Abstract
Background: Thrombotic microangiopathies (TMAs) are histological disorders characterized by non-immune microangiopathic hemolytic anemia and thrombocytopenia. They occur secondary to endothelial injury due to complement dysregulation, which leads organ ischemia. TMAs may be primary or secondary to another entity, including autoimmune disorders.
Purpose: To highlight the diagnosis of TMA secondary to Systemic Lupus Erythematosus (SLE) and Catastrophic Antiphospholipid Syndrome (CAPS), and to examine pathophysiological mechanisms and potential therapeutic strategies based on current evidence.
Case presentation: A 26-year-old female with multiorgan failure, bicytopenia, hypocomplementemia, and hemolytic anemia, in whom SLE and CAPS were diagnosed, with secondary TMA related to both entities. Renal biopsy revealed a full-house immunofluorescence pattern compatible with class IV lupus nephritis and thrombosis in glomerular arterioles and capillaries consistent with TMA changes. Timely histological diagnosis enabled the initiation of appropriate treatment, with a favorable clinical response.
References
ACHO. Consenso internacional de diagnóstico y manejo de microangiopatías trombóticas primarias [internet]. Ciudad de México: Grupo Distribuna; 2024. 94 p. https://acho.com.co/2024/05/22/consenso-internacional-de-mat-primarias-2024/
Thompson GL, Kavanagh D. Diagnosis and treatment of thrombotic microangiopathy. Int J Lab Hematol. 2022. https://doi.org/10.1111/ijlh.13954
Moake JL. Thrombotic Microangiopathies. N Engl J Med. 2002. https://doi.org/10.1056/NEJMra020528
Henao-Piedrahita N. Microangiopatías trombóticas primarias: una revisión narrativa. Rev Hematol Mex. 2021. https://doi.org/10.36384/01232576.432
Romero S, Sempere A, Gómez-Seguí I, Román E, Moret A, Jannone R, et al. Practice guidelines for the emergency treatment of thrombotic microangiopathy. Med Clin (Barc). 2018.
https://doi.org/10.1016/j.medcli.2018.01.013
Satyam A. Intertwined pathways of complement activation command the pathogenesis of lupus nephritis. Transl Res. 2022. https://doi.org/10.1016/j.trsl.2022.03.005
Román E., Mendizábal S, Jarque I, De la Rubia J, Sempere A, Morales E, et al. Microangiopatía trombótica secundaria y eculizumab: una opción terapéutica razonable. Nefrologia. 2017. https://doi.org/10.1016/j.nefro.2017.01.006
Yang F., Tian J, Peng L, Zhang Li, Liu J, Tian X, et al. Thrombocytopenia Is an Independent Risk Factor for the Prognosis of Thrombotic Microangiopathy in Chinese Patients with Systemic Lupus Erythematosus. Front Med (Lausanne). 2021. https://doi.org/10.3389/fmed.2021.772607
Cervera R., Rodríguez-Pintó I, Espinosa G. The diagnosis and clinical management of the catastrophic antiphospholipid syndrome: A comprehensive review. J Autoimmun. 2018. https://doi.org/10.1016/j.jaut.2018.05.007
Tektonidou MG, Sotsiou F, Moutsopoulos HM. Antiphospholipid syndrome (APS) nephropathy in catastrophic, primary, and systemic lupus erythematosus-related APS. J Rheumatol. 2008. https://pubmed.ncbi.nlm.nih.gov/18688914/
Kotzen ES, Roy S, Jain K. Antiphospholipid Syndrome Nephropathy and Other Thrombotic Microangiopathies Among Patients with Systemic Lupus Erythematosus. Adv Chronic Kidney Dis. 2019.
https://doi.org/10.1053/j.ackd.2019.08.012
Daugas E, Nochy D, Huong DLT, Duhaut P, Beaufils H, Caudwell V, et al. Antiphospholipid syndrome nephropathy in systemic lupus erythematosus. J Am Soc Nephrol. 2002; 13(1), 42-52. https://doi.org/10.1681/ASN.V13142
Nochy D, Daugas E, Droz D, Beaufils H, Grünfeld JP, Piette JC, et al. The intrarenal vascular lesions associated with primary antiphospholipid síndrome [internet]. J Am Soc Nephrol. 1999 [citado 30 de mayo de 2025]; 10(3), 507-518. https://doi.org/10.1681/ASN.V103507
Tektonidou MG, Sotsiou F, Nakopoulou L, Vlachoyiannopoulos PG, Moutsopoulos HM. Antiphospholipid syndrome nephropathy in patients with systemic lupus erythematosus and antiphospholipid antibodies: Prevalence, clinical associations, and long?term outcome. Arthritis Rheum. 2004; 50(8), 2569-79. https://doi.org/10.1002/art.20433
Rodriguez-Pintó I, Espinosa G, Cervera R. Catastrophic antiphospholipid syndrome: Lessons from the "CAPS Registry". Med Clin (Barc). 2024. https://doi.org/10.1016/j.medcli.2024.02.011
Li A, Khalighi PR, Wu Q, Garcia DA. External validation of the Plasmic Score: A clinical prediction tool for thrombotic thrombocytopenic purpura diagnosis and treatment. J Thromb Haemost. 2018; 16(1), 164-169. https://doi.org/10.1111/jth.13882
Kronbichler A, Frank R, Kirschfink M, Szilágyi Á, Csuka D, Prohászka Z, et al. Efficacy of eculizumab in a patient with immunoadsorption-dependent catastrophic antiphospholipid syndrome: A case report. Medicine (Baltimore). 2014; 93(26), e143. https://doi.org/10.1097/MD.0000000000000143
Strakhan M, Hurtado-Sbordoni M, Galeas N, Bakirhan K, Alexis K, Elrafei T. 36-year-old female with catastrophic antiphospholipid syndrome treated with eculizumab: A case report and review of literature. Case Rep Hematol. 2014. https://doi.org/10.1155/2014/704371
Geethakumari PR, Mille P, Gulati R, Nagalla S. Complement inhibition with eculizumab for thrombotic microangiopathy rescues a living-donor kidney transplant in a patient with antiphospholipid antibody syndrome. Transfus Apher Sci. 2017; 56(3), 400-403. https://doi.org/10.1016/j.transci.2017.02.007
Khormi AAM, Ba Gunaid M, Fayyad M, Mohrag M, AlAseeri AA. Catastrophic antiphospholipid syndrome in a lupus patient with severe recurrent life-threatening clinical manifestations. J Med Cases. 2024; 15(10), 278-282. https://doi.org/10.14740/jmc4255
Pengo V., Denas G, Zoppellaro G, Padayattil Jose S, Hoxha A, Ruffatti A., et al. Rivaroxaban vs warfarin in high-risk patients with antiphospholipid syndrome. Blood. 2018; 132(13), 1365-71. https://doi.org/10.1182/blood-2018-04-848333

This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

