Evidence map›Paper›PMID 34127049›Full record

Observational studyArthritis research & therapy2021

Clinical characteristics, visceral involvement, and mortality in at-risk or early diffuse systemic sclerosis: a longitudinal analysis of an observational prospective multicenter US cohort.

Sara Jaafar, Alain Lescoat, Suiyuan Huang, Jessica Gordon, Monique Hinchcliff, Ami A Shah, Shervin Assassi, Robyn Domsic, Elana J Bernstein, Virginia Steen and 10 more

Open access · goldAbstract readMulticenter StudyObservational Study
PubMed Full text at PMC Publisher
In one paragraph

Observational study in Arthritis research & therapy, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 33 papers, 2 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
33citing papers in PubMed, 2 pooled it
7.3field-weighted citation impact, top 2% 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

33 citing papers in PubMed, 2 syntheses or guidelines pooled it, 67 citations in OpenAlex.

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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

20 authors at 13 institutions in 2 countries.

Sara JaafarDivision of Rheumatology and Scleroderma Program, Department of Internal Medicine, University of Michigan, Suite 7C27 300 North Ingalls Street, SPC 5422, Ann Arbor, MI, 48109, USA.
Alain LescoatDivision of Rheumatology and Scleroderma Program, Department of Internal Medicine, University of Michigan, Suite 7C27 300 North Ingalls Street, SPC 5422, Ann Arbor, MI, 48109, USA.
Suiyuan HuangSchool of Public Health, University of Michigan, Ann Arbor, USA.
Jessica GordonDepartment of Medicine, Hospital for Special Surgery, New York, NY, USA.
Monique HinchcliffDepartment of Medicine, Northwestern University, Chicago, IL, USA.
Ami A ShahDepartment of Medicine, Johns Hopkins University, Baltimore, MD, USA.
Shervin AssassiDepartment of Medicine, University of Texas Health Science Center, Houston, TX, USA.
Robyn DomsicDepartment of Medicine, University of Pittsburgh Medical Center, Pittsburgh, PA, USA.
Elana J BernsteinDepartment of Medicine, Columbia University Irving Medical Center, New York, NY, USA.
Virginia SteenDepartment of Medicine, Georgetown University, Washington, DC, USA.
Sabrina ElliottDepartment of Medicine, Georgetown University, Washington, DC, USA.
Faye HantDepartment of Medicine, Medical University of South Carolina, Charleston, SC, USA.
Flavia V CastelinoDepartment of Medicine, Harvard University, Boston, MA, USA.
Victoria K ShanmugamDepartment of Medicine, George Washington University, Washington, DC, USA.
Chase CorreiaDepartment of Medicine, Northwestern University, Chicago, IL, USA.
John VargaDivision of Rheumatology and Scleroderma Program, Department of Internal Medicine, University of Michigan, Suite 7C27 300 North Ingalls Street, SPC 5422, Ann Arbor, MI, 48109, USA.
Vivek NagarajaDivision of Rheumatology and Scleroderma Program, Department of Internal Medicine, University of Michigan, Suite 7C27 300 North Ingalls Street, SPC 5422, Ann Arbor, MI, 48109, USA.
David RoofehDivision of Rheumatology and Scleroderma Program, Department of Internal Medicine, University of Michigan, Suite 7C27 300 North Ingalls Street, SPC 5422, Ann Arbor, MI, 48109, USA.
Tracy FrechDepartment of Medicine, University of Utah, Salt Lake City, UT, USA.
Dinesh KhannaDivision of Rheumatology and Scleroderma Program, Department of Internal Medicine, University of Michigan, Suite 7C27 300 North Ingalls Street, SPC 5422, Ann Arbor, MI, 48109, USA. khannad@umich.edu.ORCID 0000-0003-1412-4453
University of Michigan · USGeorgetown University · USNorthwestern University · USColumbia University Irving Medical Center · USGeorge Washington University · USHarvard University · USHospital for Special Surgery · USInserm · FRJohns Hopkins University · USMedical University of South Carolina · USThe University of Texas Health Science Center at Houston · USUniversity of Pittsburgh Medical Center · USUniversity of Utah · US

Funding

Yale Clinical and Translational Science Award (U Component)UL1TR001863 · NCATS · YALE UNIVERSITY · PI John H. Krystal, LUCILA OHNO-MACHADO · 2016 to 2026
$102.9M
Outcomes Research in Rheumatic DiseasesK24AR063120 · NIAMS · UNIVERSITY OF MICHIGAN AT ANN ARBOR · PI KHANNA, DINESH · 2012 to 2022
$1.6M
Systemic Sclerosis-Associated Interstitial Lung Disease Response IndexR01AR070470 · NIAMS · UNIVERSITY OF MICHIGAN AT ANN ARBOR · PI KHANNA, DINESH · 2017 to 2019
$642k
NCATS NIH HHS UL1 TR001863NIAMS NIH HHS K24 AR063120NIAMS NIH HHS K24-AR-063120NIAMS NIH HHS R01 AR070470
6 · The paper itself

Abstract

backgroundEarly diffuse cutaneous systemic sclerosis (dcSSc) has the highest case fatality among rheumatic diseases. We report baseline characteristics, current immunosuppressive therapies, progression of skin and internal organ involvement, and mortality in a multicenter prospective cohort from the United States (US) of America.

methodsWe performed a longitudinal analysis of participants from 12 US centers, from April 2012 to July 2020. All participants had early dcSSc or were at-risk for dcSSc, with ≤2 years since the first non-Raynaud's phenomenon (RP) symptom.

resultsThree hundred one patients were included with a baseline median disease duration of 1.2 years since RP and a mean modified skin score of 21.1 units. At baseline, 263 (87.3%) had definite dcSSc and 38 (12.7%) were classified as at-risk; 112 (49.6%) patients were positive for anti-RNA polymerase III antibodies. The median follow-up duration was 24.5 months (IQR = 10.3-40.7 months). One hundred ninety (63.1%) participants were treated with an immunosuppressive therapy, of which mycophenolate mofetil was most used at baseline and follow-up. Of 38 who were classified as at-risk at baseline, 27 (71%) went on to develop dcSSc; these patients were characterized by higher baseline mean HAQ-DI (0.8 versus 0.4, p = 0.05) and higher baseline mRSS (8.8 versus 4.4, p < 0.01) in comparison with those who remained as limited cutaneous SSc. In the overall cohort, 48 participants (21.1%) had clinically significant worsening of skin fibrosis, mainly occurring in the first year of follow-up; 41 (23.3%) had an absolute forced vital capacity decline of ≥10%. Twenty participants (6.6%) died, of which 18 died in the first 3 years of follow-up. Cardiac involvement (33.3%), gastrointestinal dysmotility (22.2%), and progressive interstitial lung disease (ILD) (16.7%) were the main causes of death.

conclusionThis US cohort highlights the management of early SSc in the current era, demonstrating progression of skin and lung involvement despite immunosuppressive therapy and high mortality due to cardiac involvement.

Indexed as

Lung Diseases, InterstitialScleroderma, DiffuseScleroderma, SystemicCohort StudiesHumansMycophenolic AcidProspective StudiesUnited StatesMycophenolic AcidDiffuse cutaneous systemic sclerosisInterstitial lung diseaseMortalitySclerodermaSurvivalSystemic sclerosis

Identifiers

PMID34127049
PMCPMC8201684
OpenAlexW3172438905

What OpenQuestion holds

Textmetadata
LicenceCC BY
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.