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Organ damage in high-risk patients with systemic and incomplete lupus syndromes

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Abstract

The objective of this study was to characterize organ damage in lupus patients enrolled in Dallas Regional Autoimmune Disease Registry (DRADR). Retrospective chart review was carried out on 99 patients with four or more diagnostic criteria for systemic lupus erythematosus (SLE) and 15 with less than four of these criteria, who were designated as having incomplete lupus erythematosus (ILE). The majority of patients (84 %) were African American or Hispanic/Latino; mean disease duration was 9.5 years. The mean damage score was 1.57 (range 0–8), and a damage score greater than 0 was present in 64 % of the patients. The ILE group had lower mean damage scores (0.67) than the SLE group (1.67; P = 0.04), explained in part by the shorter disease duration in the ILE patients (4.33 vs. 10.24 years; P = 0.003). The most prevalent damage category was renal, present in 24 % of patients. Malignancies occurred in individuals who were significantly older than those who had renal or peripheral vascular damage (P = 0.0007). The findings confirm clinical impressions that DRADR includes a high-risk lupus population. The ILE patients have less damage but also shorter disease duration, suggesting that this might represent an earlier disease stage. These results are consistent with the hypothesis that ILE patients include a subset that is likely to experience progressive organ damage. Longitudinal study of these patients has significant likelihood of tracking the changes that are correlated with disease progression to SLE.

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References

  1. Vilá LM, Alarcón GS, McGwin G, Friedman AW, Baethge BA, Bastian HM, Fessler BJ, Reveille JD, Group LS (2004) Early clinical manifestations, disease activity and damage of systemic lupus erythematosus among two distinct US Hispanic subpopulations. Rheumatology (Oxford) 43:358–363

    Article  Google Scholar 

  2. Korbet SM, Schwartz MM, Evans J, Lewis EJ, Group CS (2007) Severe lupus nephritis: racial differences in presentation and outcome. J Am Soc Nephrol 18:244–254

    Article  PubMed  Google Scholar 

  3. Tan E, Cohen A, Fries J, Masi A, McShane D, Rothfield N, Schaller J, Talal N, Winchester R (1982) The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 25:1271–1277

    Article  PubMed  CAS  Google Scholar 

  4. Ståhl Hallengren C, Nived O, Sturfelt G (2004) Outcome of incomplete systemic lupus erythematosus after 10 years. Lupus 13:85–88

    Article  PubMed  Google Scholar 

  5. Gladman D, Ginzler E, Goldsmith C, Fortin P, Liang M, Urowitz M, Bacon P, Bombardieri S, Hanly J, Hay E, Isenberg D, Jones J, Kalunian K, Maddison P, Nived O, Petri M, Richter M, Sanchez-Guerrero J, Snaith M, Sturfelt G, Symmons D, Zoma A (1996) The development and initial validation of the Systemic Lupus International Collaborating Clinics/American College of Rheumatology damage index for systemic lupus erythematosus. Arthritis Rheum 39:363–369

    Article  PubMed  CAS  Google Scholar 

  6. Gladman DD, Urowitz MB, Goldsmith CH, Fortin P, Ginzler E, Gordon C, Hanly JG, Isenberg DA, Kalunian K, Nived O, Petri M, Sanchez-Guerrero J, Snaith M, Sturfelt G (1997) The reliability of the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index in patients with systemic lupus erythematosus. Arthritis Rheum 40:809–813

    Article  PubMed  CAS  Google Scholar 

  7. Dixon WG, Carmona L, Finckh A, Hetland ML, Kvien TK, Landewe R, Listing J, Nicola PJ, Tarp U, Zink A, Askling J (2010) EULAR points to consider when establishing, analysing and reporting safety data of biologics registers in rheumatology. Ann Rheum Dis 69:1596–1602

    Article  PubMed  Google Scholar 

  8. Wandstrat A, Carr-Johnson F, Branch V, Gray H, Fairhurst A, Reimold A, Karp D, Wakeland E, Olsen N (2006) Autoantibody profiling to identify individuals at risk for systemic lupus erythematosus. J Autoimmun 27:153–160

    Article  PubMed  CAS  Google Scholar 

  9. Vilá LM, Alarcón GS, McGwin G, Bastian HM, Fessler BJ, Reveille JD, Group LS (2005) Systemic lupus erythematosus in a multiethnic cohort (LUMINA): XXIX. Elevation of erythrocyte sedimentation rate is associated with disease activity and damage accrual. J Rheumatol 32:2150–2155

    PubMed  Google Scholar 

  10. Kamen DL, Barron M, Parker TM, Shaftman SR, Bruner GR, Aberle T, James JA, Scofield RH, Harley JB, Gilkeson GS (2008) Autoantibody prevalence and lupus characteristics in a unique African American population. Arthritis Rheum 58:1237–1247

