Systemic Sclerosis

Systemic sclerosis (SSc) or scleroderma is a progressive autoimmune disorder characterized by changes in the texture and appearance of the skin caused by increased connective tissues buildup. The disease can affect many other parts of the body, but often only affects the skin in the early stages or limited forms of the disease. In 2013, the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR) published and enhanced the classification guidelines1 for SSc, which give more sensitivity to the early and limited cutaneous forms of the disease. Besides thickening of skin on the fingers, there are several factors linked to the (micro-) circulation that are involved in the new classification. One is the damage of the capillaries, that can be detected by nailfold video-capillaroscopy (NVC) and that improved the specificity and sensitivity of the 2013 EULAR/ACR SSc classification criteria.

In recent years, there have been several studies indicating that laser speckle contrast analysis (LASCA) can be a valuable technique for further SSc research and as a diagnostic tool in the classification of the disorder.2,7
Several studies also show the technique to be useful for assessing and monitoring the effect of treatment.10,12 One way to study the effect of locally administered drugs is by using iontophoresis in combination with LASCA.11
Recommended products: PSI NR and Perilont.

Perilont micropharmacology system

Iontophoresis is a technique to transport charged molecules or drugs across a tissue barrier. Combined with LASCA, iontophoresis is a valuable tool for studying the effect of locally administered drugs on systemic sclerosis patients.

Iontophoresis

Case example (University of Genova - Italy, University of Ghent - Belgium)

Maurizio Cutolo (University of Genova - Italy), Barbara Ruaro (University of Genova - Italy), Vanessa Smith (University of Ghent - Belgium)


LASCA analyses the peripheral blood perfusion and gives a value of its intensity. Our recent study performed using LASCA showed significantly lower blood perfusion in SSc patients than in healthy subjects at the level of the fingertips, periungual areas, and palms of the hands, but not at face level2,8

In addition, a statistically significant negative correlation between the extent of nailfold microangiopathy and degree of blood perfusion was detected at the level of those skin areas mentioned above2,8 (see figure 1, 2). Furthermore, we have demonstrated in SSc patients that LASCA is a safe technique that monitors the perfusion of digital ulcers, treated by local medical dressing during the healing process.

We have used LASCA to evaluate the change in blood perfusion during long term treatment with various drugs such as Bosentan, Iloprost and Aminapthone9,10.
In conclusion LASCA represents an essential safe, non-invasive, and non-contact tool for a reliable quantification of blood perfusion in SSc skin and ulcer areas. Along with NVC it may be useful to monitor disease progression, response to treatment, and ulcer outcome.


Figure 1. LASCA compared with NVC in SSc patients.
Image modified from: Ruaro B., .. , Cutolo M. Laser speckle contrast analysis: a new method to evaluate peripheral blood perfusion in systemic sclerosis patients. Ann Rheum Dis. 2014;73:1181-5.

Figure 2. LASCA evaluation in a SSc patient

Expert opinion

Perimed’s PeriCam PSI laser speckle contrast imager (LASCA) is an excellent tool to study the function of microcirculation, in particular the peripheral blood perfusion and Raynaud’s phenomenon in Systemic Sclerosis at any time of the disease 2. The effects of therapies on microcirculation in SSc patients represent also an important application of LASCA. The LASCA analysis of the microcirculation (image below) is a SSc patient before and after 6 days of iv vasodilation with prostacyclin.

Maurizio Cutolo (University of Genova – Italy)

Barbara Ruaro (University of Genova – Italy)

Vanessa Smith (University of Ghent – Belgium)

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See references

References

  • van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, et al. 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative. Ann Rheum Dis. 2013;72:1747-55.    
  • Ruaro B, Sulli A, Smith V, Pizzorni C, Gallo M, Cutolo M. speckle contrast analysis: a new method to evaluate peripheral blood perfusion in systemic sclerosis patients. Ann Rheum Dis. 2014; 73: 1181-5.    
  • Cutolo M, Vanhaecke A, Ruaro B, Deschepper E, Ickinger C, Melsens K, et al. Is laser speckle contrast analysis (LASCA) the new kid on the block in systemic sclerosis? A systematic literature review and pilot study to evaluate reliability of LASCA to measure peripheral blood perfusion in scleroderma patients. Autoimmun Rev. 2018 Jun 6. pii: S1568-9972(18)30134-4. doi: 10.1016/j.autrev.2018.01.023. [Epub ahead of print]    
  • Lambrecht V, Cutolo M, De Keyser F, Decuman S, Ruaro B, Sulli A, et al. Reliability of the quantitative assessment of peripheral blood perfusion by laser speckle contrast analysis in a systemic sclerosis cohort. Ann Rheum Dis. 2016;75:1263-4.    
  • Cutolo M, Ruaro B, Smith V. Macrocirculation versus microcirculation and digital ulcers in systemic sclerosis patients. Rheumatology (Oxford). 2017;56:1834-1836.    
  • Cutolo M, Sulli A, Smith V. Assessing microvascular changes in systemic sclerosis diagnosis and management. Nat Rev Rheumatol. 2010;6:578-87.    
  • Ruaro B, Sulli A, Smith V, Paolino S, Pizzorni C, Cutolo M. Short-term follow-up of digital ulcers by laser speckle contrast analysis in systemic sclerosis patients. Microvasc Res. 2015;101:82-5.    
  • Ruaro B, Sulli A, Pizzorni C, Paolino S, Smith V, Cutolo M. Correlations between skin blood perfusion values and nailfold capillaroscopy scores in systemic sclerosis patients. Microvasc Res. 2016;105:119-24.    
  • Trombetta AC, Pizzorni C, Ruaro B, Paolino S, Sulli A, Smith V, Cutolo M. Effects of Longterm Treatment with Bosentan and Iloprost on Nailfold Absolute Capillary Number, Fingertip Blood Perfusion, and Clinical Status in Systemic Sclerosis. J Rheumatol. 2016;43:2033-2041.    
  • Ruaro B, Paolino S, Pizzorni C, Cutolo M, Sulli A. Assessment of treatment effects on digital ulcer and blood perfusion by laser speckle contrast analysis in a patient affected by systemic sclerosis. Reumatismo. 2017;69:134-136.    
  • M Roustit, F Gaillard-Bigot, S Blaise, F Stanke-Labesque, C Cracowski, C Seinturier, J-F Jourdil, B Imbert, PH Carpentier and J-L Cracowski, Cutaneous Iontophoresis of Treprostinil in Systemic Sclerosis: A Proof-of-Concept Study, Clin Pharmacol Ther. 2014 Apr;95(4):439-45.    
  • Bo Chen; Xiaojun Wang; Xiao Long; Mingzi Zhang; Jiuzuo Huang; Nanze Yu; Jing Xu, Supportive Use of Adipose-Derived Stem Cells in Cell-Assisted Lipotransfer for Localized Scleroderma, Plastic and Reconstructive Surgery. 141(6):1395–1407    
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