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The endothelial damage that leads to this histological lesion can be triggered by several conditions or diseases, hindering an early diagnosis and aetiological treatment. However, due to systemic involvement in TMA and its low incidence, an accurate early diagnosis is often troublesome. In the last few decades, major improvements have been made in the pathophysiological knowledge of TMAs such as thrombotic thrombocytopenic purpura [TTP, caused by ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin Type 1 motif, member 13) deficiency] and atypical haemolytic uraemic syndrome (aHUS, associated with dysregulation of the alternative complement pathway), together with enhancements in patient management due to new diagnostic tools and treatments. However, diagnosis of aHUS requires the exclusion of all the other entities that can cause TMA, delaying the introduction of terminal complement blockers, which have shown high efficacy in haemolysis control and especially in avoiding organ damage if used early. Importantly, there is increasing evidence that other forms of TMA could present overactivation of the complement system, worsening their clinical progression. This review addresses the diagnostic and therapeutic approach when there is clinical suspicion of TMA, emphasizing complement evaluation as a potential tool for the inclusive diagnosis of aHUS, as well as for the improvement of current knowledge of its pathophysiological involvement in other TMAs. The development of both new complement activation biomarkers and inhibitory treatments will probably improve the management of TMA patients in the near future, reducing response times and improving patient outcomes.<\/jats:p>","DOI":"10.1093\/ckj\/sfaa195","type":"journal-article","created":{"date-parts":[[2020,9,2]],"date-time":"2020-09-02T19:15:41Z","timestamp":1599074141000},"page":"1055-1066","source":"Crossref","is-referenced-by-count":34,"title":["Thrombotic microangiopathies assessment: mind the complement"],"prefix":"10.1093","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0789-7992","authenticated-orcid":false,"given":"Miquel","family":"Blasco","sequence":"first","affiliation":[{"name":"Department of Nephrology and Kidney Transplantation, Hospital Cl\u00ednic, Centro de Referencia en Enfermedad Glomerular Compleja del Sistema Nacional de Salud (CSUR), University of Barcelona, Barcelona, Spain"},{"name":"Institute of Biomedical Research August Pi i Sunyer (IDIPABS), Malalties Nefro-Urol\u00f2giques i Trasplantament Renal, Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Guill\u00e9n","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Kidney Transplantation, Hospital Cl\u00ednic, Centro de Referencia en Enfermedad Glomerular Compleja del Sistema Nacional de Salud (CSUR), University of Barcelona, Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7582-8476","authenticated-orcid":false,"given":"Luis F","family":"Quintana","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Kidney Transplantation, Hospital Cl\u00ednic, Centro de Referencia en Enfermedad Glomerular Compleja del Sistema Nacional de Salud (CSUR), University of Barcelona, Barcelona, Spain"},{"name":"Institute of Biomedical Research August Pi i Sunyer (IDIPABS), Malalties Nefro-Urol\u00f2giques i Trasplantament Renal, Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adriana","family":"Garcia-Herrera","sequence":"additional","affiliation":[{"name":"Department of Pathology, Hospital Cl\u00ednic, University of Barcelona, Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gast\u00f3n","family":"Pi\u00f1eiro","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Kidney Transplantation, Hospital Cl\u00ednic, Centro de Referencia en Enfermedad Glomerular Compleja del Sistema Nacional de Salud (CSUR), University of Barcelona, Barcelona, Spain"},{"name":"Institute of Biomedical Research August Pi i Sunyer (IDIPABS), Malalties Nefro-Urol\u00f2giques i Trasplantament Renal, Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6492-024X","authenticated-orcid":false,"given":"Esteban","family":"Poch","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Kidney Transplantation, Hospital Cl\u00ednic, Centro de Referencia en Enfermedad Glomerular Compleja del Sistema Nacional de Salud (CSUR), University of Barcelona, Barcelona, Spain"},{"name":"Institute of Biomedical Research August Pi i Sunyer (IDIPABS), Malalties Nefro-Urol\u00f2giques i Trasplantament Renal, Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Enric","family":"Carreras","sequence":"additional","affiliation":[{"name":"Josep Carreras Leukaemia Research Institute, Hospital Clinic\/University of Barcelona Campus, Barcelona, Spain"},{"name":"Barcelona Endothelium Team, Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Josep M","family":"Campistol","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Kidney Transplantation, Hospital Cl\u00ednic, Centro de Referencia en Enfermedad Glomerular Compleja del Sistema Nacional de Salud (CSUR), University of Barcelona, Barcelona, Spain"},{"name":"Institute of Biomedical Research August Pi i Sunyer (IDIPABS), Malalties Nefro-Urol\u00f2giques i Trasplantament Renal, Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maribel","family":"Diaz-Ricart","sequence":"additional","affiliation":[{"name":"Barcelona Endothelium Team, Barcelona, Spain"},{"name":"Department of Pathology, Hematopathology Unit, Hospital Cl\u00ednic of Barcelona, Biomedical Diagnosis Centre (CDB), Institute of Biomedical Research August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marta","family":"Palomo","sequence":"additional","affiliation":[{"name":"Josep Carreras Leukaemia Research Institute, Hospital Clinic\/University of Barcelona Campus, Barcelona, Spain"},{"name":"Barcelona Endothelium Team, Barcelona, Spain"},{"name":"Department of Pathology, Hematopathology Unit, Hospital Cl\u00ednic of Barcelona, Biomedical Diagnosis 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