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Introduction Standard therapy of Systemic Sclerosis (SSc)-associated interstitial lung disease (ILD) at best decelerates progression. In 2022, we administered, for the first time in a non-cancer patient, third-generation (3rdGen) CD19.CAR-T cells to a patient with rapidly progressive Scl70+ SSc-ILD and fatal prognosis, in addition to nintedanib. 1–3 Here we show current follow-up data.Material and Methods 3rdGen CD19.CAR-T cells and B cell depletion persisted over more than 24 months.Results Stable serological remission and major improvement of SSc-ILD were achieved. The forced vital capacity increased by +38% of baseline. Scoring of CT scans revealed that the areas with ground glass opacities were reduced by -72 % and areas of fibrosis by -55 % of baseline (after 24months). In FAPI-PET/CT, the areas of tracer uptake were reduced by -58 % of baseline, as determined by a three-dimensional segmentation and computerized volumetric technique. Of note, fibrotic lesions in CT and areas of activated fibroblasts in FAPI-PET/CT further regressed in the second year of treatment, i.e. after prolonged deep B cell depletion and the achievement of serologic remission.Conclusions The case suggests serological remission and improvement of pulmonary fibrosis as new goals in the treatment of SSc.References Merkt W, et al. Third-generation CD19.CAR-T cell-containing combination therapy in Scl70+ systemic sclerosis. Ann Rheum Dis. 2024;83:543–6.Merkt W, Lorenz HM, Schmitt M. CAR T-Cell Therapy in Autoimmune Disease. N Engl J Med. 2024;390:1628–9Merkt W, et al. Persisting CD19.CAR-T cells in combination with nintedanib: clinical response in a patient with systemic sclerosis-associated pulmonary fibrosis after 2 years. Lancet Respir Med. 2025;13(7):651-4.Abstract P.256 Figure 1