{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,9]],"date-time":"2026-06-09T16:57:24Z","timestamp":1781024244394,"version":"3.54.1"},"reference-count":39,"publisher":"S. Karger AG","issue":"1","license":[{"start":{"date-parts":[[2026,4,20]],"date-time":"2026-04-20T00:00:00Z","timestamp":1776643200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,4,20]],"date-time":"2026-04-20T00:00:00Z","timestamp":1776643200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Kidney Dis"],"abstract":"<jats:p>Introduction: Anti-complement factor H autoantibodies (FHAAs)-associated complement-mediated thrombotic microangiopathy (CM-TMA) is prevalent in pediatric patients and plays an important role in the pathogenesis of CM-TMA. The epitopes of FHAAs in adult CM-TMA patients remain to be explored. Methods: A total of 114 adult patients with CM-TMA were enrolled. Their clinicopathological data as well as plasma samples were collected. Seven CFH fragments covering different short consensus repeats (SCRs) of factor H were expressed by HEK293 or ExpiCHO cells. The presence of FHAAs and the antigenic epitopes were detected by using enzyme-linked immunosorbent assay. Results: FHAAs were detected in 10 of the adult patients with CM-TMA. The main epitope of FHAAs was SCRs 15-20 (88.89%, 8\/9). The prevalence of anti-SCRs 1-4, anti-SCRs 1-6, anti-SCRs 5-7, anti-SCR 7, anti-SCRs 11-14, and anti-SCRs 19-20 autoantibodies was 22.22% (2\/9), 0% (0\/9), 33.33% (3\/9), 11.11% (1\/9), 0% (0\/9), and 44.44% (4\/9), respectively. The levels of anti-SCRs 15-20 autoantibodies were significantly decreased in the remission phase compared with that in the active phase (107.49 \u00b1 36.8 AU\/mL vs. 181.52 \u00b1 33.62 AU\/mL, p = 0.04). Malignant hypertension was found in 60% (6\/10) of patients with FHAA-associated CM-TMA. Eighty percent (8\/10) of these patients had a poor prognosis, including 5 with end-stage kidney disease, 2 with renal transplantation, and 1 patient who died. Conclusion: FHAAs were positive in 8.77% (10\/114) of adult CM-TMA patients and SCRs 15-20 might be the main epitope of FHAAs in FHAA-associated CM-TMA.<\/jats:p>","DOI":"10.1159\/000551284","type":"journal-article","created":{"date-parts":[[2026,4,20]],"date-time":"2026-04-20T16:01:11Z","timestamp":1776700871000},"page":"402-412","source":"Crossref","is-referenced-by-count":0,"title":["Immunological Characteristics of Chinese Adult Patients with Anti-Complement Factor H Autoantibodies-Associated Complement-Mediated Thrombotic Microangiopathy"],"prefix":"10.1159","volume":"12","author":[{"given":"Shuqi","family":"Yang","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaojing","family":"Sun","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1532-7922","authenticated-orcid":false,"given":"Meng","family":"Tan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8139-5758","authenticated-orcid":false,"given":"Ying","family":"Tan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Minghui","family":"Zhao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"127","published-online":{"date-parts":[[2026,4,20]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra020528"},{"key":"ref2","doi-asserted-by":"publisher","DOI":"10.1016\/s0753-3322(00)00007-x"},{"key":"ref3","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.73.9.3268"},{"key":"ref4","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2009-02-205641"},{"key":"ref5","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2022.866330"},{"key":"ref6","doi-asserted-by":"publisher","DOI":"10.1038\/ni.1755"},{"key":"ref7","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2014-10-609073"},{"key":"ref8","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2020.01728"},{"key":"ref9","doi-asserted-by":"publisher","DOI":"10.1111\/imm.12916"},{"key":"ref10","doi-asserted-by":"publisher","DOI":"10.1021\/bi4003452"},{"key":"ref11","doi-asserted-by":"publisher","DOI":"10.1016\/j.jbc.2024.107624"},{"key":"ref12","doi-asserted-by":"publisher","DOI":"10.1681\/ASN.2004050380"},{"key":"ref13","doi-asserted-by":"publisher","DOI":"10.1159\/000513009"},{"key":"ref14","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2019.01282"},{"key":"ref15","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2021.06.002"},{"key":"ref16","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2020.611830"},{"key":"ref17","doi-asserted-by":"publisher","DOI":"10.1158\/2326-6066.CIR-20-0787"},{"key":"ref18","doi-asserted-by":"publisher","DOI":"10.3760\/cma.j.issn.0578-1310.2017.08.015"},{"key":"ref19","doi-asserted-by":"publisher","DOI":"10.1159\/000445127"},{"key":"ref20","doi-asserted-by":"publisher","DOI":"10.1007\/s00467-018-4074-4"},{"key":"ref21","doi-asserted-by":"publisher","DOI":"10.1016\/j.copbio.2009.10.008"},{"key":"ref22","doi-asserted-by":"publisher","DOI":"10.1007\/s00467-016-3562-7"},{"key":"ref23","doi-asserted-by":"publisher","DOI":"10.1080\/0886022X.2022.2161396"},{"key":"ref24","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra0902814"},{"key":"ref25","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2019.00853"},{"key":"ref26","doi-asserted-by":"publisher","DOI":"10.1080\/0886022X.2022.2089167"},{"key":"ref27","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2020.607211"},{"key":"ref28","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2009-05-221549"},{"key":"ref29","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M114.630871"},{"key":"ref30","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.1200679"},{"key":"ref31","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2007-02-071472"},{"key":"ref32","doi-asserted-by":"publisher","DOI":"10.1016\/j.molimm.2015.12.005"},{"key":"ref33","doi-asserted-by":"publisher","DOI":"10.1007\/s00251-003-0605-6"},{"key":"ref34","doi-asserted-by":"publisher","DOI":"10.1016\/j.jmb.2022.167928"},{"key":"ref35","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2007-09-109876"},{"key":"ref36","doi-asserted-by":"publisher","DOI":"10.2215\/CJN.04760512"},{"key":"ref37","doi-asserted-by":"publisher","DOI":"10.1038\/ki.1979.70"},{"key":"ref38","doi-asserted-by":"publisher","DOI":"10.2215\/CJN.01260212"},{"key":"ref39","doi-asserted-by":"publisher","DOI":"10.1155\/2021\/6904858"}],"container-title":["Kidney 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