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Research ArticleArticle

Risk Factors for Future Scleroderma Renal Crisis at Systemic Sclerosis Diagnosis

Sarah M. Gordon, Rodger S. Stitt, Robert Nee, Wayne T. Bailey, Dustin J. Little, Kendral R. Knight, James B. Hughes, Jess D. Edison and Stephen W. Olson
The Journal of Rheumatology July 2018, jrheum.171186; DOI: https://doi.org/10.3899/jrheum.171186
Sarah M. Gordon
From the Nephrology Department, and the Rheumatology Department, Walter Reed National Military Medical Center; the Uniformed Services University of the Health Sciences, Bethesda, Maryland, USA. The views expressed in this article are those of the authors and do not reflect the official policy of the US Department of Army/Navy/Air Force, the Department of Defense, or the US government. S.W. Olson, MD, Nephrology Department, Walter Reed National Military Medical Center; S.M. Gordon, MD, Nephrology Department, Walter Reed National Military Medical Center; R. Nee, MD, Nephrology Department, Walter Reed National Military Medical Center; R.S. Stitt, MD, Rheumatology Department, Walter Reed National Military Medical Center; W.T. Bailey, MD, Rheumatology Department, Walter Reed National Military Medical Center; D.J. Little, MD, Nephrology Department, Walter Reed National Military Medical Center; K.R. Knight, MD, Nephrology Department, Walter Reed National Military Medical Center; J.B. Hughes, Medical Student, Uniformed Services University of the Health Sciences; J.D. Edison, MD, Rheumatology Department, Walter Reed National Military Medical Center. Address correspondence to Dr. S.W. Olson, Walter Reed National Military Medical Center, Nephrology, 8901 Rockville Pike, Bethesda, Maryland 20889, USA. E-mail: stephen.w.olson.mil@mail.mil. Accepted for publication April 13, 2018.
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Rodger S. Stitt
From the Nephrology Department, and the Rheumatology Department, Walter Reed National Military Medical Center; the Uniformed Services University of the Health Sciences, Bethesda, Maryland, USA. The views expressed in this article are those of the authors and do not reflect the official policy of the US Department of Army/Navy/Air Force, the Department of Defense, or the US government. S.W. Olson, MD, Nephrology Department, Walter Reed National Military Medical Center; S.M. Gordon, MD, Nephrology Department, Walter Reed National Military Medical Center; R. Nee, MD, Nephrology Department, Walter Reed National Military Medical Center; R.S. Stitt, MD, Rheumatology Department, Walter Reed National Military Medical Center; W.T. Bailey, MD, Rheumatology Department, Walter Reed National Military Medical Center; D.J. Little, MD, Nephrology Department, Walter Reed National Military Medical Center; K.R. Knight, MD, Nephrology Department, Walter Reed National Military Medical Center; J.B. Hughes, Medical Student, Uniformed Services University of the Health Sciences; J.D. Edison, MD, Rheumatology Department, Walter Reed National Military Medical Center. Address correspondence to Dr. S.W. Olson, Walter Reed National Military Medical Center, Nephrology, 8901 Rockville Pike, Bethesda, Maryland 20889, USA. E-mail: stephen.w.olson.mil@mail.mil. Accepted for publication April 13, 2018.
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Robert Nee
From the Nephrology Department, and the Rheumatology Department, Walter Reed National Military Medical Center; the Uniformed Services University of the Health Sciences, Bethesda, Maryland, USA. The views expressed in this article are those of the authors and do not reflect the official policy of the US Department of Army/Navy/Air Force, the Department of Defense, or the US government. S.W. Olson, MD, Nephrology Department, Walter Reed National Military Medical Center; S.M. Gordon, MD, Nephrology Department, Walter Reed National Military Medical Center; R. Nee, MD, Nephrology Department, Walter Reed National Military Medical Center; R.S. Stitt, MD, Rheumatology Department, Walter Reed National Military Medical Center; W.T. Bailey, MD, Rheumatology Department, Walter Reed National Military Medical Center; D.J. Little, MD, Nephrology Department, Walter Reed National Military Medical Center; K.R. Knight, MD, Nephrology Department, Walter Reed National Military Medical Center; J.B. Hughes, Medical Student, Uniformed Services University of the Health Sciences; J.D. Edison, MD, Rheumatology Department, Walter Reed National Military Medical Center. Address correspondence to Dr. S.W. Olson, Walter Reed National Military Medical Center, Nephrology, 8901 Rockville Pike, Bethesda, Maryland 20889, USA. E-mail: stephen.w.olson.mil@mail.mil. Accepted for publication April 13, 2018.
