Evidence map›Paper›PMID 21531040›Full record

ArticleJournal of the American Academy of Dermatology2011

The mucocutaneous and systemic phenotype of dermatomyositis patients with antibodies to MDA5 (CADM-140): a retrospective study.

David Fiorentino, Lorinda Chung, Jeff Zwerner, Antony Rosen, Livia Casciola-Rosen

Open access · greenAbstract readComparative Study
In one paragraph

Article in Journal of the American Academy of Dermatology, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 227 papers, 2 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
227citing papers in PubMed, 2 pooled it
16.8field-weighted citation impact, top 1% of its field
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

227 citing papers in PubMed, 2 syntheses or guidelines pooled it, 545 citations in OpenAlex.

  1. Pooled it
  2. Pooled it
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  5. How loss-of-function mutations inJournal of translational autoimmunity · 2026
    Review
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  11. Digital ulcerations in amyopathic dermatomyositis.The Korean journal of internal medicine · 2026
    Article
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  16. Review
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167 more citing papers are in PubMed but not listed here.

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

5 authors at 4 institutions in 1 country.

David FiorentinoDepartment of Dermatology, Stanford University School of Medicine, Stanford, California, USA. fiorentino@stanford.edu
Lorinda Chung
Jeff Zwerner
Antony Rosen
Livia Casciola-Rosen
Johns Hopkins University · USStanford Medicine · USStanford University · USVA Palo Alto Health Care System · US

Funding

Phenotype-Specific Immune Responses in the Systemic VasculitidesP30AR053503 · NIAMS · JOHNS HOPKINS UNIVERSITY · PI ROSEN, ANTONY · 2006 to 2015
$6.4M
Apoptosis in Sjogrens SyndromeR37DE012354 · NIDCR · JOHNS HOPKINS UNIVERSITY · PI ROSEN, ANTONY · 2002 to 2011
$5.3M
Rheumatic Disease Sera: Probes of Disease MechanismsR01AR044684 · NIAMS · JOHNS HOPKINS UNIVERSITY · PI CASCIOLA-ROSEN, LIVIA A · 1997 to 2012
$3.7M
Mechanisms in Sjogrens SyndromeR01DE012354 · NIDCR · JOHNS HOPKINS UNIVERSITY · PI ROSEN, ANTONY · 1998 to 2017
$3.4M
NIAMS NIH HHS P30 AR053503NIAMS NIH HHS P30-AR-053503NIAMS NIH HHS R01 AR044684NIAMS NIH HHS R01 AR-44684NIDCR NIH HHS R01 R37-DE-12354NIDCR NIH HHS R37 DE012354
6 · The paper itself

Abstract

backgroundDermatomyositis (DM) is a multisystem autoimmune disease, in which serologic evidence of immune responses to disease-specific antigenic targets is found in approximately 50% to 70% of patients. Recently, melanoma differentiation-associated gene 5 (MDA5) has been identified as a DM-specific autoantigen that appears to be targeted in patients with DM and mild or absent muscle inflammation and with an increased risk of interstitial lung disease.

objectiveWe wished to understand the role of MDA5 in DM skin inflammation by testing it to determine if a specific cutaneous phenotype is associated with MDA5 reactivity.

methodsWe retrospectively screened plasma from 77 patients with DM in the outpatient clinics at the Stanford University Department of Dermatology in California.

resultsWe found that 10 (13%) patients had circulating anti-MDA5 antibodies, and had a characteristic cutaneous phenotype consisting of skin ulceration, tender palmar papules, or both. Typical areas of skin ulceration included the lateral nailfolds, Gottron papules, and elbows. Biopsy specimens of the palmar papules showed a vasculopathy characterized by vascular fibrin deposition with variable perivascular inflammation. Patients with anti-MDA5 antibodies also had an increased risk of oral pain and/or ulceration, hand swelling, arthritis/arthralgia, and diffuse hair loss. Consistent with previous reports, these patients had little or no myositis and had increased risk of interstitial lung disease. LIMITATIONS: This study was conducted at a tertiary referral center. Multiple associations with MDA5 antibodies were tested retrospectively on a relatively small cohort of 10 anti-MDA5-positive patients.

conclusionWe suggest that MDA5 reactivity in DM characterizes a patient population with severe vasculopathy.

Indexed as

AdultAutoantibodiesBiopsy, NeedleCohort StudiesDEAD-box RNA HelicasesDermatomyositisDisease ProgressionFemaleGene Expression RegulationGenetic Predisposition to DiseaseHumansImmunohistochemistryIntercellular Signaling Peptides and ProteinsInterferon-Induced Helicase, IFIH1MaleMiddle AgedAutoantibodiesCADM-140 peptide, humanDEAD-box RNA HelicasesIFIH1 protein, humanIntercellular Signaling Peptides and ProteinsInterferon-Induced Helicase, IFIH1Peptides

Identifiers

PMID21531040
PMCPMC3167687
OpenAlexW2115837022

What OpenQuestion holds

Textmetadata
LicenceTDM
Read underepoch 390

Registered trials

None linked

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.