Efficacy and safety of abatacept for interstitial lung disease associated with antisynthetase syndrome: a case series

Clin Exp Rheumatol. 2024 Feb;42(2):377-385. doi: 10.55563/clinexprheumatol/53puzu. Epub 2023 Dec 5.

Abstract

Objectives: This study investigated the efficacy and safety of abatacept (ABA) in interstitial lung disease (ILD) associated with antisynthetase syndrome (ASS).

Methods: Eight patients were identified through retrospective analysis of the medical records of our centre. All patients fulfilled the Solomon criteria and had a disease complicated with ILD. Lung function, imaging, serum markers, clinical evaluation indicators of ILD, peripheral blood cell classification, cytokines, and prednisone doses were analysed.

Results: Seven of the eight patients were female. The mean age was 54.4 (standard deviation [SD] 6.0) years. Antibodies against Jo-1, PL-12, and PL-7 were present in three, three, and two patients respectively. At baseline, the mean diffusing lung capacity for carbon monoxide (DLCO) was 53.8% (SD 9.2%), the mean score of King's Brief Interstitial Lung Disease (KBILD) was 40.6 (SD 13.8), the median Krebs Von den Lungen-6 (KL-6) was 1612.5 (interquartile range [IQR] 1180.5-2431.5) U/ml. All patients experienced symptom alleviation after ABA therapy. The mean and median changes in DLCO percentage, KBILD, and KL-6 were 12.3% (p<0.05), 21.4 (p<0.01), and 174.5U/ml (p<0.01), respectively. No obvious adverse events related to ABA were observed during the treatment.

Conclusions: Our study offers preliminary, but encouraging, clinical evidence in favour of ABA as a therapy for ASS-ILD. ABA demonstrated favourable effects on ILD and was well-tolerated. Well-designed randomised controlled studies are required to confirm the efficacy and safety of this strategy.

MeSH terms

  • Abatacept / adverse effects
  • Female
  • Humans
  • Lung
  • Lung Diseases, Interstitial* / complications
  • Lung Diseases, Interstitial* / etiology
  • Male
  • Middle Aged
  • Myositis* / complications
  • Myositis* / diagnosis
  • Myositis* / drug therapy
  • Retrospective Studies

Substances

  • Abatacept

Supplementary concepts

  • Antisynthetase syndrome