Inflammatory myopathies

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

RMD OpenPublished Retrospective cohort study

Authors' conclusion

ILD occurred in 39% of anti-Ku-positive patients and progressed in 75% of them

Study context

Population
Anti-Ku antibody-positive patients with interstitial lung disease (ILD), 10 French University Hospitals, January 2010 to June 2025of 154 anti-Ku-positive patients
Participants
60 patients
Outcome
ILD progression (criteria inspired by ATS/ERS/JRS/ALAT guidelines, applied across entire follow-up)
Methodological detailsobservational, multicentre, 10 French University Hospitals, Cox proportional hazards models
  • Observational
  • Multicentre
  • 10 French University Hospitals
  • Cox proportional hazards models

Main finding

As reported in the abstract

ILD progression

ILD in anti-Ku-positive patients

60

39%

Patients progressed

45

75%

Median time to progression, years

4

2–11

Male sex associated with ILD progression, aHR

2.7 (1.4 to 5.2) (95% CI)

Observational data: associations, not effects.

Further findings

Predominant ILD pattern: fibrotic non-specific interstitial pneumonia

Fibrotic NSIP
27%, n=16

ILD present at AID diagnosis

ILD at AID diagnosis
48 (80%)

Progressive fibrosing ILD with strict 12-month cut-off

Patients fulfilling definition
6

Baseline pulmonary fibrosis and survival

Baseline pulmonary fibrosis
34 (57%)
Reduced survival with baseline fibrosis, aHR
6.5 (1.5 to 28.2) (95% CI)
  • Adjusted for age at ILD diagnosis and cardiac involvement

Original publication

Characteristics and outcomes of anti-Ku antibody–associated interstitial lung disease: a retrospective study of 60 patients. RMD Open. Published 27 July 2026. doi:10.1136/rmdopen-2026-006943

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