Background Interstitial lung disease (ILD) is a major source of mortality in systemic sclerosis (SSc). Autologous hematopoietic stem cell transplantation (HSCT) is employed as a new therapeutic strategy in patients with severe/progressive form of SSc and its effectiveness for SSc-related ILD was reported (1, 2).
Objectives To evaluate effect of HSCT for SSc-related ILD.
Methods A total of 77 SSc patients were screened and evaluated for ILD, using high-resolution computed tomography (HRCT) and pulmonary function tests. Ten patients treated with HSCT and 30 patients treated with conventional therapy were evaluated retrospectively. In both groups, disease durations of the patients were less than 3 years at baseline. HSCT was performed as previously reported (3). Image findings were quantified according to HRCT score as reported (4). We evaluated ΔHRCT score from the baseline to 2 years’ time-point, and compared those between HSCT group and conventional group.
Results Baseline demographics such as sex, age, disease duration, the observation period, smoking history, the phenotype of SSc (limited or diffuse), the prevalence of ILD, autoantibodies, serum KL-6, respiratory function and HRCT findings including HRCT score, were not significantly different between two groups. HSCT group revealed significantly more improved ΔHRCT score than Conventional group at the observation periods (-0.1±2.8 vs. 1.3±1.8; p=0.018). No one died due to progression of SSc or transplantation in HSCT group, whereas three patients died due to exacerbation of lung disease or malignancy in Conventional group.
Conclusions HSCT therapy improved the HRCT findings in SSc patients with ILD.
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Burt RK, et al. Autologous non-myeloablative haemopoietic stem-cell transplantation compared with pulse cyclophosphamide once per month for systemic sclerosis (ASSIST): an open-label, randomised phase 2 trial. Lancet. 2011; 378:498-506
Bohgaki T et al., Immunological reconstitution after autologous hematopoietic stem cell transplantation in patients with systemic sclerosis: relationship between clinical benefits and intensity of immunosuppression. J Rheumatol. 2009;36:1240-8.
Goldin JG, et al. High-resolution CT scan findings in patients with symptomatic scleroderma-related interstitial lung disease. Chest. 2008; 134:358-67.
Disclosure of Interest None Declared