Evidence map›Paper›PMID 40375186›Full record

ArticleBMC nephrology2025

PLASMIC score to aid diagnosis of aHUS: an analysis of C5 inhibitor clinical trials and the PINC AI™ healthcare database.

Miguel G Uriol-Rivera, Frank R Ernst, John N Booth, Àngels Comas, Christoph Gasteyger, Ioannis Tomazos, Ching Lum, Yan Wang, Ana I Ávila

2 registry-linked trialsAbstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in BMC nephrology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to 2 registered trials, which are not on this map. Cited by 3 papers.

0numbers the graph read from it
0cells of the map it votes in
3citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

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.

2 · The registry

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.

NCT01194973 phase2completednot on this map

An Open-label, Multi-center Clinical Trial of Eculizumab in Adult Patients With Atypical Hemolytic-uremic Syndrome

TypeinterventionalSponsorAlexion Pharmaceuticals, Inc.Ran2010 to 2014Enrolled44ConditionsAtypical Hemolytic-Uremic SyndromeArmsEculizumab
NCT02949128 phase3completednot on this map

Single Arm Study of ALXN1210 in Complement Inhibitor Treatment-naïve Adult and Adolescent Patients With Atypical Hemolytic Uremic Syndrome (aHUS)

TypeinterventionalSponsorAlexion Pharmaceuticals, Inc.Ran2017 to 2023Enrolled58ConditionsAtypical Hemolytic Uremic Syndrome (aHUS)ArmsRavulizumab
3 · Its place in the literature

Who cites it

3 citing papers in PubMed.

  1. Review
  2. Review
  3. Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

9 authors.

Miguel G Uriol-RiveraGlomerular Diseases Unit, Son Espases University Hospital, Palma de Mallorca, Spain. miguelg.uriol@ssib.es.
Frank R ErnstCTI Clinical Trial and Consulting Services, Inc, Covington, KY, USA.
John N BoothCTI Clinical Trial and Consulting Services, Inc, Covington, KY, USA.
Àngels ComasAlexion, AstraZeneca Rare Disease, Barcelona, Spain.
Christoph GasteygerAlexion, AstraZeneca Rare Disease, Baar, Switzerland.
Ioannis TomazosAlexion, AstraZeneca Rare Disease, Boston, MA, USA.
Ching LumAlexion, AstraZeneca Rare Disease, Boston, MA, USA.
Yan WangAlexion, AstraZeneca Rare Disease, Boston, MA, USA.
Ana I ÁvilaNephrology Department, Dr Peset University Hospital, Valencia, Spain.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundAtypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy (TMA) that can lead to end organ damage and death without treatment. The ability to rapidly distinguish aHUS from other forms of TMA is key for optimal patient management. The PLASMIC Score was developed to identify individuals with thrombotic thrombocytopenic purpura (TTP), a TMA subtype characterized by severe ADAMTS13 deficiency (< 10%), using 7 commonly available laboratory variables and aspects of the patient's medical history. This study aimed to assess the distribution of PLASMIC Scores in patients with known aHUS, and evaluate the utility of the PLASMIC Score in the diagnostic pathway of aHUS in patients with confirmed TMA.

methodsData from eculizumab (NCT01194973) and ravulizumab (NCT02949128) clinical trials were utilized to calculate and evaluate PLASMIC Score distribution in aHUS patients. Real-world patient-level data from the PINC AI™ Healthcare Database (PHD) were used to evaluate the performance of the PLASMIC Score in identifying aHUS in patients with documented TMA diagnoses and renal impairment (primary analysis population; n = 110), and subsequent sensitivity analyses were performed in alternative populations.

resultsA total of 94 aHUS patients from the eculizumab and ravulizumab clinical trials dataset were evaluated; 18/36 (50.0%) and 27/58 (46.6%) patients in the eculizumab and ravulizumab trials, respectively, had a PLASMIC Score of 4, and most patients (~ 85%) had PLASMIC Scores ≤ 5 (range: 3-5), which were distributed similarly between the trials. Among the 110 patients with undifferentiated TMA (primary analysis) from the PHD, a PLASMIC Score cutoff of ≤ 5 yielded sensitivity, specificity and positive predictive value (PPV) and negative predictive values (NPV) of 86.5%, 71.4%, 92.8% and 55.6%, respectively, for identifying probable aHUS. Similar diagnostic performance was observed at a cutoff value of ≤ 5 in further sensitivity analyses. A cutoff value of ≤ 4 yielded a lower PPV (62.9%), yet a higher NPV (85.7%), with only 3 patients misclassified as TTP.

conclusionApplication of the PLASMIC Score in the aHUS diagnostic pathway may support clinical judgement and ascertain confidence in the earlier identification and subsequent treatment of patients with aHUS, thereby improving patient outcomes.

Indexed as

Atypical Hemolytic Uremic SyndromeComplement C5AdultAntibodies, Monoclonal, HumanizedClinical Trials as TopicClinical Trials, Phase II as TopicClinical Trials, Phase III as TopicComplement Inactivating AgentsDatabases, FactualFemaleHumansMaleMiddle AgedMulticenter Studies as TopicAntibodies, Monoclonal, HumanizedComplement C5Complement Inactivating AgentseculizumabravulizumabaHUSDiagnosisEculizumabPLASMIC scoreRavulizumabTMATTP

Identifiers

PMID40375186
PMCPMC12080142

What OpenQuestion holds

Textmetadata
LicenceCC BY-NC-ND
Read underepoch 390

Registered trials

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.