Systemic Lupus Erythematosus Presenting With Thrombotic Thrombocytopenic Purpura at Onset: A Case Report
BackgroundThrombotic microangiopathy (TMA) is a syndrome associated with hemolytic anemia, thrombocytopenia, and various organ disorders. Thrombotic thrombocytopenic purpura (TTP) is a disease that develops when a disintegrin-like and metalloproteinase with thrombospondin type l motif 13 (ADAMTS13)...
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Frontiers Media S.A.
2022-08-01
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| Series: | Frontiers in Pediatrics |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fped.2022.931669/full |
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| author | Yoko Takagi Yasuko Kobayashi Ayako Hirakata Mariko Takei Satoshi Ogasawara Chikage Yajima Yuka Ikeuchi Akira Matsumoto Yoshiyuki Ogawa Hiroshi Handa Masanori Matsumoto Hirokazu Arakawa Takumi Takizawa |
| author_facet | Yoko Takagi Yasuko Kobayashi Ayako Hirakata Mariko Takei Satoshi Ogasawara Chikage Yajima Yuka Ikeuchi Akira Matsumoto Yoshiyuki Ogawa Hiroshi Handa Masanori Matsumoto Hirokazu Arakawa Takumi Takizawa |
| author_sort | Yoko Takagi |
| collection | DOAJ |
| description | BackgroundThrombotic microangiopathy (TMA) is a syndrome associated with hemolytic anemia, thrombocytopenia, and various organ disorders. Thrombotic thrombocytopenic purpura (TTP) is a disease that develops when a disintegrin-like and metalloproteinase with thrombospondin type l motif 13 (ADAMTS13) activity decreases to < 10% of that in normal plasma, causing platelet thrombosis in microvessels throughout the body. Currently, ADAMTS13-deficient TMA is diagnosed as TTP. Systemic lupus erythematosus (SLE)-related TMA includes both acquired TTP, in which ADAMTS13 activity is significantly reduced, and secondary TMA, in which ADAMTS13 activity is not reduced. Both diseases have different prognoses.Case PresentationAn 11-year-old girl was admitted to our hospital on suspicion of TMA with thrombocytopenia and hemolytic anemia. Because the patient had hypocomplementemia, SLE-related TMA or complement-related TMA was considered. Therefore, we initiated plasma exchange (PE) for the patient. Subsequently, she fulfilled the pediatric SLE diagnostic criteria, and ADAMTS13 activity was shown to be decreased and the anti-ADAMTS13 antibody titer increased. She was thus diagnosed with acquired TTP caused by SLE. Treatment response was good as a platelet count and ADAMTS13 activity improved with three times of PE, followed by methylprednisolone pulse therapy and administration of mycophenolate mofetil. Renal pathology showed thrombus formation in glomerular arterioles and lupus nephritis categorized as Class III (A) of the International Society of Nephrology and the Renal Pathology Society classification. Because the patient was thought to be in the high-risk group of SLE, three courses of intravenous cyclophosphamide pulse therapy were administered as an additional induction therapy. No recurrence of TTP was observed.ConclusionIn SLE-related TMA, measurement of ADAMTS13 activity and the anti-ADAMTS13 antibody titer are necessary for diagnosis, and for predicting prognosis and recurrence of the disease; however, in the acute phase of immune-mediated TMA, it is important to initiate proper treatments even before knowing the results to improve prognosis. |
| first_indexed | 2024-04-12T08:07:40Z |
| format | Article |
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| language | English |
| last_indexed | 2024-04-12T08:07:40Z |
| publishDate | 2022-08-01 |
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| series | Frontiers in Pediatrics |
| spelling | doaj.art-442560d8e3e3422c9550853cfcf08b602022-12-22T03:41:04ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602022-08-011010.3389/fped.2022.931669931669Systemic Lupus Erythematosus Presenting With Thrombotic Thrombocytopenic Purpura at Onset: A Case ReportYoko Takagi0Yasuko Kobayashi1Ayako Hirakata2Mariko Takei3Satoshi Ogasawara4Chikage Yajima5Yuka Ikeuchi6Akira Matsumoto7Yoshiyuki Ogawa8Hiroshi Handa9Masanori Matsumoto10Hirokazu Arakawa11Takumi Takizawa12Department of Pediatrics, Gunma University Graduate School of Medicine, Maebashi, JapanDepartment of Pediatrics, Gunma University Graduate School of Medicine, Maebashi, JapanDepartment of Pediatrics, Gunma University Graduate School of Medicine, Maebashi, JapanDepartment of Pediatrics, Gunma University Graduate School of Medicine, Maebashi, JapanDepartment of Pediatrics, Gunma University Graduate School of Medicine, Maebashi, JapanDepartment