Concurrent Presentation of Thrombotic Thrombocytopenic Purpura and Membranous Nephropathy

Al‐Rabadi, Laith; Quillen, Karen; Shashar, Moshe; Marji, Catreena Al; Jaberi, Aala; Chitalia, Vipul C.; Henderson, Joel M.; Salant, David J.; Beck, Laurence H.

doi:10.1016/j.ekir.2017.08.005

Cited by 3 publications

(3 citation statements)

References 29 publications

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“…In this case, it is possible that these 2 diseases merged by chance. However, as far as our literature review, 3 cases of idiopathic MN and acquired TTP co-existing have been reported [ 5 – 7 ], which suggested to us a possible association between these 2 diseases. Several mechanisms which could explain this association might be proposed.…”

Section: Discussionmentioning

confidence: 88%

“…However, the anti-PLA 2 R antibody and ADAMTS13 inhibitor were not measured. In the second case, a renal biopsy also showed characteristic findings of MN and TMA, although the case had a history of hepatitis C, with no history of proteinuria [ 7 ]. Anti-PLA 2 R antibody and ADAMTS13 inhibitor were detected in the serum, and immunofluorescence staining for renal tissue showed positive for the PLA 2 R and IgG1 subclass, but not for IgG4, whereas in idiopathic MN, IgG4 is usually predominant with immunofluorescence detection of the IgG subclass [ 21 ].…”

Section: Discussionmentioning

confidence: 99%

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Thrombotic thrombocytopenic purpura developed during the conservative treatment of anti-phospholipase A2 receptor antibody-positive idiopathic membranous nephropathy: a case report

et al. 2020

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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.

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Section: Discussionmentioning

confidence: 88%

Section: Discussionmentioning

confidence: 99%

Thrombotic thrombocytopenic purpura developed during the conservative treatment of anti-phospholipase A2 receptor antibody-positive idiopathic membranous nephropathy: a case report

et al. 2020

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“…As per an analysis of 14,400 cases of TTP approximately 9% had one or more underlying connective tissue disease [ 8 ]. TTP has been reported with systemic lupus erythematosus (SLE), Graves' disease, adult onset still's disease, membranous nephropathy, Good pasture disease, Legionella pneumonia, antiphospholipid antibody syndrome (APLA), multiple endocrine neoplasia 1 (MEN I) [ [9] , [10] , [11] , [12] , [13] , [14] , [15] , [16] ].…”

Section: Clinical Discussionmentioning

confidence: 99%

Concurrent thrombotic thrombocytopenic purpura and Guillian Barre Syndrome post infectious diarrhea

Hussain

Kumar

Singh³

et al. 2022

Annals of Medicine &Amp; Surgery

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Update on IgG4-mediated autoimmune diseases: New insights and new family members

Koneczny

2020

Autoimmunity Reviews

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Concurrent Presentation of Thrombotic Thrombocytopenic Purpura and Membranous Nephropathy

Cited by 3 publications

References 29 publications

Thrombotic thrombocytopenic purpura developed during the conservative treatment of anti-phospholipase A2 receptor antibody-positive idiopathic membranous nephropathy: a case report

Thrombotic thrombocytopenic purpura developed during the conservative treatment of anti-phospholipase A2 receptor antibody-positive idiopathic membranous nephropathy: a case report

Concurrent thrombotic thrombocytopenic purpura and Guillian Barre Syndrome post infectious diarrhea

Update on IgG4-mediated autoimmune diseases: New insights and new family members

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