Abstract Introduction Interstitial lung disease (ILD) is a known complication of systemic sclerosis (SSc) and a significant cause of morbidity and mortality. Treatment regimens generally include immunosuppression with or without additional antifibrotic therapy. Autologous hematopoietic stem cell transplant (HSCT) is a treatment option reserved for systemic sclerosis refractory to multiple lines of traditional immunosuppression. The long-term effect of HSCT on systemic sclerosis-associated ILD (SSc-ILD) is not well described. We report a patient with SSc-ILD and ten years of pulmonary follow up following allogenic HSCT (alloHSCT). Case Report The patient was diagnosed with limited cutaneous SSc in 2002. Her disease manifestations included ILD, skin thickening/tightening, Raynaud’s, and esophageal dysmotility. She was treated with oral cyclophosphamide until 2012 when she was switched to mycophenolate. In late 2015 she was diagnosed with treatment-related myelodysplastic syndrome (MDS). She underwent related-donor allogenic-HSCT in early 2016 with reduced-intensity conditioning with fludarabine/melphalan. Tacrolimus and methotrexate were used for GVHD prophylaxis and stopped by day +120. Post-transplant her %FVC has been relatively stable and ranged from 97% to 82%. Her %DLCO has ranged from 53% to 37% and shows mild decline since 2016. Her imaging demonstrates fibrotic ILD in a probable UIP pattern, slowly progressive between 2014 and 2025. She has been maintained primarily on low dose prednisone (10 MG daily) with intermittent prednisone bursts given in the setting of respiratory viruses and subjective dyspnea. She re-trialed mycophenolate for dyspnea in 2021 but did not receive any benefit from it and so it was discontinued after several months. Regarding other residual manifestations of her SSc, none have required treatment though she continues to have severe esophageal dysmotility, which was present prior to alloHSCT. A 2022 evaluation via esophageal manometry and pH/impedance monitoring revealed aperistalsis with poor control of distal esophageal acid despite excellent control of gastric acid, consistent with persistent severe esophageal disease secondary to SSc. She has not had GVHD. Discussion We present a case of SSc-ILD who underwent alloHSCT for MDS with ten years of pulmonary follow up. Despite stability in disease activity post-alloHSCT with minimal therapy, her imaging and PFTs demonstrate mild fibrotic progression over the follow up period. This suggests that while alloHSCT can induce remission in SSc-related disease activity, esophageal dysmotility can persist and may contribute to the development of pulmonary fibrosis unlikely to be ameliorated with further immunosuppression. Antifibrotic therapy and aggressive esophageal care should be considered. This abstract is funded by: None
Blake et al. (Fri,) studied this question.