Outcome of incomplete systemic lupus erythematosus after 10 years

Abstract

The objective was to identify cases of incomplete systemic lupus erythematosus (SLE) within a defined population in southern Sweden, risk factors for development of complete SLE (≥4 classification criteria) and study outcome of the patients. During prospective retrieval of SLE cases within a defined population in southern Sweden, 28 patients (26 women, two men) with incomplete SLE (< 4 ACR criteria) were identified between 1981 and 1992. All patient records were reviewed and clinical and laboratory data were extracted from standardized formats. Organ damage was defined according to the SLICC/ACR damage index. During follow-ups, 16 of 28 patients developed complete SLE (median 13 years; range 10-20 years). The time to develop complete SLE varied between one and ten years with a median time of 5.3 years. Three patients were anti-DNA positive at inclusion; only one of them developed complete SLE. False positive Wasserman reaction and anti-cardiolipinantibodies (aCl) were only found in patients who developedcomplete SLE (P < 0.04; Fisher exact test). Six patientshad malar rash from the start and they all had complete SLE at follow-up (P < 0.04; Fisher exact test). Of eight patients with arthritis, three developed complete SLE. Thrombocytopeniawas only found in two patients, both developing complete disease. At follow-up, patients that developed complete SLE had high SLICC damage scores (mean 1.5) compared with patients that remained as incomplete SLE (mean 0.16). In conclusion, in this follow-up study of patients with incompleteSLE 57% developedcomplete disease after a median time of 5.3 years.Malar rash and aCl were predictors of complete SLE. Individuals that developed complete SLE were more prone to organ damage.

Keywords

anticardiolipin antibodies incomplete SLE SLICC/ACR damage index

Get full access to this article

View all access options for this article.

References

Tan EM , Cohen AS , Fries IF et al. The 1982 revised criteria for the classification of systemic lupus erythematosus . Arthritis Rheum 1982; 25: 1271-1277 .

Swaak AJG , van de Brink H , Smeenk RJT et al. Incomplete lupus erythematosus: results of a multicentre study under the supervision of the EULAR Standing Committee on International Clinical Studies Including Therapeutic Trials (ESCISIT) . Rheumatology 2001; 40: 89-94 .

Ganczarczyk L , Urowitz MB , Gladman DD. Latent lupus . J Rheumatol 1989; 16: 475-478 .

Vila LM , Mayor AM , Valentin AH , Garcia-Soberal M , Vila S. Clinical outcome and predictors of disease evolution in patients with incomplete lupus erythematosus . Lupus 2000; 9: 110-115 .

Ståhl-Hallengren C , Jönsen A , Nived O , Sturfelt G. Incidence studies of systemic lupus erythematosus in Southern Sweden: increasing age, decreasing frequency of renal manifestations and good prognosis . J Rheumatol 2000; 27: 685-691 .

Jonsson H , Nived O , Sturfelt G. Outcome in systemic lupus erythematosus: a prospective study of patients from a defined population . Medicine (Baltimore) 1989; 68: 141-150 .

Jonson H , Nived O , Sturfelt G , Silman A. Estimating the incidence of systemic lupus erythematosus in a defined population using multiple sources of retrieval . Br J Rheumatol 1990; 29: 185-188 .

Sturfelt G , Eskilsson J , Nived O , Truedsson L , Valind S. Cardiovascular disease in systemic lupus erythematosus. A study of 75 patients from a defined population . Medicine 1992; 71: 216-223 .

Gladman DD , Urowitz MB , Goldsmith CH et al. The reliability of the Systemic Lupus International Collaborating Clinics/American College of Rheumatology damage index in patients with systemic lupus erythematosus . Arthritis Rheum 1997; 40: 809-813 .

10.

Cavazzana I , Franceschini F , Belfiore N et al. Undifferentiated connective tissue disease with antibodies to Ro/SSa: clinical features and follow-up of 148 patients . Clin Exp Rheumatol 2001; 19: 403-409 .

11.

Greer JM , Panush RS. Incomplete lupus erythematosus . Arch Intern Med 1989; 149: 2473-2476 .

12.

Calvo-Alen J , Bastian HM , Straaton KV , Burgard SL , Mikhail IS , Alarcon GS. Identification of patient subsets among those presumptively diagnosed with, referred, and/or followed up for systemic lupus erythematosus at a large tertiary care center . Arthritis Rheum 1995; 38: 1475-1484 .

13.

Costenbader KH , Karlsson EW , Mandl LA. Defining lupus cases for clinical studies: the Boston weighted criteria for the classification of systemic lupus erythematosus . J Rheumatol 2002; 29: 2545-2550 .

14.

Calvo-Alen J , Alarcon GS , Burgard SL , Burst N , Bartolucci AA , Williams HJ. Systemic lupus erythematosus: predictors of its occurrence among a cohort of patients with early undifferentiated connective tissue disease: multivariate analyses and identification of risk factors . J Rheumatol 1996; 23: 469-475 .

15.

Arbuckle MR , James JA , Kohlhase KF , Rubertone MC , Dennis GJ , Harley JB. Development of anti-dsDNA auto antibodies prior to clinical diagnosis of systemic lupus erythematosus . Scand j Immunol 2001; 54: 211-219 .

16.

Esdaile JM , Abrahamowicz M , MacKenzie T , Hayslett JP , Kashgaria M. The time-dependence of long-term prediction in lupus nephritis . Arthritis Rheum 1994; 37: 359-368 .

17.

Rivest C , Lew RA , Welsing PM et al. Association between clinical factors, socioeconomic status, and organ damage in recent onset systemic lupus erythematosus . J Rheumatol 2000; 27: 680-684 .

18.

Rahman P , Gladman DD , Urowitz MB , Hallet D , Tam LS. Early damage as measured by the SLICC/ACR damage index is a predictor of mortality in systemic lupus erythematosus . Lupus 2001; 10: 93-96 .

19.

Nived O , Jönsen A , Bengtsson AA , Bengtsson C , Sturfelt G. High predictive value of SLICC/ACR damage index for survival in SLE . J Rheumatol 2002; 29: 1398-1400 .