Evidence map›Paper›PMID 36624431›Full record

ArticleRespiratory research2023

Expert consensus on the management of systemic sclerosis-associated interstitial lung disease.

Franck F Rahaghi, Vivien M Hsu, Robert J Kaner, Maureen D Mayes, Ivan O Rosas, Rajan Saggar, Virginia D Steen, Mary E Strek, Elana J Bernstein, Nitin Bhatt and 14 more

Open access · goldAbstract readConsensus Statement
PubMed Full text at PMC Publisher
In one paragraph

Article in Respiratory research, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 71 papers, 6 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
71citing papers in PubMed, 6 pooled it
34.2field-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

71 citing papers in PubMed, 6 syntheses or guidelines pooled it, 132 citations in OpenAlex.

  1. International Society for Heart and Lung Transplantation Scientific Statement on pulmonary antibody-mediated rejection and proposed graft, antibody, and pathology (GAP) definition.The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation · 2026
    Guideline
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11 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

24 authors at 20 institutions in 1 country.

Franck F RahaghiRespiratory Center, Cleveland Clinic Florida, 2950 Cleveland Clinic Blvd, Weston, FL, 33331, USA. RAHAGHF@ccf.org.
Vivien M HsuRutgers-RWJ Medical School, New Brunswick, NJ, USA.
Robert J KanerWeill Cornell Medicine, New York, NY, USA.
Maureen D MayesUniversity of Texas, Houston, TX, USA.
Ivan O RosasBrigham and Women's Hospital, Boston, MA, USA.
Rajan SaggarUniversity of California Los Angeles, Los Angeles, CA, USA.
Virginia D SteenGeorgetown University, Washington, D.C, USA.
Mary E StrekUniversity of Chicago, Chicago, IL, USA.
Elana J BernsteinColumbia University Irving Medical Center, New York, NY, USA.
Nitin BhattOhio State University, Columbus, OH, USA.
Flavia V CastelinoHarvard Medical School, Boston, MA, USA.
Lorinda ChungStanford University School of Medicine and Palo Alto VA Health Care System, Stanford, CA, USA.
Robyn T DomsicUniversity of Pittsburgh Medical Center, Pittsburgh, PA, USA.
Kevin R FlahertyUniversity of Michigan Scleroderma Clinic, Ann Arbor, MI, 48105, USA.
Nishant GuptaUniversity of Cincinnati, Cincinnati, OH, USA.
Bashar KahalehUniversity of Toledo Medical Center, Toledo, OH, USA.
Fernando J MartinezWeill Cornell Medicine, New York, NY, USA.
Lee E MorrowCreighton University, Omaha, NE, USA.
Teng MouaMayo Clinic, Rochester, MN, USA.
Nina PatelColumbia University Irving Medical Center, New York, NY, USA.
Oksana A ShlobinInova Fairfax Hospital, Falls Church, VA, USA.
Brian D SouthernCleveland Clinic, Cleveland, OH, USA.
Elizabeth R VolkmannUniversity of California Los Angeles, Los Angeles, CA, USA.
Dinesh KhannaUniversity of Michigan Scleroderma Clinic, Ann Arbor, MI, 48105, USA. khannad@umich.edu.
Columbia University Irving Medical Center · USCornell University · USUniversity of California, Los Angeles · USBrigham and Women's Hospital · USCleveland Clinic · USCleveland Clinic Florida · USCreighton University · USGeorgetown University · USHarvard University · USInova Fairfax Hospital · USMayo Clinic in Arizona · USRutgers, The State University of New Jersey · USScleroderma Foundation · USStanford University · USThe Ohio State University · USUniversity of Cincinnati · USUniversity of Houston · USUniversity of Illinois Chicago · USUniversity of Michigan–Ann Arbor · USUniversity of Pittsburgh Medical Center · US

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundSystemic sclerosis (SSc) is a rare, complex, connective tissue disorder. Interstitial lung disease (ILD) is common in SSc, occurring in 35-52% of patients and accounting for 20-40% of mortality. Evolution of therapeutic options has resulted in a lack of consensus on how to manage this condition. This Delphi study was initiated to develop consensus recommendations based on expert physician insights regarding screening, progression, treatment criteria, monitoring of response, and the role of recent therapeutic advances with antifibrotics and immunosuppressants in patients with SSc-ILD.

methodsA modified Delphi process was completed by pulmonologists (n = 13) and rheumatologists (n = 12) with expertise in the management of patients with SSc-ILD. Panelists rated their agreement with each statement on a Likert scale from - 5 (complete disagreement) to + 5 (complete agreement). Consensus was predefined as a mean Likert scale score of ≤  - 2.5 or ≥  + 2.5 with a standard deviation not crossing zero.

resultsPanelists recommended that all patients with SSc be screened for ILD by chest auscultation, spirometry with diffusing capacity of the lungs for carbon monoxide, high-resolution computed tomography (HRCT), and/or autoantibody testing. Treatment decisions were influenced by baseline and changes in pulmonary function tests, extent of ILD on HRCT, duration and degree of dyspnea, presence of pulmonary hypertension, and potential contribution of reflux. Treatment success was defined as stabilization or improvement of signs or symptoms of ILD and functional status. Mycophenolate mofetil was identified as the initial treatment of choice. Experts considered nintedanib a therapeutic option in patients with progressive fibrotic ILD despite immunosuppressive therapy or patients contraindicated/unable to tolerate immunotherapy. Concomitant use of nintedanib with MMF/cyclophosphamide can be considered in patients with advanced disease at initial presentation, aggressive ILD, or significant disease progression. Although limited consensus was achieved on the use of tocilizumab, the experts considered it a therapeutic option for patients with early SSc and ILD with elevated acute-phase reactants.

conclusionsThis modified Delphi study generated consensus recommendations for management of patients with SSc-ILD in a real-world setting. Findings from this study provide a management algorithm that will be helpful for treating patients with SSc-ILD and addresses a significant unmet need.

Indexed as

Lung Diseases, InterstitialScleroderma, SystemicConsensusHumansImmunosuppressive AgentsLungMycophenolic AcidImmunosuppressive AgentsMycophenolic AcidAlgorithmsAutoimmune diseasesConnective tissue diseasesDrug therapyPulmonary fibrosis

Identifiers

PMID36624431
PMCPMC9830797
OpenAlexW4313893987

What OpenQuestion holds

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LicenceCC BY
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Registered trials

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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.