Systemic Sclerosis

  • Julia Spierings
  • Femke C. van Rhijn-Brouwer
  • Jacob M. van LaarEmail author


Systemic sclerosis (SSc) is a rare systemic autoimmune connective tissue disease with clinical and laboratory features of inflammation, vasculopathy, and fibrosis of the skin and visceral organs. The role of the microbiota is an area that is largely unexplored in SSc. An overview is given of the studies that investigated the composition of the microbiome in the gastrointestinal tract and the skin. Furthermore, hypotheses that may strengthen the rationale for further investigation of the microbiome in SSc are discussed. There is tentative evidence that alterations in the microbiome can be associated with SSc. However, more research is needed to establish if these altered populations are the cause or the result of the disease. The fact that SSc affects the skin, lungs, and gastrointestinal tract, which are prominent locations of the microbiome, makes it both more challenging and interesting to explore the role of microorganisms in the disease process.


Microbiome Systemic sclerosis Borrelia Faecalibacterium prausnitzii 


B. burgdorferi

Borrelia burgdorferi




Diffuse cutaneous systemic sclerosis


Endothelial cells

F. prausnitzii

Faecalibacterium prausnitzii


Graft versus host disease


Hepatitis B virus


Limited cutaneous systemic sclerosis


Small intestinal bacterial overgrowth


Systemic sclerosis


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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Julia Spierings
    • 1
  • Femke C. van Rhijn-Brouwer
    • 1
    • 2
  • Jacob M. van Laar
    • 1
    Email author
  1. 1.Department of Rheumatology and Clinical ImmunologyUniversity Medical Centre UtrechtUtrechtThe Netherlands
  2. 2.Department of Nephrology and HypertensionUniversity Medical Centre UtrechtUtrechtThe Netherlands

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