PT  - JOURNAL ARTICLE
AU  - Arriens, Cristina
AU  - Alexander, Roberta Vezza
AU  - Narain, Sonali
AU  - Saxena, Amit
AU  - Collins, Christopher E
AU  - Wallace, Daniel J
AU  - Massarotti, Elena
AU  - Conklin, John
AU  - Kalunian, Kenneth C
AU  - Putterman, Chaim
AU  - Ramsey-Goldman, Rosalind
AU  - Buyon, Jill P
AU  - Askanase, Anca
AU  - Furie, Richard A
AU  - James, Judith A
AU  - Bello, Ghalib A
AU  - Manzi, Susan
AU  - Ahearn, Joseph
AU  - O"Malley, Tyler
AU  - Weinstein, Arthur
AU  - Dervieux, Thierry
TI  - Cell-bound complement activation products associate with lupus severity in SLE
AID  - 10.1136/lupus-2019-000377
DP  - 2020 Apr 01
TA  - Lupus Science & Medicine
PG  - e000377
VI  - 7
IP  - 1
4099  - http://lupus.bmj.com/content/7/1/e000377.short
4100  - http://lupus.bmj.com/content/7/1/e000377.full
SO  - Lupus Sci Med2020 Apr 01; 7
AB  - Objectives To evaluate the association between lupus severity and cell-bound complement activation products (CB-CAPs) or low complement proteins C3 and C4.Methods All subjects (n=495) fulfilled the American College of Rheumatology (ACR) classification criteria for SLE. Abnormal CB-CAPs (erythrocyte-bound C4d or B-lymphocyte-bound C4d levels >99th percentile of healthy) and complement proteins C3 and C4 were determined using flow cytometry and turbidimetry, respectively. Lupus severity was estimated using the Lupus Severity Index (LSI). Statistical analysis consisted of multivariable linear regression and groups comparisons.Results Abnormal CB-CAPs were more prevalent than low complement values irrespective of LSI levels (62% vs 38%, respectively, p<0.0001). LSI was low (median 5.44, IQR: 4.77–6.93) in patients with no complement abnormality, intermediate in patients with abnormal CB-CAPs (median 6.09, IQR: 5.31–8.20) and high in the group presenting with both abnormal CB-CAPs and low C3 and/or C4 (median 7.85, IQR: 5.51–8.37). Odds of immunosuppressant use was higher in subjects with LSI ≥5.95 compared with subjects with LSI <5.95 (1.60 vs 0.53, p<0.0001 for both). Multivariable regression analysis revealed that higher LSI scores associated with abnormal CB-CAPs—but not low C3/C4—after adjusting for younger age, race and longer disease duration (p=0.0001), which were also independent predictors of disease severity (global R2=0.145).Conclusion Abnormalities in complement activation as measured by CB-CAPs are associated with increased LSI.