The first edition of this volume on Progress in Heritable Soft Connective Tissue Diseases was published in 2014.  The volume proved to be a success with readers – it has been downloaded more than 90,000 times.  At least two factors seem to account for this achievement.  One is the topic itself: important and still intriguing disorders with gaps in what we know about their pathogenesis, be it Marfan symdrome, the conglomerate of Ehlers-Danlos syndrome and the expanding slew of hypermobility entities.  I suspect that the roster of  accomplished authors is the 2nd factor responsible for wide readership.  The author list of the 2nd edition is similar to the first edition. Several contributors to the 1st edition were not able to participate because of previous commitments. However, we were able to recruit other, prominent scientists and rising stars. The new edition will bring updates on disorders already discussed in the 1st edition.  Marfan and Ehlers-Danlos syndromes are the two best known and most studied heritable disorders of soft tissues, however, there are many other conditions, though less common and even rare which represent continuum in clinical presentation and thus make diagnosis more challenging.  Perhaps even more importantly, what was suggested as one important factor in the first edition is becoming a thread woven through these diseases. This thread is transforming growth factor ß and its canonical and non-canonical pathways which play havoc to organization and function of connective tissue. Several chapters touch on these last issues as well.  weiterlesen