Evidence map›Paper›PMID 42508943›Full record

Observational studyRMD open2026

Characteristics and outcomes of anti-Ku antibody-associated interstitial lung disease: a retrospective study of 60 patients.

Marie Robert, Yann Nguyen, Marie-Pierre Debray, Raphael Borie, Yurdagül Uzunhan, Yves Allenbach, Karim Sacré, Benjamin Terrier, Zahir Amoura, Céline Comparon and 29 more

Abstract readMulticenter StudyObservational Study
In one paragraph

Observational study in RMD open, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

0numbers the graph read from it
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

39 authors.

Marie RobertDepartment of Rheumatology, Université Paris Saclay, FHU CARE, INSERM UMR1184, CEA, AP-HP, Hôpital Bicêtre, Le Kremlin-Bicetre, France.ORCID http://orcid.org/0000-0002-1374-6210
Yann NguyenDepartment of Internal Medicine, Hôpital Beaujon, Assistance Publique-Hôpitaux de Paris, Université Paris Cité, Clichy, France.ORCID http://orcid.org/0000-0002-0866-3824
Marie-Pierre DebrayDepartment of Radiology, Hôpital Bichat, Assistance Publique-Hôpitaux de Paris, Université Paris Cité, Paris, France.ORCID http://orcid.org/0000-0003-2109-5662
Raphael BorieUMR Inserm 1149, CRI, Service de Pneumologie Allergologie et Transplantation, Hôpital Bichat, FHU INFIRE, Université Paris Cité, Assistance Publique-Hôpitaux de Paris, Paris, France.
Yurdagül UzunhanDepartment of Pneumology, Hôpital Avicenne, Assistance Publique-Hôpitaux de Paris, INSERM U1272, Université Sorbonne Paris Nord, Bobigny, France.
Yves AllenbachDepartment of Internal Medicine and Clinical Immunology, Hôpital Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Sorbonne Université, Paris, France.ORCID http://orcid.org/0000-0002-3185-7993
Karim SacréDepartment of Internal Medicine, Hôpital Bichat, Assistance Publique-Hôpitaux de Paris, Université Paris Cité, Paris, France.ORCID http://orcid.org/0000-0002-6544-234X
Benjamin TerrierDepartment of Internal Medicine, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris Cité, Paris, France.ORCID http://orcid.org/0000-0001-6612-7336
Zahir AmouraDepartment of Internal Medicine 2, Hôpital Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Sorbonne Université, Paris, France.
Céline ComparonDepartment of Internal Medicine, Hôpital Avicenne, Assistance Publique-Hôpitaux de Paris, Sorbonne Université, Bobigny, France.
Philippe DieudéDepartment of Rheumatology, Hôpital Bichat, Assistance Publique-Hôpitaux de Paris, Université Paris Cité, Paris, France.ORCID http://orcid.org/0000-0002-4814-0307
Véronique Le GuernDepartment of Internal Medicine, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris Cité, Paris, France.
Capucine Morélot-PanziniDepartment of Pneumology, département R3S, Hôpital Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Sorbonne Université, Paris, France.
Marc HumbertUniversité Paris-Saclay, INSERM Unité Mixte de Recherche en Santé 999, Hypertension Pulmonaire, Physiopathologie et Innovation Thérapeutique, Service de Pneumologie et Soins Intensifs Respiratoires, Hôpital Bicêtre (Assistance Publique-Hôpitaux de Paris), FHU André Cournand, European Reference Network for Rare Respiratory Diseases, Le Kremlin-Bicêtre, France.
Olivier SitbonUniversité Paris-Saclay, INSERM Unité Mixte de Recherche en Santé 999, Hypertension Pulmonaire, Physiopathologie et Innovation Thérapeutique, Service de Pneumologie et Soins Intensifs Respiratoires, Hôpital Bicêtre (Assistance Publique-Hôpitaux de Paris), FHU André Cournand, European Reference Network for Rare Respiratory Diseases, Le Kremlin-Bicêtre, France.
Cécile GoujardDepartment of Internal Medicine, Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris, Université Paris-Saclay, Le Kremlin-Bicêtre, France.
Brigitte Bader-MeunierDepartment of Paediatric Rheumatology and Immunology, Hôpital Necker-Enfants malades, Assistance Publique-Hôpitaux de Paris, Inserm U1163 Institut Imagine, Université Paris Cité, Paris, France.
Bruno FautrelSorbonne Université - Assistance Publique-Hôpitaux de Paris, FHU INFLAME, INSERM UMRS 1136, Department of Rheumatology, Hôpital Pitié-Salpêtrière, Paris, France.ORCID http://orcid.org/0000-0001-8845-4274
Jérome HadjadjSorbonne Université, Internal Medicine Department, Saint Antoine Hospital, Assistance Publique - Hôpitaux de Paris, Paris, France.
Pascale ChrétienImmunology Laboratory, Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris, Université Paris-Saclay, Le Kremlin-Bicêtre, France.
Pascale Roland-NicaiseImmunology Laboratory, Hôpital Bichat, Assistance Publique-Hôpitaux de Paris, Université Paris Cité, Paris, France.
Claire GoulvestreImmunology Laboratory, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris Cité, Paris, France.
Jean-Luc CharuelImmunology Department, Hôpital Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Sorbonne Université, Paris, France.
Olivier BenvenisteDepartment of Internal Medicine and Clinical Immunology, Hôpital Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Sorbonne Université, Paris, France.ORCID http://orcid.org/0000-0002-1167-5797
Luc MouthonDepartment of Internal Medicine, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris Cité, Paris, France.
Victoire De LastoursDepartment of Internal Medicine, Hôpital Beaujon, Assistance Publique-Hôpitaux de Paris, Université Paris Cité, Clichy, France.
Perrine DusserDepartment of Paediatric Rheumatology, Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris, Université Paris-Saclay, Le Kremlin-Bicêtre, France.ORCID http://orcid.org/0000-0001-8118-0848
Mohamad ZaidanDepartment of Nephrology, Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris, Université Paris-Saclay, Le Kremlin-Bicêtre, France.
Elisabeth AslangulDepartment of Internal Medicine, Hôpital Louis-Mourier, Assistance Publique-Hôpitaux de Paris, Université Paris Cité, Colombes, France.
Marie SaillourDepartment of Pneumology, Hôpital Louis-Mourier, Assistance Publique-Hôpitaux de Paris, Université Paris Cité, Colombes, France.
Glory DinguluDepartment of Paediatrics, Hôpital Robert Debré, Assistance Publique-Hôpitaux de Paris, Centre de référence Rhumatismes et Auto-immunité systémique de l'enfant (RAISE), Université Paris Cité, Paris, France.
François ChassetDepartment of Dermatology, Hôpital Tenon, Assistance Publique-Hôpitaux de Paris, Sorbonne Université, Faculté de médecine, INSERM U1135, CIMI, Paris, France.
Thach Bui-QuangDepartment of Pneumology, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris Cité, Paris, France.
Jacques CadranelDepartment of Pneumology and Thoracic Oncology; reference center for Adults Rare Pulmonary Diseases (RespiFil), Hôpital Tenon, Assistance Publique-Hôpitaux de Paris, Sorbonne Université, Paris, France.
Gaétane NocturneDepartment of Rheumatology, Université Paris Saclay, FHU CARE, INSERM UMR1184, CEA, AP-HP, Hôpital Bicêtre, Le Kremlin-Bicetre, France.ORCID http://orcid.org/0000-0001-6809-0733
Xavier MarietteDepartment of Rheumatology, Université Paris Saclay, FHU CARE, INSERM UMR1184, CEA, AP-HP, Hôpital Bicêtre, Le Kremlin-Bicetre, France.ORCID http://orcid.org/0000-0002-4244-5417
Samuel BitounDepartment of Rheumatology, Université Paris Saclay, FHU CARE, INSERM UMR1184, CEA, AP-HP, Hôpital Bicêtre, Le Kremlin-Bicetre, France.ORCID http://orcid.org/0000-0003-0891-2269
Pierre-Yves BrilletDepartment of Radiology, Hôpital Avicenne, Assistance Publique-Hôpitaux de Paris, Sorbonne Université, Bobigny, France.
Raphaèle SerorDepartment of Rheumatology, Université Paris Saclay, FHU CARE, INSERM UMR1184, CEA, AP-HP, Hôpital Bicêtre, Le Kremlin-Bicetre, France raphaele.seror@aphp.fr.ORCID http://orcid.org/0000-0002-5523-1856

