{
  "abstract": "Background/Objectives: Systemic lupus erythematosus (SLE)-associated thrombotic microangiopathy (TMA) is a severe complication with high mortality. This study aimed to examine clinical manifestations, treatment strategies, and outcomes in these patients.\n\nMethods: This retrospective cohort study included 64 patients diagnosed with SLE-associated TMA between January 2016 and April 2025. Patients were stratified by ADAMTS13 activity into SLE-associated immune-mediated thrombotic thrombocytopenic purpura (SLE-iTTP, ADAMTS13 <10%) and non-iTTP TMA (ADAMTS13 ≥10%) groups. Clinical characteristics, laboratory findings, treatment responses, and outcomes were compared between the groups. Within the SLE-iTTP subgroup, outcomes were compared between patients receiving rituximab (RTX) and those receiving cyclophosphamide (CYC) as adjunctive immunosuppressive therapy.\n\nResults: Among 64 patients, 36 (56.3%) were classified as SLE-iTTP and 28 (43.7%) as non-iTTP TMA. Patients with SLE-iTTP exhibited more severe thrombocytopenia (median platelet count: 5.5 vs 39 × 109/L, p<0.001), lower hemoglobin levels (49.5 vs 69 g/L, p<0.001), and a higher frequency of neuropsychiatric manifestations (88.9% vs 57.1%, p=0.008). Anti-dsDNA positivity (86.1% vs 57.1%, p=0.012) and hypocomplementemia (94.4% vs 71.4%, p=0.016) were more prevalent in the SLE-iTTP group. Within the SLE-iTTP subgroup, all patients received glucocorticoids and plasma exchange. Patients treated with RTX (n=20) achieved a significantly higher rate of clinical remission or response than those treated with CYC (n=16) (85.0% vs 50.0%, p=0.034) and showed faster platelet count normalization (median 14 vs 21 days, p=0.018). Overall mortality was 34.4% (22/64).\n\nConclusion: ADAMTS13 activity identifies a distinct subset of SLE-associated TMA with characteristic features and differential treatment responses. In SLE-iTTP, RTX-based regimens was associated with better clinical efficacy than CYC, supporting a pathophysiology-guided therapeutic approach for this life-threatening condition.",
  "abstract_shown": true,
  "affiliations": [
    "Department of Rheumatology and Immunology, The Second Affiliated Hospital of Xiamen Medical College, Xiamen, 361021, People’s Republic of China"
  ],
  "article_type": "research article",
  "arxiv": null,
  "author_total": 8,
  "authors": [
    {
      "affs": [
        0
      ],
      "name": "Jinmei Huang"
    },
    {
      "affs": [
        0
      ],
      "name": "Wei Fan"
    },
    {
      "affs": [
        0
      ],
      "name": "Pingping Xiao"
    },
    {
      "affs": [
        0
      ],
      "name": "Shufan Wu"
    },
    {
      "affs": [
        0
      ],
      "name": "Yi Zhang",
      "orcid": "0000-0002-3962-4465"
    },
    {
      "affs": [
        0
      ],
      "name": "Hua Li"
    },
    {
      "affs": [
        0
      ],
      "name": "Xuyan Chen"
    },
    {
      "affs": [
        0
      ],
      "name": "Zhigao Dong"
    }
  ],
  "bibtex": "https://research.draconitic.com/paper/f8xzfzwe6q0k0.bib",
  "cited_by": 0,
  "doi": "10.2147/ijgm.s653266",
  "fulltext_url": "https://pmc.ncbi.nlm.nih.gov/articles/PMC13730106/",
  "id": "f8xzfzwe6q0k0",
  "issns": [
    "1178-7074"
  ],
  "landing_url": "https://doi.org/10.2147/ijgm.s653266",
  "language": "en",
  "licence": "CC-BY-NC-4.0",
  "licence_label": "CC BY-NC 4.0",
  "licence_source": "article",
  "licence_url": "https://creativecommons.org/licenses/by-nc/4.0/",
  "metadata_licence": null,
  "native_subjects": [
    [
      "heading",
      "Original Research"
    ],
    [
      "openalex",
      "2403|Complement system in diseases"
    ]
  ],
  "openalex": "W7224805703",
  "pdf_url": "https://www.dovepress.com/article/download/121609",
  "pmcid": "PMC13730106",
  "pmid": "42979218",
  "published": "2026-10-30",
  "publisher": "Dove Press",
  "ris": "https://research.draconitic.com/paper/f8xzfzwe6q0k0.ris",
  "sources": [
    {
      "first_seen": "2026-11-07T12:22:44Z",
      "native_id": "PMC13730106",
      "source": "pmc",
      "url": "https://doi.org/10.2147/ijgm.s653266"
    }
  ],
  "subjects": [
    {
      "field": "biology",
      "origin": "openalex:2403|Complement system in diseases",
      "subfield": "immunology"
    }
  ],
  "title": "ADAMTS13 Activity Stratifies SLE-Associated Thrombotic Microangiopathy: Rituximab versus Cyclophosphamide in Severe Deficiency Subgroup: A Retrospective Cohort Study",
  "title_text": "ADAMTS13 Activity Stratifies SLE-Associated Thrombotic Microangiopathy: Rituximab versus Cyclophosphamide in Severe Deficiency Subgroup: A Retrospective Cohort Study",
  "type": "peer-reviewed",
  "updated_at": "2026-11-07T12:22:44Z",
  "url": "https://research.draconitic.com/paper/f8xzfzwe6q0k0",
  "venue": "International Journal of General Medicine",
  "versions": []
}