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Wayne T. Bailey
From the Nephrology Department, and the Rheumatology Department, Walter Reed National Military Medical Center; the Uniformed Services University of the Health Sciences, Bethesda, Maryland, USA. The views expressed in this article are those of the authors and do not reflect the official policy of the US Department of Army/Navy/Air Force, the Department of Defense, or the US government. S.W. Olson, MD, Nephrology Department, Walter Reed National Military Medical Center; S.M. Gordon, MD, Nephrology Department, Walter Reed National Military Medical Center; R. Nee, MD, Nephrology Department, Walter Reed National Military Medical Center; R.S. Stitt, MD, Rheumatology Department, Walter Reed National Military Medical Center; W.T. Bailey, MD, Rheumatology Department, Walter Reed National Military Medical Center; D.J. Little, MD, Nephrology Department, Walter Reed National Military Medical Center; K.R. Knight, MD, Nephrology Department, Walter Reed National Military Medical Center; J.B. Hughes, Medical Student, Uniformed Services University of the Health Sciences; J.D. Edison, MD, Rheumatology Department, Walter Reed National Military Medical Center. Address correspondence to Dr. S.W. Olson, Walter Reed National Military Medical Center, Nephrology, 8901 Rockville Pike, Bethesda, Maryland 20889, USA. E-mail: stephen.w.olson.mil@mail.mil. Accepted for publication April 13, 2018.
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Dustin J. Little
From the Nephrology Department, and the Rheumatology Department, Walter Reed National Military Medical Center; the Uniformed Services University of the Health Sciences, Bethesda, Maryland, USA. The views expressed in this article are those of the authors and do not reflect the official policy of the US Department of Army/Navy/Air Force, the Department of Defense, or the US government. S.W. Olson, MD, Nephrology Department, Walter Reed National Military Medical Center; S.M. Gordon, MD, Nephrology Department, Walter Reed National Military Medical Center; R. Nee, MD, Nephrology Department, Walter Reed National Military Medical Center; R.S. Stitt, MD, Rheumatology Department, Walter Reed National Military Medical Center; W.T. Bailey, MD, Rheumatology Department, Walter Reed National Military Medical Center; D.J. Little, MD, Nephrology Department, Walter Reed National Military Medical Center; K.R. Knight, MD, Nephrology Department, Walter Reed National Military Medical Center; J.B. Hughes, Medical Student, Uniformed Services University of the Health Sciences; J.D. Edison, MD, Rheumatology Department, Walter Reed National Military Medical Center. Address correspondence to Dr. S.W. Olson, Walter Reed National Military Medical Center, Nephrology, 8901 Rockville Pike, Bethesda, Maryland 20889, USA. E-mail: stephen.w.olson.mil@mail.mil. Accepted for publication April 13, 2018.
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Kendral R. Knight
From the Nephrology Department, and the Rheumatology Department, Walter Reed National Military Medical Center; the Uniformed Services University of the Health Sciences, Bethesda, Maryland, USA. The views expressed in this article are those of the authors and do not reflect the official policy of the US Department of Army/Navy/Air Force, the Department of Defense, or the US government. S.W. Olson, MD, Nephrology Department, Walter Reed National Military Medical Center; S.M. Gordon, MD, Nephrology Department, Walter Reed National Military Medical Center; R. Nee, MD, Nephrology Department, Walter Reed National Military Medical Center; R.S. Stitt, MD, Rheumatology Department, Walter Reed National Military Medical Center; W.T. Bailey, MD, Rheumatology Department, Walter Reed National Military Medical Center; D.J. Little, MD, Nephrology Department, Walter Reed National Military Medical Center; K.R. Knight, MD, Nephrology Department, Walter Reed National Military Medical Center; J.B. Hughes, Medical Student, Uniformed Services University of the Health Sciences; J.D. Edison, MD, Rheumatology Department, Walter Reed National Military Medical Center. Address correspondence to Dr. S.W. Olson, Walter Reed National Military Medical Center, Nephrology, 8901 Rockville Pike, Bethesda, Maryland 20889, USA. E-mail: stephen.w.olson.mil@mail.mil. Accepted for publication April 13, 2018.
