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

Abstract

The objective of this study was to characterize organ damage in lupus patients enrolled in the Dallas Regional Autoimmune Disease Registry (DRADR). Retrospective chart review was carried out on 99 patients with 4 or more diagnostic criteria for systemic lupus erythematosus (SLE) and 15 with less than 4 of these criteria, who were designated as having incomplete lupus (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 to 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 years 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 the 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.

Clinical and Immunologic Profiles in Incomplete Lupus Erythematosus and Improvement with Hydroxychloroquine Treatment

“…In some of these ILE patients, loss of scoring for photosensitivity was counterbalanced by the addition of hypocomplementemia, resulting in no change in the overall score. As in most of the other reports, renal and neurologic criteria were rare or absent in ILE [18] and the more specific antibodies such as dsDNA were also significantly less prevalent than in SLE.…”

Preclinical Systemic Lupus Erythematosus

“…In this retrospective study cohort, 15 individuals out of 124 were classified as ILE and 99 out of 124 were classified as SLE. (31) The ILE patients were shown to have a significantly lower mean damage score than SLE patients (0.67 ± 0.32 vs 1.67 ± 0.17; p=0.036) and a significantly shorter disease duration (4.33 ± 0.94 years vs 10.24 ± 0.75 years; p=0.003). (31) In another US-based study from the DRADR following 22 ILE Patients (<4 ACR criteria) for an average of 2.4 years, 14% (n=3) transitioned to SLE classification.…”

“…(31) The ILE patients were shown to have a significantly lower mean damage score than SLE patients (0.67 ± 0.32 vs 1.67 ± 0.17; p=0.036) and a significantly shorter disease duration (4.33 ± 0.94 years vs 10.24 ± 0.75 years; p=0.003). (31) In another US-based study from the DRADR following 22 ILE Patients (<4 ACR criteria) for an average of 2.4 years, 14% (n=3) transitioned to SLE classification. (32) All patients who transitioned were female, of younger age, and showed increases in IgG autoreactivity.…”

We Need Better Classification and Terminology for “People at High Risk of or in the Process of Developing Lupus”

“…In most of these studies, only a small proportion of patients (approximately 20%) evolve to either SLE classification or SLE diagnosis 9,10 The term “undifferentiated connective tissue disease” (UCTD) has been used to refer to individuals presenting with clinical symptoms suggestive of some autoimmune connective tissue disease (SLE, Sjögren’s syndrome, scleroderma, mixed connective tissue disease or RA), but not clearly meeting ACR classification criteria for any of these diseases, a common clinical scenario. Follow-up studies of UCTD patients have revealed the rates of progressing to SLE are very similar to those in incomplete lupus cohorts, approximately 20% 9–19 .…”

“…Follow-up studies of UCTD patients have revealed the rates of progressing to SLE are very similar to those in incomplete lupus cohorts, approximately 20% 9–19 . Additionally, those patients who have a prolonged clinical onset of their SLE tend to have a mild disease course, without severe organ involvement 10 .…”