Risk Factors for Future Scleroderma Renal Crisis at Systemic Sclerosis Diagnosis

Sarah M Gordon^{1

2}, Rodger S Stitt^{1

2}, Robert Nee^{1

2}, Wayne T Bailey^{1

2}, Dustin J Little^{1

2}, Kendral R Knight^{1

2}, James B Hughes^{1

2}, Jess D Edison^{1

2}, Stephen W Olson^{3

4}

Affiliations

¹ 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.
² 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.
³ 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. stephen.w.olson.mil@mail.mil.
⁴ 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. stephen.w.olson.mil@mail.mil.

PMID: 30008456
DOI: 10.3899/jrheum.171186

Risk Factors for Future Scleroderma Renal Crisis at Systemic Sclerosis Diagnosis

Sarah M Gordon et al. J Rheumatol. 2019 Jan.

. 2019 Jan;46(1):85-92.

doi: 10.3899/jrheum.171186. Epub 2018 Jul 15.

Authors

Affiliations

¹ 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.
² 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.
³ 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. stephen.w.olson.mil@mail.mil.
⁴ 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. stephen.w.olson.mil@mail.mil.

PMID: 30008456
DOI: 10.3899/jrheum.171186

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.

Keywords: HYPERTENSION; PROTEINURIA; RISK FACTORS; SCLERODERMA RENAL CRISIS; SYSTEMIC SCLEROSIS.

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Comment in

Scleroderma Renal Crisis: Still a Lot To Do.
Codullo V, Montecucco C. Codullo V, et al. J Rheumatol. 2019 Jan;46(1):3-4. doi: 10.3899/jrheum.180594. J Rheumatol. 2019. PMID: 30600234 No abstract available.

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Risk Factors for Future Scleroderma Renal Crisis at Systemic Sclerosis Diagnosis

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Risk Factors for Future Scleroderma Renal Crisis at Systemic Sclerosis Diagnosis

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