Thrombotic thrombocytopenic purpura developed during the conservative treatment of anti-phospholipase A2 receptor antibody-positive idiopathic membranous nephropathy: a case report
| Autor: | Hiroaki Fushimi, Rei Iio, Terumasa Hayashi, Masanori Matsumoto, Hiroki Okushima, Yukimasa Iwata, Tatsuya Shoji, Shinichi Akiyama, Naomi Ota, Karin Shimada, Kensuke Mitsumoto, Kodo Tomida |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2020 |
| Předmět: |
Hemolytic anemia
medicine.medical_specialty Thrombotic microangiopathy 030232 urology & nephrology Thrombotic thrombocytopenic purpura Anti-PLA2R antibody Acquired thrombotic thrombocytopenic purpura 030204 cardiovascular system & hematology lcsh:RC870-923 Gastroenterology 03 medical and health sciences 0302 clinical medicine Internal medicine hemic and lymphatic diseases Medicine Idiopathic membranous nephropathy Acquired Thrombotic Thrombocytopenic Purpura business.industry Autoantibody Glomerulonephritis ADAMTS13 inhibitor medicine.disease lcsh:Diseases of the genitourinary system. Urology ADAMTS13 Schistocyte Nephrology business |
| Zdroj: | BMC Nephrology, Vol 21, Iss 1, Pp 1-6 (2020) |
| ISSN: | 1471-2369 |
| DOI: | 10.1186/s12882-020-02086-z |
| Popis: | Background Idiopathic membranous nephropathy (MN) is one of the major glomerulonephritis that cause nephrotic syndrome. The phospholipase A2 receptor (PLA2R) has recently been identified as an endogenous antigen of idiopathic MN. Thrombotic thrombocytopenic purpura (TTP) is a disorder characterized by schistocytes, hemolytic anemia, thrombocytopenia, and organ dysfunction which occurs as a result of thrombi. Patients with acquired TTP have autoantibodies against a disintegrin and metalloprotease with thrombospondin type 1 motif 13 (ADAMTS13). These autoantibodies act as an inhibitor and cause ADAMTS13 deficiency. Idiopathic MN and acquired TTP are usually considered as independent autoimmune diseases. We experienced a patient who developed TTP during the conservative treatment of idiopathic MN, with the coexistence of ADAMTS13 inhibitor and anti-PLA2R antibody. Case presentation A 73-year-old man presented with thrombocytopenia, hemolytic anemia, disturbance of consciousness, and acute kidney injury after 4-year course of biopsy-proven idiopathic MN. ADAMTS13 activity was undetectable and the ADAMTS13 inhibitor was identified. Additionally, he was positive for anti-PLA2R antibody. The patient did not have any diseases that could cause secondary thrombotic microangiopathy, and he was diagnosed with acquired TTP. Steroid therapy and plasma exchange were initiated and the acquired TTP resolved. MN achieved remission 3 months after the anti-PLA2R antibody disappeared. Conclusions This is the first reported case of acquired TTP developed during conservative treatment of idiopathic MN, with both ADAMTS13 inhibitor and anti-PLA2R antibody positive at the onset of the TTP. The present case suggests that idiopathic MN might be associated with the development of some cases of acquired TTP. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|
Full text from SpringerLink