Relationship between decrease in CCL18 plasma levels and lumbar spine bone marrow fat fraction. (A-C) Inverse relationship between the decrease in plasma CCL18 levels and increase in lumbar spine marrow fat fraction on enzyme replacement therapy. Patient A: Spearman ρ –0.952; P = .0011; patient B: Spearman ρ –0.802; P = .0218; patient C: Spearman ρ –0.815; P = .0108 (•, CCL18; □, bone marrow fat fraction). Right hand side shows changes in the bone marrow fat fraction of the lumbar spine during enzyme replacement therapy as visualized by QCSI.