Hematopoietic stem cell transplantation (HSCT) and cyclophosphamide (CYC) are treatment options for a progressive interstitial lung disease associated with systemic sclerosis (SSc‐ILD). This retrospective observational study aimed to evaluate:
1) The evolution of SSc‐ILD assessed by high‐resolution computed tomography (HRCT) in SSc patients treated with HSCT. A group of patients treated with CYC was included as a reference frame;
2) How pulmonary function tests (PFTs) associate with HRCT findings;
3) Which factors predict ILD reduction.
Fifty-one patients (HSCT n=20; CYC n=31) were included. Mean change in total ILD score was ‐5.1% (95%CI ‐10.2 to 0.0) in HSCT (p=0.050), and ‐1.0% (95%CI ‐4.3 to 2.3) in CYC group (p=0.535). For all patients, the evolution of HRCT scores was weakly associated with relative changes in PFTs. In univariate logistic regression, higher GGO and total ILD scores and lower diffusing capacity of the lungs for carbon monoxide at baseline (DLCO) predicted the ILD extent after multivariable treatment model could not be built to assess the independence of predictors.
Conclusively, one year after treatment with HSCT, a non‐significant but clear reduction of SSc‐ILD extent was observed. Changes in PFTs were associated with HRCT scores changes, but the correlation was weak and cannot be considered conclusive.
Ref: https://onlinelibrary.wiley.com/doi/10.1002/acr.24451
