No active MMPs in BOS patients, only MMP‐7 activity was detected in stable LTR. However, TIMP‐1‐bound MMP‐7, ‐8, and ‐9 and TIMP‐2‐bound MMP‐8 and ‐9 were increased in BOS, suggesting earlier activity of these MMPs. Here, TIMP2 is linked to Buschke-Ollendorff syndrome.