Evidence map›Paper›PMID 42018270›Full record

ReviewClinical rheumatology2026

MDA5-associated juvenile dermatomyositis and interstitial lung disease from rapidly progressive to silent: a report of three cases in South African children and a review of the literature.

Maurane Lepage, Gabriella Pereira, Shehnaaz Akhalwaya, Taryn Gray, Marco Zampoli, Mignon McCulloch, Peter Nourse, Ashton Coetzee, Claire Procter, Khanyisile Hlongwa and 2 more

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PubMed Full text at PMC Publisher
In one paragraph

Review in Clinical rheumatology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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0cells of the map it votes in
0citing papers in PubMed
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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

0 citing papers in PubMed.

No citing paper in PubMed yet.

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

12 authors.

Maurane LepageDepartment of Paediatric Rheumatology, Red Cross War Memorial Children's Hospital, University of Cape Town, Cape Town, South Africa.ORCID http://orcid.org/0009-0006-3592-5347
Gabriella PereiraDepartment of Paediatric Rheumatology, Red Cross War Memorial Children's Hospital, University of Cape Town, Cape Town, South Africa.
Shehnaaz AkhalwayaDepartment of Paediatric Rheumatology, Red Cross War Memorial Children's Hospital, University of Cape Town, Cape Town, South Africa.
Taryn GrayChest and Allergy Centre, Christian Barnard Memorial Hospital, Cape Town, South Africa.
Marco ZampoliDepartment of Paediatric Pulmonary, Red Cross War Memorial Children's Hospital, University of Cape Town, Cape Town, South Africa.
Mignon McCullochDepartment of Paediatric Nephrology, Red Cross War Memorial Children's Hospital, University of Cape Town, Cape Town, South Africa.
Peter NourseDepartment of Paediatric Nephrology, Red Cross War Memorial Children's Hospital, University of Cape Town, Cape Town, South Africa.
Ashton CoetzeeDepartment of Paediatric Nephrology, Red Cross War Memorial Children's Hospital, University of Cape Town, Cape Town, South Africa.
Claire ProcterPaediatric Intensive Care Unit, Red Cross War Memorial Children's Hospital, University of Cape Town, Cape Town, South Africa.
Khanyisile HlongwaNuclear Medicine, Red Cross War Memorial Children's Hospital, University of Cape Town, Cape Town, South Africa.
Tanyia PillayDepartment of Radiology, Red Cross War Memorial Children's Hospital, University of Cape Town, Rondebosch, Cape Town, South Africa.
Kate WebbDepartment of Paediatric Rheumatology, Red Cross War Memorial Children's Hospital, University of Cape Town, Cape Town, South Africa. kate.webb@uct.ac.za.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundJuvenile dermatomyositis (JDM) is a rare pediatric autoimmune disease. A distinct clinical phenotype is associated with anti-melanoma differentiation-associated gene 5 (anti-MDA5) autoantibodies, which are linked to features such as arthritis, ulcerative skin lesions, and a heightened risk of interstitial lung disease (ILD), including its rapidly progressive form (RP-ILD). Despite increased recognition of this phenotype in East Asian, European, and North American populations, significant gaps remain in understanding its pathogenesis, and no consensus has been reached regarding optimal treatment strategies. Moreover, data on anti-MDA5-associated JDM in African populations are nonexistent. CASE PRESENTATION: We report the first three documented cases of anti-MDA5-positive JDM with ILD in African children. All patients exhibited characteristic extramuscular manifestations, and all had pulmonary involvement, which was rapidly progressive in two children, one of whom died. The clinical course, diagnostic findings, and treatment strategies are discussed in the context of existing literature.

methodsA review of the literature was performed to evaluate the prevalence, clinical presentation, and treatment approaches for RP-ILD in anti-MDA5-associated JDM across different populations.

conclusionThese cases highlight the wide heterogeneity of clinical phenotypes associated with anti-MDA5 autoantibodies in JDM. Given this variability, individualized monitoring and management strategies are essential to optimize outcomes.

Indexed as

AutoantibodiesDermatomyositisInterferon-Induced Helicase, IFIH1Lung Diseases, InterstitialChildChild, PreschoolDisease ProgressionFemaleHumansMaleSouth AfricaAutoantibodiesIFIH1 protein, humanInterferon-Induced Helicase, IFIH1AfricaAnti-MDA5Juvenile dermatomyositisRapidly progressive interstitial lung disease

Identifiers

PMID42018270
PMCPMC13342017

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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.