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

objectiveAnti-Ku antibodies (Abs) are rare and detected in various autoimmune diseases (AIDs), presenting in various phenotypes, potentially affecting different organs including the lungs. This study aimed to describe the characteristics and evolution of interstitial lung disease (ILD) in anti-Ku-positive patients.

methodsAn observational, multicentre, retrospective study was conducted across 10 French University Hospitals between January 2010 and June 2025, including patients with anti-Ku Abs with ILD. Clinical data and all pulmonary function tests and chest CT scans available were reviewed for this study. ILD progression was defined using criteria inspired by the American Thoracic Society/European Respiratory Society/Japenese Respiratory Society/Asociación Latinoamericana de Tórax Clinical Practice Guidelines, applied across the entire follow-up period rather than within the 12-month timeframe, to capture all clinically meaningful progression events. To account for event timing, time to ILD progression was analysed as a time-to-event outcome using Cox proportional hazards models.

resultsAmong 154 anti-Ku-positive patients (51 with idiopathic inflammatory myopathy, 46 with systemic lupus erythematosus, 30 with Sjögren's disease, 27 with systemic sclerosis), 60 (39%) had ILD (68% women, median age 56 years). The predominant ILD pattern was fibrotic non-specific interstitial pneumonia (27%, n=16). ILD was already present at AID diagnosis in 48 patients (80%). Forty-five (75%) patients progressed after a median time of 4 (2-11) years. Male sex (adjusted HR (aHR) 2.7; 95% CI 1.4 to 5.2) was associated with ILD progression. When the 12-month cut-off was strictly applied, only six patients fulfilled the progressive fibrosing ILD definition. Baseline pulmonary fibrosis was present in 34 (57%) patients and was associated with a reduced survival when adjusting for age at ILD diagnosis and cardiac involvement (aHR 6.5, 95% CI 1.5 to 28.2).

conclusionILD occurred in 39% of anti-Ku-positive patients and progressed in 75% of them, underscoring the importance of systematic ILD screening and monitoring in these patients.

Indexed as

Antibodies, AntinuclearAutoantibodiesLung Diseases, InterstitialAdultAgedAutoimmune DiseasesDisease ProgressionFemaleHumansMaleMiddle AgedPrognosisRespiratory Function TestsRetrospective StudiesTomography, X-Ray ComputedAntibodies, AntinuclearAutoantibodiesAutoantibodiesAutoimmune DiseasesConnective Tissue DiseasesLung Diseases, InterstitialPulmonary Fibrosis

Identifiers

PMID42508943
PMCPMC13409090

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