    Article  PubMed  Google Scholar 

  11. Sung YK, Hur NW, Sinskey JL, Park D, Bae SC (2007) Assessment of damage in Korean patients with systemic lupus erythematosus. J Rheumatol 34:987–991

    PubMed  Google Scholar 

  12. Alarcón GS, McGwin G, Petri M, Reveille JD, Ramsey-Goldman R, Kimberly RP, Group PS (2002) Baseline characteristics of a multiethnic lupus cohort: PROFILE. Lupus 11:95–101

    PubMed  Google Scholar 

  13. Zonana-Nacach A, Barr SG, Magder LS, Petri M (2000) Damage in systemic lupus erythematosus and its association with corticosteroids. Arthritis Rheum 43:1801–1808

    Article  PubMed  CAS  Google Scholar 

  14. Laustrup H, Voss A, Green A, Junker P (2010) SLE disease patterns in a Danish population-based lupus cohort: an 8-year prospective study. Lupus 19:239–246

    Article  PubMed  CAS  Google Scholar 

  15. Grönhagen CM, Fored CM, Granath F, Nyberg F (2011) Cutaneous lupus erythematosus and the association with systemic lupus erythematosus: a population-based cohort of 1,088 patients in Sweden. Br J Dermatol 164:1335–1341

    Article  PubMed  Google Scholar 

  16. Laustrup H, Voss A, Green A, Junker P (2009) Occurrence of systemic lupus erythematosus in a Danish community: an 8-year prospective study. Scand J Rheumatol 38:128–132

    Article  PubMed  CAS  Google Scholar 

  17. Chambers SA, Allen E, Rahman A, Isenberg D (2009) Damage and mortality in a group of British patients with systemic lupus erythematosus followed up for over 10 years. Rheumatology (Oxford) 48:673–675

    Article  Google Scholar 

  18. Mok CC, Ho CT, Wong RW, Lau CS (2003) Damage accrual in southern Chinese patients with systemic lupus erythematosus. J Rheumatol 30:1513–1519

    PubMed  Google Scholar 

  19. Rabbani MA, Habib HB, Islam M, Ahmad B, Shah SM, Tahir S, Merchant D, Ahmad A (2010) Early renal damage assessed by the SLICC/ACR damage index is predictor of severe outcome in lupus patients in Pakistan. Lupus 19:1573–1578

    Article  PubMed  CAS  Google Scholar 

  20. Danila MI, Pons-Estel GJ, Zhang J, Vilá LM, Reveille JD, Alarcón GS (2009) Renal damage is the most important predictor of mortality within the damage index: data from LUMINA LXIV, a multiethnic US cohort. Rheumatology (Oxford) 48:542–545

    Article  Google Scholar 

  21. Boya P, Gonzalez-Polo RA, Poncet D, Andreau K, Vieira HL, Roumier T, Perfettini JL, Kroemer G (2003) Mitochondrial membrane permeabilization is a critical step of lysosome-initiated apoptosis induced by hydroxychloroquine. Oncogene 22:3927–3936

    Article  PubMed  CAS  Google Scholar 

  22. Lightstone L (2013) Minimising steroids in lupus nephritis—will B cell depletion pave the way? Lupus 22:390–399

    Article  PubMed  CAS  Google Scholar 

  23. Petri M, Purvey S, Fang H, Magder LS (2012) Predictors of organ damage in systemic lupus erythematosus: the Hopkins Lupus Cohort. Arthritis Rheum 64:4021–4028

    Article  PubMed  CAS  Google Scholar 

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Acknowledgments

This work was supported by NIH/NIAMS P50AR0455503. NJO is also supported in part, under a grant with the Pennsylvania Department of Health using Tobacco CURE Funds. The Department specifically disclaims responsibility for any analyses, interpretations or conclusions. We appreciate the assistance of Alice Cox, Pratik Doshi, Heidi Prather, Justin Ribault and Sarmistha Sen for enrolling and evaluating the individuals enrolled in DRADR. Michelle Christadoss and Ferdicia Carr-Johnson processed the samples and carried out the laboratory assessments. We are deeply indebted to our late colleague, Valerie K. Branch, MS, MBA, whose dedication to establishing DRADR made this study possible.

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Correspondence to Nancy J. Olsen.

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Olsen, N.J., Yousif, M., Mutwally, A. et al. Organ damage in high-risk patients with systemic and incomplete lupus syndromes. Rheumatol Int 33, 2585–2590 (2013). https://doi.org/10.1007/s00296-013-2783-3

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