic lupus erythematosus: a retrospective study |
| title_full | Immune-mediated thrombotic thrombocytopenic purpura in patients with and without systemic lupus erythematosus: a retrospective study |
| title_fullStr | Immune-mediated thrombotic thrombocytopenic purpura in patients with and without systemic lupus erythematosus: a retrospective study |
| title_full_unstemmed | Immune-mediated thrombotic thrombocytopenic purpura in patients with and without systemic lupus erythematosus: a retrospective study |
| title_short | Immune-mediated thrombotic thrombocytopenic purpura in patients with and without systemic lupus erythematosus: a retrospective study |
| title_sort | immune mediated thrombotic thrombocytopenic purpura in patients with and without systemic lupus erythematosus a retrospective study |
| topic | ADAMTS13 protein Acquired thrombotic thrombocytopenic purpura Systemic lupus erythematosus Thrombotic microangiopathies |
| url | http://link.springer.com/article/10.1186/s13023-020-01510-9 |
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