ArticleKidney international reports2021
Role of C5 inhibition in Idiopathic Inflammatory Myopathies and Scleroderma Renal Crisis-Induced Thrombotic Microangiopathies.
Article in Kidney international reports, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.
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Who cites it
13 citing papers in PubMed, 20 citations in OpenAlex.
- Distinct Effects of Complement C4A and C4B Copy Numbers in Systemic Sclerosis Serological and Clinical Subtypes.Arthritis & rheumatology (Hoboken, N.J.) · 2026Article
- Update in the diagnosis of complement-mediated thrombotic microangiopathy/atypical hemolytic uremic syndrome.Hematology. American Society of Hematology. Education Program · 2025Review
- Histologic Features Associated with Kidney Survival in Scleroderma Renal Crisis.Journal of the American Society of Nephrology : JASN · 2025Article
- Management of Atypical Haemolytic Uraemic Syndrome With Triggers: Diagnostic and Treatment Algorithms From an Asia-Pacific Perspective.Nephrology (Carlton, Vic.) · 2025Review
- The complement system in autoimmune diseases: pathogenesis, diagnostic markers, and therapeutic strategies.Inflammation research : official journal of the European Histamine Research Society ... [et al.] · 2025Review
- Targeting complement dysregulation: eculizumab in scleroderma renal crisis management-a case-based review.Rheumatology international · 2024Review
- Thrombotic Microangiopathy and Multiple Organ Failure in Scleroderma Renal Crisis: A Case Report.Cureus · 2023Article
- Renal Disease and Systemic Sclerosis: an Update on Scleroderma Renal Crisis.Clinical reviews in allergy & immunology · 2023Review
- Article
- Complement component C4 structural variation and quantitative traits contribute to sex-biased vulnerability in systemic sclerosis.NPJ genomic medicine · 2022Article
- Thrombotic Microangiopathy Secondary to Systemic Sclerosis with Severe Complement Activation Not Responsive to Eculizumab: A Case Report.The American journal of case reports · 2022Article
- A glance into the future of myositis therapy.Therapeutic advances in musculoskeletal disease · 2022Review
- C5 Inhibition in Secondary Thrombotic Microangiopathies: A Yet Unresolved Question.Kidney international reports · 2021Article
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Authors and funding
11 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
introductionConnective tissue diseases, including systemic sclerosis and idiopathic inflammatory myopathies (IIMs), are a very rare cause of thrombotic microangiopathies (TMAs). Whether dysregulation of the complement pathways underlies these secondary forms of TMA and may be targeted by complement blocking agents remains elusive.
methodsKidney pathology and outcomes of 18 critically ill patients with TMA related to inflammatory myopathy flare-up (IIM,
resultsIIM-TMA is characterized by acute thrombotic lesions only, whereas SRC-TMA patients also harbored chronic vascular lesions and more interstitial fibrosis. C5b9 deposits, a marker of complement component 5 (C5) cleavage, were observed in the 2 subgroups at the junction of media and intima of arterioles, colocalizing with subendothelial edema. Thus, kidney biopsy distinguished between acute and chronic renal phenotypes that may help to individualize treatment. Treatment of IIM-TMA patients with combined full-code organ support, corticosteroids, B-cell depletion, and complement C5 blocking led to 1-year survival of 72%, compared with 19% in historical cohorts. Treatment of SRC-TMA was more heterogenous and relied on conversion enzyme inhibitor only or with eculizumab (
conclusionC5 blocking may reverse hematologic abnormalities in IIM- and SRC-TMA, and adding an early and aggressive immunosuppressive regimen may improve the survival of IIM-TMA. Underlying chronic vascular and interstitial lesions mitigate renal response in SRC-TMA.
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