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James B. Hughes
From the Nephrology Department, and the Rheumatology Department, Walter Reed National Military Medical Center; the Uniformed Services University of the Health Sciences, Bethesda, Maryland, USA. The views expressed in this article are those of the authors and do not reflect the official policy of the US Department of Army/Navy/Air Force, the Department of Defense, or the US government. S.W. Olson, MD, Nephrology Department, Walter Reed National Military Medical Center; S.M. Gordon, MD, Nephrology Department, Walter Reed National Military Medical Center; R. Nee, MD, Nephrology Department, Walter Reed National Military Medical Center; R.S. Stitt, MD, Rheumatology Department, Walter Reed National Military Medical Center; W.T. Bailey, MD, Rheumatology Department, Walter Reed National Military Medical Center; D.J. Little, MD, Nephrology Department, Walter Reed National Military Medical Center; K.R. Knight, MD, Nephrology Department, Walter Reed National Military Medical Center; J.B. Hughes, Medical Student, Uniformed Services University of the Health Sciences; J.D. Edison, MD, Rheumatology Department, Walter Reed National Military Medical Center. Address correspondence to Dr. S.W. Olson, Walter Reed National Military Medical Center, Nephrology, 8901 Rockville Pike, Bethesda, Maryland 20889, USA. E-mail: stephen.w.olson.mil@mail.mil. Accepted for publication April 13, 2018.
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Jess D. Edison
From the Nephrology Department, and the Rheumatology Department, Walter Reed National Military Medical Center; the Uniformed Services University of the Health Sciences, Bethesda, Maryland, USA. The views expressed in this article are those of the authors and do not reflect the official policy of the US Department of Army/Navy/Air Force, the Department of Defense, or the US government. S.W. Olson, MD, Nephrology Department, Walter Reed National Military Medical Center; S.M. Gordon, MD, Nephrology Department, Walter Reed National Military Medical Center; R. Nee, MD, Nephrology Department, Walter Reed National Military Medical Center; R.S. Stitt, MD, Rheumatology Department, Walter Reed National Military Medical Center; W.T. Bailey, MD, Rheumatology Department, Walter Reed National Military Medical Center; D.J. Little, MD, Nephrology Department, Walter Reed National Military Medical Center; K.R. Knight, MD, Nephrology Department, Walter Reed National Military Medical Center; J.B. Hughes, Medical Student, Uniformed Services University of the Health Sciences; J.D. Edison, MD, Rheumatology Department, Walter Reed National Military Medical Center. Address correspondence to Dr. S.W. Olson, Walter Reed National Military Medical Center, Nephrology, 8901 Rockville Pike, Bethesda, Maryland 20889, USA. E-mail: stephen.w.olson.mil@mail.mil. Accepted for publication April 13, 2018.
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Stephen W. Olson
From the Nephrology Department, and the Rheumatology Department, Walter Reed National Military Medical Center; the Uniformed Services University of the Health Sciences, Bethesda, Maryland, USA. The views expressed in this article are those of the authors and do not reflect the official policy of the US Department of Army/Navy/Air Force, the Department of Defense, or the US government. S.W. Olson, MD, Nephrology Department, Walter Reed National Military Medical Center; S.M. Gordon, MD, Nephrology Department, Walter Reed National Military Medical Center; R. Nee, MD, Nephrology Department, Walter Reed National Military Medical Center; R.S. Stitt, MD, Rheumatology Department, Walter Reed National Military Medical Center; W.T. Bailey, MD, Rheumatology Department, Walter Reed National Military Medical Center; D.J. Little, MD, Nephrology Department, Walter Reed National Military Medical Center; K.R. Knight, MD, Nephrology Department, Walter Reed National Military Medical Center; J.B. Hughes, Medical Student, Uniformed Services University of the Health Sciences; J.D. Edison, MD, Rheumatology Department, Walter Reed National Military Medical Center. Address correspondence to Dr. S.W. Olson, Walter Reed National Military Medical Center, Nephrology, 8901 Rockville Pike, Bethesda, Maryland 20889, USA. E-mail: stephen.w.olson.mil@mail.mil. Accepted for publication April 13, 2018.
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Abstract

