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Part of the book series: In Clinical Practice ((ICP))

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Abstract

Critical ischemia is defined as the presence of ischemic symptoms at rest. It identifies patients with high risk of imminent tissue loss. Critical ischemia in SSc results from progressive microvascular insufficiency but occasionally it may result from macrovascular compromise by emboli, thrombosis or vasculitis. Outcome of critical ischemia is contingent on the duration of ischemia, presences of comorbidities, prompt institution of appropriate workup and therapy. Timely and comprehensive therapy is key to prevent loss of threatened tissues. Therapies vary based on location of ischemia and the severity of symptoms. Primary prevention and control of modifiable risk factors remain paramount in the ongoing care of patients.

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References

  1. Abraham DJ, Varga J. Scleroderma: from cell and molecular mechanisms to disease models. Trends Immunol. 2005;26(11):587–95.

    Article  CAS  Google Scholar 

  2. Rajendran P, et al. The vascular endothelium and human diseases. Int J Biol Sci. 2013;9(10):1057–69.

    Article  CAS  Google Scholar 

  3. Kahaleh B. The microvascular endothelium in scleroderma. Rheumatology (Oxford). 2008;47(5):14–5.

    Article  Google Scholar 

  4. Distler JH, Gay S, Distler O. Angiogenesis and vasculogenesis in systemic sclerosis. Rheumatology (Oxford). 2008;47(2):234–5.

    Google Scholar 

  5. Rho YH, Choi SJ, Lee YH, Ji JD, Song GG. Scleroderma associated with ANCA associated vasculitis. Rheumatol Int. 2006;26(5):369–75.

    Article  Google Scholar 

  6. La Civita L, Jeracitano G, Ferri C, et al. Wegener’s granulomatosis of the elderly: a case report of uncommon severe gangrene of the feet. Ann Rheum Dis. 1995;54(4):328.

    Article  Google Scholar 

  7. Yannick A, Denton C, Krieg T, Ornelisse P, Rosenberg D, Schwierin B, Matucci-Cerinic M. Clinical characteristics and predictors of gangrene in patients with systemic sclerosis and digital ulcers in the digital ulcer outcome registry: a prospective, observational cohort. Ann Rheum Dis. 2016;209481

    Google Scholar 

  8. Wigley FM, Wise RA, Miller R, Needleman BW, Spence RJ. Anticentromere antibody as a predictor of digital ischemic loss in patients with systemic sclerosis. Arthritis Rheum. 1992;35:688–93.

    Article  CAS  Google Scholar 

  9. Takahashi M, Okada J, Kondo H. Six cases positive for anti-centromere antibodies with ulcer and gangrene in the extremities. Br J Rheumatol. 1997;36:889–93.

    Article  CAS  Google Scholar 

  10. Hughes M, Herrick AL. Digital ulcers in systemic sclerosis. Rheumatology. 2017;56:4–25.

    Article  Google Scholar 

  11. Hirsch AT, Haskal ZJ, Hertzer NR, et al. ACC/AHA 2005 practice guidelines for the Management of Patients with peripheral arterial disease (lower extremity, renal, mesenteric, and abdominal aortic). Circulation. 2006;113:e463–654.

    Article  Google Scholar 

  12. Antoniou GA, Chalmers N, Georgiadis GS, et al. A meta-analysis of endovascular versus surgical reconstruction of femoropopliteal arterial disease. J Vasc Surg. 2013;57:242–53.

    Article  Google Scholar 

  13. Rooke TW, Hirsch AT, Hertzer NR, et al. ACCF/AHA focused update of the guideline for the Management of Patients with peripheral artery disease (updating the 2005 guideline) a report of the American College of Cardiology Foundation/American Heart Association task force on practice guidelines. Circulation. 2011;124(18):2020–4529.

