Systemic sclerosis

Systemic sclerosis, also termed scleroderma, is a multisystem connective tissue disease involving autoimmunity, inflammation, fibrosis and vasculopathy. The clinical presentation of systemic sclerosis is characterized by skin thickening, Raynaud's phenomenon, vasculopathy-induced tissue death and fibrosis of internal organs.

Latest Research and Reviews

  • Research |

    The transcription factor PU.1 is an essential regulator of the pro-fibrotic gene expression program in fibroblasts; PU.1 expression is upregulated in various fibrotic diseases, whereas inactivation of PU.1 induces regression of fibrosis in a number of organs.

    • Thomas Wohlfahrt
    • , Simon Rauber
    • , Steffen Uebe
    • , Markus Luber
    • , Alina Soare
    • , Arif Ekici
    • , Stefanie Weber
    • , Alexandru-Emil Matei
    • , Chih-Wei Chen
    • , Christiane Maier
    • , Emmanuel Karouzakis
    • , Hans P. Kiener
    • , Elena Pachera
    • , Clara Dees
    • , Christian Beyer
    • , Christoph Daniel
    • , Kolja Gelse
    • , Andreas E. Kremer
    • , Elisabeth Naschberger
    • , Michael Stürzl
    • , Falk Butter
    • , Michael Sticherling
    • , Susetta Finotto
    • , Alexander Kreuter
    • , Mark H. Kaplan
    • , Astrid Jüngel
    • , Steffen Gay
    • , Stephen L. Nutt
    • , David W. Boykin
    • , Gregory M. K. Poon
    • , Oliver Distler
    • , Georg Schett
    • , Jörg H. W. Distler
    •  & Andreas Ramming
    Nature 566, 344-349
  • Reviews |

    Our ability to interrogate the genetic and epigenetic processes that underpin disease are advancing rapidly. In this Review, Radstake and colleagues highlight insights gained into the pathogenesis of systemic sclerosis from the past 4 years of genetic and epigenetic research.

    • Chiara Angiolilli
    • , Wioleta Marut
    • , Maarten van der Kroef
    • , Eleni Chouri
    • , Kris A. Reedquist
    •  & Timothy R. D. J. Radstake
  • Reviews |

    The two major lung complications in systemic sclerosis, lung fibrosis and pulmonary arterial hypertension, share some pathogenic mechanisms. Strategies for managing patients with these complications have greatly advanced in the past decade, and many tools and treatments are now available.

    • Christopher P. Denton
    • , Athol U. Wells
    •  & John G. Coghlan

News and Comment