Objective Systemic sclerosis (SSc) is a disease of autoimmunity, fibrosis, and vasculopathy. Scleroderma renal crisis (SRC) is one of the most severe complications. Corticosteroid exposure, presence of anti-RNA polymerase III antibodies (ARA), skin thickness, and significant tendon friction rubs are among the known risk factors at SSc diagnosis for developing future SRC. Identification of additional clinical characteristics and laboratory findings could expand and improve the risk profile for future SRC at SSc diagnosis.

Methods In this retrospective cohort study of the entire military electronic medical record between 2005 and 2016, we compared the demographics, clinical characteristics, and laboratory results at SSc diagnosis for 31 cases who developed SRC after SSc diagnosis to 322 SSc without SRC disease controls.

Results After adjustment for potential confounding variables, at SSc diagnosis these conditions were all associated with future SRC: proteinuria (p < 0.001; OR 183, 95% CI 19.1–1750), anemia (p = 0.001; OR 9.9, 95% CI 2.7–36.2), hypertension (p < 0.001; OR 13.1, 95% CI 4.7–36.6), chronic kidney disease (p = 0.008; OR 20.7, 95% CI 2.2–190.7), elevated erythrocyte sedimentation rate (p < 0.001; OR 14.3, 95% CI 4.8–43.0), thrombocytopenia (p = 0.03; OR 7.0, 95% CI 1.2–42.7), hypothyroidism (p = 0.01; OR 2.8, 95% CI 1.2–6.7), Anti-Ro antibody seropositivity (p = 0.003; OR 3.9, 95% CI 1.6–9.8), and ARA (p = 0.02; OR 4.1, 95% CI 1.2–13.8). Three or more of these risk factors present at SSc diagnosis was sensitive (77%) and highly specific (97%) for future SRC. No SSc without SRC disease controls had ≥ 4 risk factors.

Conclusion In this SSc cohort, we present a panel of risk factors for future SRC. These patients may benefit from close observation of blood pressure, proteinuria, and estimated glomerular filtration rate, for earlier SRC identification and intervention. Future prospective therapeutic studies could focus specifically on this high-risk population.

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Risk Factors for Future Scleroderma Renal Crisis at Systemic Sclerosis Diagnosis
Sarah M. Gordon, Rodger S. Stitt, Robert Nee, Wayne T. Bailey, Dustin J. Little, Kendral R. Knight, James B. Hughes, Jess D. Edison, Stephen W. Olson
The Journal of Rheumatology Jul 2018, jrheum.171186; DOI: 10.3899/jrheum.171186

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Risk Factors for Future Scleroderma Renal Crisis at Systemic Sclerosis Diagnosis
Sarah M. Gordon, Rodger S. Stitt, Robert Nee, Wayne T. Bailey, Dustin J. Little, Kendral R. Knight, James B. Hughes, Jess D. Edison, Stephen W. Olson
The Journal of Rheumatology Jul 2018, jrheum.171186; DOI: 10.3899/jrheum.171186
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