Systemic sclerosis – dermatological aspects. Part 1: Pathogenesis, epidemiology, clinical findings

@article{Sticherling2012SystemicS,
  title={Systemic sclerosis – dermatological aspects. Part 1: Pathogenesis, epidemiology, clinical findings},
  author={Michael Sticherling},
  journal={JDDG: Journal der Deutschen Dermatologischen Gesellschaft},
  year={2012},
  volume={10}
}
  • M. Sticherling
  • Published 1 October 2012
  • Medicine
  • JDDG: Journal der Deutschen Dermatologischen Gesellschaft
Systemic sclerosis is a chronic inflammatory multiorgan disease belonging to the group of collagen‐vascular disorders. With a prevalence of 10/100,000 inhabitants it may be regarded a rather rare disease. Its etiology and pathogenesis have still not been elucidated in detail, especially with regard to the differential involvement of skin and the cause of the clinically heterogeneous disease courses. Various components of the vasculature, connective tissue as well as the immune system are… 

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References

SHOWING 1-10 OF 51 REFERENCES

The pathogenesis of systemic sclerosis.

TLDR
Potential mechanisms underlying the vascular, autoimmune, and fibrotic points of dysregulation in systemic sclerosis are discussed.

Autoimmunity in scleroderma: the origin, pathogenetic role, and clinical significance of autoantibodies

TLDR
Improvements in multiplex autoantibody assays will aid in the diagnosis and prognosis of the complications associated with systemic sclerosis and yield critical insights into the pathogenesis of, and suggest novel therapeutic targets for, this chronic, debilitating disease.

Systemic sclerosis - a systematic overview: part 1 - disease characteristics and classification, pathophysiologic concepts, and recommendations for diagnosis and surveillance.

TLDR
A systematic overview of the classification concepts of scleroderma disorders, the epidemiologic and genetic burden, the complex pathophysiologic background, and the clinical features and the stage-dependent capillary microscopic features of SSc are summarized.

The many faces of scleroderma sine scleroderma: a literature review focusing on cardiopulmonary complications

TLDR
Overall the clinical presentation is subtle and heightened clinical awareness is required to facilitate prompt recognition and treatment of ssSSc and the range and frequency of the clinical manifestations are described.

Clinical burden of digital vasculopathy in limited and diffuse cutaneous systemic sclerosis

TLDR
Digital vasculopathy is a serious complication of SSc contributing significant morbidity and often requiring hospital-based management, often requiring treatment with intravenous prostacyclin/CGRP.

Infectious disease as aetiological factor in the pathogenesis of systemic sclerosis.

TLDR
The current state of knowledge of infectious risk factors in systemic sclerosis and the possible mechanisms by which infectious exposures might induce pathologic processes are reviewed.

Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials And Research group database

TLDR
The clinical distinction seemed to be superseded by an antibody-based classification in predicting some scleroderma complications, and the EUSTAR MEDS database facilitates the analysis of clinical patterns in SSc, and contributes to the standardised assessment and monitoring of SSc internationally.

[Clinical aspects of progressive systemic scleroderma (PSS). Multicenter studies of 194 patients].

TLDR
Patients from five German Departments of Dermatology affected with progressive systemic scleroderma (PSS) were classified and examined, and the Raynaud symptom could be identified as being the main clinical symptom in 90% of the patients.

Mechanisms and consequences of fibrosis in systemic sclerosis

TLDR
Overall, this is an exciting time for new therapies in SSc and advances are being made in synchrony with an improved understanding of the molecular and biochemical basis of the disease.

Prognostic markers for systemic sclerosis.

  • O. Meyer
  • Biology, Medicine
    Joint, bone, spine : revue du rhumatisme
  • 2006
...