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Improvements in digital vasculature observed using micro magnetic resonance angiography after high-dose immunosuppression for severe systemic sclerosis

Bone Marrow Transplantation volume 44, pages 387–389 (2009)Cite this article

Peripheral vasculopathy is a characteristic feature of systemic sclerosis (SSc).1 It is frequently associated with early onset of Raynaud's phenomenon and can cause digital ischemia.2, 3 A recent report of two cases4 showed the potential reversibility of SSc-related micro-vascular changes at 1 or 5 month after high-dose immunosuppressive therapy (HDIT) and autologous hematopoietic cell transplantation (HCT). The effect of HDIT and autologous HCT on the SSc-related vascular changes of the digital arteries, however, has not been reported. A micro magnetic resonance angiography (MRA) technique was recently developed for the non-invasive visualization and assessment of the digital arteries and veins in vivo.5 In this case report, we present quantitative observations of the digital vasculature status using micro MRA before and after HDIT and autologous HCT.

The subject was a 49-year-old Caucasian male, with diffuse cutaneous SSc of 8 months’ duration. At the time of diagnosis, the patient presented with Raynaud's syndrome, scleroderma, interstitial lung disease with ground-glass appearance on high-resolution computed tomography of the chest, numbness and pain in both hands, joint contractures in fingers, wrists and elbows, gastroesophageal reflux disease and telangiectasias. No digital ulcers or calcinosis were found. The modified Rodnan skin score was 28. The patient consented to participate in the SCOT trial (Scleroderma: CY or Transplantation) and was randomized to HDIT and autologous HCT.6 HDIT consisted of high-dose CY (120 mg/kg), fractionated TBI (800 cGy with lung and kidney shielding) and equine anti-thymocyte globulin (90 mg/kg) followed by transplantation of CD34 selected autologous hematopoietic progenitor cells (⩾2.5 × 106 CD34 cells/kg).

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Acknowledgements

This work was supported by awards N01-AI-05419 from the National Institute of Allergy and Infectious Diseases and research funding from Scleroderma Research Foundation. The authors thank Zach Miller for his help in editing the paper.

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Authors and Affiliations

  1. Department of Bioengineering, University of Washington, Seattle, WA, USA

    J Wang

  2. Department of Radiology, University of Washington, Seattle, WA, USA

    J Wang, B Chu, V L Yarnykh & C Yuan

  3. Fred Hutchinson Cancer Research Center, Seattle, WA, USA

    R A Nash

  4. Department of Pathology, University of Washington, Seattle, WA, USA

    S M Schwartz

  5. Division of Cellular Therapy and Transplantation, Duke University Medical Center, Durham, NC, USA

    K M Sullivan

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  1. J Wang
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  2. R A Nash
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  3. B Chu
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  7. C Yuan
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Correspondence to J Wang.

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Wang, J., Nash, R., Chu, B. et al. Improvements in digital vasculature observed using micro magnetic resonance angiography after high-dose immunosuppression for severe systemic sclerosis. Bone Marrow Transplant 44, 387–389 (2009). https://doi.org/10.1038/bmt.2009.35

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