of Pediatrics, Gunma University Graduate School of Medicine, Maebashi, JapanDepartment of Pediatrics, Gunma University Graduate School of Medicine, Maebashi, JapanDepartment of Hematology, Gunma University Graduate School of Medicine, Maebashi, JapanDepartment of Hematology, Gunma University Graduate School of Medicine, Maebashi, JapanDepartment of Hematology, Gunma University Graduate School of Medicine, Maebashi, JapanDepartment of Blood Transfusion Medicine, Nara Medical University, Kashihara, JapanDepartment of Pediatrics, Gunma University Graduate School of Medicine, Maebashi, JapanDepartment of Pediatrics, Gunma University Graduate School of Medicine, Maebashi, JapanBackgroundThrombotic microangiopathy (TMA) is a syndrome associated with hemolytic anemia, thrombocytopenia, and various organ disorders. Thrombotic thrombocytopenic purpura (TTP) is a disease that develops when a disintegrin-like and metalloproteinase with thrombospondin type l motif 13 (ADAMTS13) activity decreases to < 10% of that in normal plasma, causing platelet thrombosis in microvessels throughout the body. Currently, ADAMTS13-deficient TMA is diagnosed as TTP. Systemic lupus erythematosus (SLE)-related TMA includes both acquired TTP, in which ADAMTS13 activity is significantly reduced, and secondary TMA, in which ADAMTS13 activity is not reduced. Both diseases have different prognoses.Case PresentationAn 11-year-old girl was admitted to our hospital on suspicion of TMA with thrombocytopenia and hemolytic anemia. Because the patient had hypocomplementemia, SLE-related TMA or complement-related TMA was considered. Therefore, we initiated plasma exchange (PE) for the patient. Subsequently, she fulfilled the pediatric SLE diagnostic criteria, and ADAMTS13 activity was shown to be decreased and the anti-ADAMTS13 antibody titer increased. She was thus diagnosed with acquired TTP caused by SLE. Treatment response was good as a platelet count and ADAMTS13 activity improved with three times of PE, followed by methylprednisolone pulse therapy and administration of mycophenolate mofetil. Renal pathology showed thrombus formation in glomerular arterioles and lupus nephritis categorized as Class III (A) of the International Society of Nephrology and the Renal Pathology Society classification. Because the patient was thought to be in the high-risk group of SLE, three courses of intravenous cyclophosphamide pulse therapy were administered as an additional induction therapy. No recurrence of TTP was observed.ConclusionIn SLE-related TMA, measurement of ADAMTS13 activity and the anti-ADAMTS13 antibody titer are necessary for diagnosis, and for predicting prognosis and recurrence of the disease; however, in the acute phase of immune-mediated TMA, it is important to initiate proper treatments even before knowing the results to improve prognosis.https://www.frontiersin.org/articles/10.3389/fped.2022.931669/fullsystemic lupus erythematosusthrombotic microangiopathythrombotic thrombocytopenic purpuraADAMTS13 activityanti-ADAMTS13 antibodyplatelet thrombosis |
| spellingShingle | Yoko Takagi Yasuko Kobayashi Ayako Hirakata Mariko Takei Satoshi Ogasawara Chikage Yajima Yuka Ikeuchi Akira Matsumoto Yoshiyuki Ogawa Hiroshi Handa Masanori Matsumoto Hirokazu Arakawa Takumi Takizawa Systemic Lupus Erythematosus Presenting With Thrombotic Thrombocytopenic Purpura at Onset: A Case Report Frontiers in Pediatrics systemic lupus erythematosus thrombotic microangiopathy thrombotic thrombocytopenic purpura ADAMTS13 activity anti-ADAMTS13 antibody platelet thrombosis |
| title | Systemic Lupus Erythematosus Presenting With Thrombotic Thrombocytopenic Purpura at Onset: A Case Report |
| title_full | Systemic Lupus Erythematosus Presenting With Thrombotic Thrombocytopenic Purpura at Onset: A Case Report |
| title_fullStr | Systemic Lupus Erythematosus Presenting With Thrombotic Thrombocytopenic Purpura at Onset: A Case Report |
| title_full_unstemmed | Systemic Lupus Erythematosus Presenting With Thrombotic Thrombocytopenic Purpura at Onset: A Case Report |
| title_short | Systemic Lupus Erythematosus Presenting With Thrombotic Thrombocytopenic Purpura at Onset: A Case Report |
| title_sort | systemic lupus erythematosus presenting with thrombotic thrombocytopenic purpura at onset a case report |
| topic | systemic lupus erythematosus thrombotic microangiopathy thrombotic thrombocytopenic purpura ADAMTS13 activity anti-ADAMTS13 antibody platelet thrombosis |
| url | https://www.frontiersin.org/articles/10.3389/fped.2022.931669/full |
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