    Article  Google Scholar 

  14. Cheun T, Jayakumar L, Sheehan M, et al. Outcomes of upper extremity interventions for chronic critical ischemia. J Vasc Surg. 2019;69(1):120–8. e2

    Article  Google Scholar 

  15. Stringer T, Femia AN. Raynaud’s phenomenon: current concepts. Clin Dermatol. 2018;36(4):498–507.

    Article  Google Scholar 

  16. McMahan ZH, Wigley FM. Raynaud’s phenomenon and digital ischemia: a practical approach to risk stratification, diagnosis and management. Int J Clin Rheumtol. 2010;5(3):355–70.

    Article  Google Scholar 

  17. Ruch DS, Holden M, Smith B, Koman A. Periarterial sympathectomy in scleroderma patients: intermediate-term follow-up. J Hand Surg. 2002;27A:258–64.

    Article  Google Scholar 

  18. Tristain L, Hartzell E, Makhni C, Sampson C. Long-term results of Periarterial Sympathectomy. J Hand Surg Am. 2009;34(8):1454–60.

    Article  Google Scholar 

  19. Visalli E, Amato G, Di Gangi M, et al. Treatment with intravenous iloprost in patients with systemic sclerosis: a short review. J Rare Dis Res Treat. 2017;2(4):6–13.

    Article  Google Scholar 

  20. Fati R, Visalli E, Amato G, et al. Long-term clinical stabilization of scleroderma patients treated with a chronic and intensive IV iloprost regimen. Rheumatol Int. 2017;37:245–9.

    Article  Google Scholar 

  21. Law S, Simms RW, Farber HW. Use of intravenous Epoprostenol as a treatment for the digital vasculopathy associated with the scleroderma Spectrum of diseases [abstract]. Arthritis Rheumatol. 2016;68(suppl 10)

    Google Scholar 

  22. Wigley FM, Wise RA, Seibold JR, et al. Intravenous iloprost infusion in patients with Raynaud phenomenon secondary to systemic sclerosis. A multicenter, placebo-controlled, double-blind study. Ann Intern Med. 1994;120(3):199–206.

    Article  CAS  Google Scholar 

  23. Rodriguez-Franco K, Miranda-Diaz A, Hoyes-Restrepo J, Melendez G. Systemic scleroderma: an approach from plastic surgery. Rev Fac Med. 2018;66(2):237–45.

    Article  Google Scholar 

  24. Rirash F, Tingey PC, Harding SE, Maxwell LJ, et al. Calcium channel blockers for primary and secondary Raynaud’s phenomenon. Cochrane Database Syst Rev. 2017;(12):CD000467.

    Google Scholar 

  25. Fries R, Shariat K, Wilmowsky H, Böhm M. Sildenafil in the treatment of Raynaud’s phenomenon resistant to vasodilatory therapy. Circulation. 2005;112:2980–5.

    Article  CAS  Google Scholar 

  26. Andrigueti F, Ebbing P, Arismendi M, Kayser C. Evaluation of the effect of sildenafil on the microvascular blood flow in patients with systemic sclerosis: a randomized, double-blind, placebo- controlled study. Clin Exp Rheumatol. 2017;35:151–8.

    PubMed  Google Scholar 

  27. Kowal-Bielecka O, Fransen J, Eustar AJ, et al. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis. 2017;76:1327–39.

    Article  Google Scholar 

  28. Harding SE, Tingey PC, Pope J, et al. Prazosin for Raynaud’s phenomenon in progressive systemic sclerosis. Cochrane Database Syst Rev. 1998;(2):CD000956.

    Google Scholar 

  29. Qiu O, Chan T, Luen M, et al. Use of nitroglycerin ointment to treat primary and secondary Raynaud’s phenomenon: a systemic literature review. Rheumatol Int. 2018;38:2209–16.

    Article  CAS  Google Scholar 

  30. Chung L, Shapiro L, Fiorentino D, Baron M, et al. MQX-503, a novel formulation of nitroglycerin, improves the severity of Raynaud’s phenomenon: a randomized, controlled trial. Arthritis Rheum. 2009;60:870–7.

    Article  CAS  Google Scholar 

  31. Khanna PP, Maranian P, Gregory J, et al. The minimally important difference and patient acceptable symptom state for the Raynaud’s condition score in patients with Raynaud’s phenomenon in a large randomized controlled clinical trial. Ann Rheum Dis. 2010;69:588–91.

