Prevalence of the limited vs. extensive scleroderma-related interstitial lung disease at the time of diagnosis of SSc-ILD based on Goh et al. criteria. Systematic review and meta-analysis

Abstract

Introduction

Goh et al. proposed in 2008 a classificatory algorithm of limited or extensive SSc-ILD. The prevalence of both at the time of diagnosis of SSc-ILD is not known with exactitude.

Methods

The review was undertaken by means of MEDLINE and SCOPUS from 2008 to 2023 and using the terms: "systemic", "scleroderma" or "interstitial lung disease" [MesH]. The Newcastle-Ottawa Scale was used for the qualifying assessment for observational studies and the Jadad scale for clinical trials. The inverse variance-weighted method was performed.

Results

Twenty-seven studies were initially included in the systematic review and meta-analysis (SRMA). Of these, 17 studies had no overlapping data. They reported data from 2,149 patients, 1,369 (81.2%) were female. The mean age was 52.4 (SD 6.6) years. 45.2% of the patients had the diffuse subtype and 54.8% had the limited or sine scleroderma subtype. A total of 38.7% of the patients showed positive antitopoisomerase antibodies (ATA) and 14.2% positive anticentromere antibodies (ACA). The mean percentage of forced vital capacity (FVC) at baseline was 80.5% (SD 6.9) and of diffusing capacity of the lungs for carbon monoxide (DLco) was 59.1% (SD 9.6). Twelve studies presented SSc-ILD extension data adjusted for PFTs and were included in the meta-analysis. The 10 observational cohort studies were analyzed separately. The overall percentage of limited extension was estimated at 63.5% (95%CI 55.3–73; p < 0.001) using the random-effects model. Heterogeneity between studies (I²) was 9.8% (95%CI 0–68.2%) with the random-effects model. Extensive pulmonary involvement was estimated at 34.3% (95%CI 26–45.4; p < 0.001). Heterogeneity between studies (I²) was 0% (95%CI 0–61.6%) with the random-effects model.

Conclusion

The overall percentage of limited SSc-ILD at the time of diagnosis of SSc-ILD was estimated at 63.5% and extensive at 34.3%.