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Risk Factors for Future Scleroderma Renal Crisis at Systemic Sclerosis Diagnosis.
J Rheumatol. 2019 01; 46(1):85-92.JR

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.

Authors+Show Affiliations

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. 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.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. 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.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. 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.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. 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.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. 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.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. 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.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. 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.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. 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.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. 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.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

30008456

Citation

Gordon, Sarah M., et al. "Risk Factors for Future Scleroderma Renal Crisis at Systemic Sclerosis Diagnosis." The Journal of Rheumatology, vol. 46, no. 1, 2019, pp. 85-92.
Gordon SM, Stitt RS, Nee R, et al. Risk Factors for Future Scleroderma Renal Crisis at Systemic Sclerosis Diagnosis. J Rheumatol. 2019;46(1):85-92.
Gordon, S. M., Stitt, R. S., Nee, R., Bailey, W. T., Little, D. J., Knight, K. R., Hughes, J. B., Edison, J. D., & Olson, S. W. (2019). Risk Factors for Future Scleroderma Renal Crisis at Systemic Sclerosis Diagnosis. The Journal of Rheumatology, 46(1), 85-92. https://doi.org/10.3899/jrheum.171186
Gordon SM, et al. Risk Factors for Future Scleroderma Renal Crisis at Systemic Sclerosis Diagnosis. J Rheumatol. 2019;46(1):85-92. PubMed PMID: 30008456.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Risk Factors for Future Scleroderma Renal Crisis at Systemic Sclerosis Diagnosis. AU - Gordon,Sarah M, AU - Stitt,Rodger S, AU - Nee,Robert, AU - Bailey,Wayne T, AU - Little,Dustin J, AU - Knight,Kendral R, AU - Hughes,James B, AU - Edison,Jess D, AU - Olson,Stephen W, Y1 - 2018/07/15/ PY - 2018/04/13/accepted PY - 2018/7/17/pubmed PY - 2020/6/17/medline PY - 2018/7/17/entrez KW - HYPERTENSION KW - PROTEINURIA KW - RISK FACTORS KW - SCLERODERMA RENAL CRISIS KW - SYSTEMIC SCLEROSIS SP - 85 EP - 92 JF - The Journal of rheumatology JO - J. Rheumatol. VL - 46 IS - 1 N2 - 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. SN - 0315-162X UR - https://www.unboundmedicine.com/medline/citation/30008456/Risk_Factors_for_Future_Scleroderma_Renal_Crisis_at_Systemic_Sclerosis_Diagnosis_ L2 - http://www.jrheum.org/cgi/pmidlookup?view=long&amp;pmid=30008456 DB - PRIME DP - Unbound Medicine ER -