Scleroderma: from pathophysiology to novel therapeutic approaches

@article{Hunzelmann2010SclerodermaFP,
  title={Scleroderma: from pathophysiology to novel therapeutic approaches},
  author={Nicolas Hunzelmann and Thomas M. Krieg},
  journal={Experimental Dermatology},
  year={2010},
  volume={19}
}
Please cite this paper as: Scleroderma: from pathophysiology to novel therapeutic approaches. Experimental Dermatology 2010; 19: 393–400. 

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References

SHOWING 1-10 OF 130 REFERENCES

Imatinib for the treatment of pulmonary arterial hypertension.

TLDR
A 61-year-old man with a rapidly progressing form of familial idiopathic pulmonary arterial hypertension is reported on, who had presented with signs of rigor five years earlier.

Sequential dermal microvascular and perivascular changes in the development of scleroderma

TLDR
Using conventional light and electron microscopy, immunohistochemistry, and labelled adenosine uptake techniques, changes in the dermal microvasculature have been related to the various clinical stages of skin disease in systemic sclerosis.

Alternate trafficking of cathepsin L in dermal fibroblasts induced by UVA radiation

TLDR
This research presents an alternative trafficking of cathepsin L in dermal fibroblasts induced by UVA radiation that differs from the one reported in this paper.

Scleroderma--clinical and pathological advances.

Comparison of patients with and without digital ulcers in systemic sclerosis: detection of possible risk factors

TLDR
This work has shown that digital ulcers are a major complication in the course of systemic sclerosis and identifying the risk factors for DU is useful for optimal management.

Molecular pathways as novel therapeutic targets in systemic sclerosis

TLDR
The identification of novel signaling pathways and mediators that are altered in systemic sclerosis and contribute to tissue damage allows their selective targeting and opens the door for novel therapeutic strategies utilizing novel compounds, or innovative ways of using already-approved drugs.

EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR)

TLDR
Evidence-based, consensus-derived recommendations for the treatment of systemic sclerosis are useful for rheumatologists to help guide treatment for patients with SSc and may also help to define directions for future clinical research in SSc.

Clinical trials in systemic sclerosis: lessons learned and outcomes

TLDR
Data from recent clinical trials and the lessons derived from retrospective observational studies, databases, and patient registries are reviewed to improve understanding of the diverse clinical course of systemic sclerosis and permit refinement of existing outcome measures for future clinical trials.

B cell depletion with rituximab in patients with diffuse cutaneous systemic sclerosis.

TLDR
Treatment with rituximab appeared to be safe and well tolerated among patients with diffuse cutaneous systemic sclerosis and the potential efficacy in other organs such as the lung could not be clearly evaluated in this small open-label trial.

EULAR Scleroderma Trials and Research group statement and recommendations on endothelial precursor cells.

TLDR
Recommendations for standardisation of future research on EPCs will help to unify EPC research and allow better comparison between the results of different studies.
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