    Article  Google Scholar 

  32. Dowling GB, Copeman PW, Ashfield R. Raynaud’s phenomenon in scleroderma treated with hyperbaric oxygen. Proc R Soc Med. 1967;60(12):1268–9.

    CAS  PubMed  PubMed Central  Google Scholar 

  33. Sato T, Arai K, Ichioka S. Hyperbaric oxygen therapy for digital ulcers due to Raynaud’s disease. Case Reports Plast Surg Hand Surg. 2018;5(1):72–4.

    Article  Google Scholar 

  34. Schweizer D, Schweizer R, Zhang S, et al. Botulinum toxin a and B raise blood flow and increase survival of critically ischemic skin flaps. J Surg Res. 2013;184:1205–13.

    Article  CAS  Google Scholar 

  35. Berk-Krauss J, Christman MP, Franks A, Sicco KL, Liebman TN. Botulinum toxin for treatment of Raynaud phenomenon in CREST syndrome. Dermatol Online J. 2018;24(12)

    Google Scholar 

  36. Jenkins S, Neyman K, Veledar E, et al. A pilot study evaluating the efficacy of botulinum toxin a in the treatment of Raynaud phenomenon. J Am Acad Dermatol. 2013;69:834–5.

    Article  Google Scholar 

  37. Bello R, Cooney C, Melamed E, et al. The therapeutic efficacy of botulinum toxin in treating scleroderma-associated Raynaud’s phenomenon: a randomized, double-blind, placebo-controlled clinical trial. Arthritis Rheumatol. 2017;69:1661–9.

    Article  CAS  Google Scholar 

  38. Tosi S, Marchesoni A, Messina K, et al. Treatment of Raynaud’s phenomenon with captopril. Drugs Exp Clin Res. 1987;13(1):37–42.

    CAS  PubMed  Google Scholar 

  39. Coleiro B, Marshall SE, Denton CP, Howell K, Blann A, Welsh KI, et al. Treatment of Raynaud’s phenomenon with the selective serotonin reuptake inhibitor fluoxetine. Rheumatology. 2001;40(9):1038–43.

    Article  CAS  Google Scholar 

  40. Khouri C, Gailland T, Lepelley M, Roustit M, Cracowski JL. Fluoxetine and Raynaud’s phenomenon: friend or foe? Br J Clin Pharmacol. 2017;83(10):2307–9.

    Article  Google Scholar 

  41. Kuwana M, Okazaki Y, Kaburaki J. Long-term beneficial effects of statins on vascular manifestations in patients with systemic sclerosis. Mod Rheumatol. 2009;19:530–5.

    Article  CAS  Google Scholar 

  42. Abou-Raya A, Abou-Raya S, Helmii M. Statins: potentially useful in therapy of systemic sclerosis-related Raynaud’s phenomenon and digital ulcers. J Rheumatol. 2008;35:1801–8.

    CAS  PubMed  Google Scholar 

  43. Sambo P, Amico D, Giacomelli R, et al. Intravenous N-acetylcysteine for treatment of Raynaud’s phenomenon secondary to systemic sclerosis: a pilot study. J Rheumatol. 2001;28:2257–62.

    CAS  PubMed  Google Scholar 

  44. Rosato E, Borghese F, Pisarri S, Salsano F. The treatment with N-acetylcysteine of Raynaud’s phenomenon and ischemic ulcers therapy in sclerodermic patients: a prospective observational study of 50 patients. Clin Rheumatol. 2009;28:1379–84.

    Article  Google Scholar 

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Correspondence to Bashar Kahaleh .

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Huffstuter, J.G., Kahaleh, B. (2021). Critical Ischemia. In: Matucci-Cerinic, M., Denton, C.P. (eds) Practical Management of Systemic Sclerosis in Clinical Practice. In Clinical Practice. Springer, Cham. https://doi.org/10.1007/978-3-030-53736-4_8

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  • DOI: https://doi.org/10.1007/978-3-030-53736-4_8

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