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Scleroderma renal crisis: a review for emergency physicians

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Abstract

Scleroderma renal crisis (SRC) remains a high-risk clinical presentation, and many patients require emergency department (ED) management for complications and stabilization. This narrative review provides an evidence-based summary of the current data for the emergency medicine evaluation and management of SRC. While SRC remains a rare clinical presentation, surveillance data suggest an overall incidence between 4 and 6% of patients with scleroderma. The diagnostic criteria for SRC include a new onset blood pressure > 150/85 mm Hg OR increase ≥ 20 mm Hg from baseline systolic blood pressure, along with a decline in renal function, defined as an increase serum creatinine of ≥ 10% and supportive features. There are many risk factors for SRC, including diffuse and rapidly progressive skin thickening, palpable tendon friction rubs, and new anemia or cardiac events. Critical patients should be evaluated in the resuscitation bay, and consultation with the nephrology team for appropriate patients improves patient outcomes.

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Fig. 1
Fig. 2

Adapted from Lynch BM, Stern EP, Ong V, Harber M, Burns A, Denton CP. UK Scleroderma Study Group (UKSSG) guidelines on the diagnosis and management of scleroderma renal crisis. Clin Exp Rheumatol. 2016;34 Suppl 100(5):106–109

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Acknowledgements

TM, AK, and BL conceived the idea for this manuscript, obtained permission for submission from Dr. Adams, and contributed substantially to the writing and editing of the review. This manuscript did not utilize any grants or funding, and it has not been presented in abstract form. This clinical review has not been published, it is not under consideration for publication elsewhere, its publication is approved by all authors and tacitly or explicitly by the responsible authorities where the work was carried out, and that, if accepted, it will not be published elsewhere in the same form, in English or in any other language, including electronically without the written consent of the copyright-holder. The view(s) expressed herein are those of the author(s) and do not reflect the official policy or position of Brooke Army Medical Center, the US Army Medical Department, the U.S. Army Office of the Surgeon General, the Department of the Army, the Department of the Air Force and Department of Defense or the US Government.

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  1. Department of Emergency Medicine, Jackson Memorial Hospital/Miller School of Medicine, University of Miami, 1611 N.W. 12th Avenue, Miami, FL, 33136, USA

    Tim Montrief

  2. Department of Emergency Medicine, The University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, TX, 75390, USA

    Alex Koyfman

  3. Department of Emergency Medicine, Brooke Army Medical Center, 3841 Roger Brooke Dr, Fort Sam Houston, TX, 78234, USA

    Brit Long

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Montrief, T., Koyfman, A. & Long, B. Scleroderma renal crisis: a review for emergency physicians. Intern Emerg Med 14, 561–570 (2019). https://doi.org/10.1007/s11739-019-02096-2

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