Trial reportBlood advances2018
Complement-mediated thrombotic microangiopathy associated with lupus nephritis.
Trial report in Blood advances, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 56 papers, 2 of them syntheses that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
56 citing papers in PubMed, 2 syntheses or guidelines pooled it, 101 citations in OpenAlex.
- II Brazilian Society of Rheumatology consensus for lupus nephritis diagnosis and treatment.Advances in rheumatology (London, England) · 2024Guideline
- A systematic review of the role of eculizumab in systemic lupus erythematosus-associated thrombotic microangiopathy.BMC nephrology · 2020Pooled it
- IPNA clinical practice recommendations for the management of aHUS and secondary TMA in children.Pediatric nephrology (Berlin, Germany) · 2026Article
- Article
- Thrombotic Microangiopathy in Childhood Steroid-Resistant Nephrotic Syndrome: Case Series.Nephron · 2026Article
- Article
- Sustained remission with eculizumab in refractory lupus nephritis with atypical haemolytic uraemic syndrome.BMJ case reports · 2025Article
- Complement activation in secondary thrombotic microangiopathies.Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association · 2025Review
- Article
- Management of Atypical Haemolytic Uraemic Syndrome With Triggers: Diagnostic and Treatment Algorithms From an Asia-Pacific Perspective.Nephrology (Carlton, Vic.) · 2025Review
- Plasminogen mutation-associated thrombotic microangiopathy and role of anticoagulation: a single institution case series.Research and practice in thrombosis and haemostasis · 2025Article
- Outcome of proliferative lupus nephritis with thrombotic microangiopathy; An ambispective observational single-center study.BMC nephrology · 2025Observational
- Post-transplant Thrombotic Microangiopathy.Journal of the American Society of Nephrology : JASN · 2025Review
- Recurrent Wunderlich syndrome in systemic lupus erythematosus: a case report.International journal of emergency medicine · 2025Article
- Systemic lupus erythematosus: updated insights on the pathogenesis, diagnosis, prevention and therapeutics.Signal transduction and targeted therapy · 2025Review
- Targeted Complement Treatments in Glomerulopathies: A Comprehensive Review.Journal of clinical medicine · 2025Review
- Renal vascular lesions in childhood-onset lupus nephritis.Pediatric nephrology (Berlin, Germany) · 2025Article
- Cholecystectomy-induced thrombotic microangiopathy (TMA) in a postpartum patient successfully treated with eculizumab: a case report.Journal of medical case reports · 2024Article
- Primary immunodeficiency as a cause of immune-mediated kidney diseases.Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association · 2024Review
- Systemic lupus erythematosus presenting with atypical hemolytic uremic syndrome: a case report and review of the literature.Rheumatology international · 2024Review
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
4 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Complement-mediated thrombotic microangiopathy (CM-TMA) is a clinical disorder driven by the generation of excess complement. It is characterized by thrombocytopenia and microangiopathic hemolytic anemia (MAHA) with microvascular thrombosis resulting in systemic organ damage (TMA). One form of CM-TMA, atypical hemolytic uremic syndrome (aHUS), is characterized by pathologic complement activation due to the loss of the natural regulators of the complement system, which results in systemic endothelial and organ damage. Lupus erythematosus is a multisystem immune complex disorder associated with activation of complement, as well as renal failure termed lupus nephritis (LN). A subset of these patients also develop TMA, with progressive life-threatening thrombocytopenia, MAHA, and progressive renal failure similar to aHUS. This subset of patients is poorly responsive to corticosteroids, cyclophosphamide, immunomodulation, and plasma exchange. In this article, we report 11 cases of LN associated with TMA progressing through these therapies, 10 of which were successfully treated with complement inhibition. Complement-regulatory protein mutations, including complement factor H (CFH), factor I, factor B, membrane cofactor/(CD46) and thrombomodulin, and CFH-related 1-3, were identified in 6 of 10 patients tested. One patient had a loss of a renal allograft. Three patients had concurrent antiphospholipid syndrome. Two of the 3 patients had normal D-dimers at this presentation. We believe that this subset of lupus patients, with clinical and pathologic manifestations of aHUS, define a separate entity that we believe should be termed CM-TMA associated with LN.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.