Evidence map›Paper›PMID 35590114›Full record

ArticleClinical rheumatology2022

Performance of the EULAR/ACR 2019 classification criteria for systemic lupus erythematous in monogenic lupus.

Sulaiman M Al-Mayouf, Lujayn Akbar, Reem Abdwani, Giulia Ginesi, Stefano Volpi, Marco Gattorno, Reima Bakry, Samia AlHashim, Alhanouf Alsaleem

Abstract readMulticenter Study
PubMed Publisher
In one paragraph

Article in Clinical rheumatology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
7citing papers in PubMed, 1 pooled it
–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.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

7 citing papers in PubMed, 1 synthesis or guideline pooled it.

  1. Pooled it
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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.

Sulaiman M Al-MayoufPediatric Rheumatology, King Faisal Specialist Hospital and Research Center, College of Medicine, Alfaisal University, Po Box 3354, Riyadh, 11211, Saudi Arabia. mayouf@kfshrc.edu.sa.ORCID http://orcid.org/0000-0003-0142-6698
Lujayn AkbarPediatric Rheumatology, King Faisal Specialist Hospital and Research Center, College of Medicine, Alfaisal University, Po Box 3354, Riyadh, 11211, Saudi Arabia.
Reem AbdwaniPediatric Rheumatology, Sultan Qaboos University, Muscat, Oman.
Giulia GinesiPediatric Rheumatology, Centre for Autoinflammatory Diseases and Immunodeficiencies, IRCCS Istituto Giannina Gaslini, Genova, Italy.
Stefano VolpiPediatric Rheumatology, Centre for Autoinflammatory Diseases and Immunodeficiencies, IRCCS Istituto Giannina Gaslini, Genova, Italy.
Marco GattornoPediatric Rheumatology, Centre for Autoinflammatory Diseases and Immunodeficiencies, IRCCS Istituto Giannina Gaslini, Genova, Italy.
Reima BakryPediatric Rheumatology, East Jeddah Hospital, Jeddah, Saudi Arabia.
Samia AlHashimPediatric Rheumatology, King Faisal Specialist Hospital and Research Center, College of Medicine, Alfaisal University, Po Box 3354, Riyadh, 11211, Saudi Arabia.
Alhanouf AlsaleemPediatric Rheumatology, King Faisal Specialist Hospital and Research Center, College of Medicine, Alfaisal University, Po Box 3354, Riyadh, 11211, Saudi Arabia.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

objectiveTo evaluate the application of the EULAR/ACR-2019 criteria to monogenic lupus patients and compare its performance against the SLICC-2012 criteria.

methodsIn a multicenter retrospective cohort study, consecutive patients with monogenic lupus from three tertiary lupus clinics were enrolled. The diagnosis of monogenic lupus was based on the expert physician's opinion or fulfilling the SLICC-2012 criteria. All enrolled patients had genetic variants. A control group of sporadic childhood SLE (cSLE) and non-SLE patients, were included. A descriptive data analysis was conducted, and the EULAR/ACR-2019 and SLICC-2012 criteria were applied to both groups.

resultsForty-nine patients with monogenic lupus with a median age at diagnosis of 6.0 (IQR 3.0-10.8) years and 104 controls (55 patients with cSLE and 49 non-lupus patients with a median age at diagnosis of 10.0 and 5.0 respectively) were included. Forty-four (89.8%) patients with monogenic lupus fulfilled the EULAR/ACR-2019 with a mean score of 22.3±8.9. The most frequent domains were immunologic (93.9%), musculoskeletal and renal (each 57.1%), and mucocutaneous (55.1%). Fifty-four (98.2%) cSLE patients and six (12.2%) non-lupus patients met the EULAR/ACR-2019 criteria with a mean score of 22.5±9.2 and 8.5±5.2, respectively. The sensitivity of the EULAR/ACR-2019 criteria in monogenic lupus was 89.9% (95% CI: 78.3-90.2), while the specificity was 87.6% (95% CI: 75.2-88.7).

conclusionThis is the first and largest cohort of monogenic lupus patients testing the performance of the 2019-EULAR/ACR criteria. It efficiently classifies monogenic lupus patients, irrespective of the underlying genetic variants. Further studies are needed before these criteria are adopted worldwide. Key Points • Typically, patients with monogenic lupus have early onset severe disease, especially with mucocutaneous manifestations and a strong family history of SLE. • Monogenic lupus is a distinctive entity and might differ from the sporadic childhood SLE. • Our study includes a large multinational cohort of monogenic lupus with heterogeneous phenotypic features and underlying genetic variants. • Our study demonstrates that the EULAR/ACR-2019 criteria efficiently classified monogenic lupus patients, irrespective of the diversity of the underlying genetic variants.

Indexed as

Lupus Erythematosus, SystemicRheumatologyChildCohort StudiesHumansKidneyRetrospective StudiesEULAR/ACR-2019 criteriaMonogenic lupusSLICC-2012 criteriaSystemic lupus erythematosus

Identifiers

PMID35590114

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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.