Welcome to the 2021 LUPUS CORA Meeting Program Scheduling

Background and Aims

To evaluate the rate and features of SLE patients who successfully tapered prednisone (equivalent) below 5 mg/day, within 2-year since diagnosis, in a real-life prospective study.

Methods

The Early Lupus Project recruited a multicenter inception cohort of patients within 12 months of SLE diagnosis. At enrolment and then every 6 months a panel of data (including demographic, comorbidities, serologic, clinic by BILAG2004 domains, ECLAM, HRQoL, and treatment) was recorded. Forward-Backward Cox-regression models were fitted with covariates with univariate p<0.05 to identify baseline factors independently associated with the successful tapering of prednisone below 5 mg/day.

Results

Overall, 127 patients (17.3% males) were eligible for this study having at least a 24-month follow-up and a prednisone dose ≥5 mg at enrolment. Mean age was 36.7 (±13.4), median disease duration was 183 (40 - 346) days and prednisone dose at baseline was 17.9 (±14.4) mg/day. During the 2-year follow-up, 99 patients (78%) tapered the prednisone dose <5 mg/day (see Figure 1). However, 42/99 (42.4%) required to increase the prednisone dose ≥5 mg/day because of inadequate disease activity control within the end of the 2-year follow-up. Only 17/127 (13.4%) patients successfully discontinued prednisone treatment.

Hematologic (HR 0.41; p<0.001) and renal (HR 0.54; p=0.019) involvement, SDI≥1 (HR 0.55; p=0.022), and C3 complement levels (HR 1.04; P=0.021) at baseline were negatively associated with the successful tapering of prednisone < 5mg/day which was not influenced by baseline dose and concomitant medications.

Conclusions

Prednisone tapering below 5 mg/day is achievable within 2-years since diagnosis by more than half of patients.

Background and Aims

Pre-clinical autoimmunity can reveal immune changes that initiate or prevent clinical disease. We reported that (i) 16% ANA-positive At-Risk individuals progressed to SLE/pSS in year 1; (ii) ANA-positive individuals without clinical disease have defective pDC function and non-haematopoietic interferon-production. Here we describe the immunophenotype of our ANA-positive cohort compared to healthy controls.

Methods

A prospective observational study of At-Risk individuals was conducted over 3 years. Annual follow up data was used to categorise patients as: ‘Progressors’ (classification criteria for SLE, pSS or other RMD); 'Undifferentiated autoimmune disease' (at least one clinical criterion but without progression); 'Benign autoreactivity' (no clinical criteria). Previously validated IFN-Scores and flow cytometry for major circulating subsets were analysed at baseline.

Results

148/150 patients had 3-year follow up. Outcomes were: progressors: 30/148 (20%) [SLE=25; pSS=5]; Undifferentiated autoimmune disease: 51/148 (34%); Benign autoreactivity: 67/148 (45%).

Compared to healthy controls, Progressors had increased IFN-Score-A(p<0.001), IFN-Score-B(p=0.073), reduced monocytes(p=0.077), but increased naïve-B(p<0.001), transitional-B(p=0.014) and CD4 T-cells(p=0.018). Similarly, Undifferentiated autoimmune disease had increased IFN-Score-A(p=0.005), reduced monocytes(p=0.002), and increased naïve B(p<0.001), transitional B(p=0.014) and CD 4 T cells(p=0.018).

In patients with benign autoreactivity, despite no clinical criteria for RMD after 3 years, we found increased IFN-Score-A(p=0.090), reduced monocytes(p=0.066), increased naïve-B cells(p<0.001), transitional B-cells(p=0.046) and CD4 T-cells(p=0.032) similar to the other groups, but in contrast, this group also had significantly reduced CD8 T-cells(p=0.006).

Conclusions

Clinically benign ANA positivity is a complex immune state with features seen in SLE; pDC exhaustion, keratinocyte interferon production, increased blood interferon score, and disturbance of peripheral blood mononuclear cell (PBMC) subsets.

Background and Aims

Focal necrotizing glomerular lesions (FNGL) in patients with lupus nephritis (LN) are commonly associated with crescents formation and may have worse renal outcomes. Clinical factors predictive of FNGL remain unclear. This study was aimed to identify such factors.

Methods

The cross-sectional study included 133 cases of biopsy-proven LN. Clinical and histological data were analyzed at the time of kidney biopsy (Bx). FNGL defined as the presence of cellular/fibrocellular crescents.

Results

FNGL were found in 27% of LN cases and had more pronounced active glomerular and tubulointerstitial histological lesions and clinical manifestations (table 1). Acute kidney injury (AKI) at the time of Bx (HR 6.10, 95%CI 1.57-23.75) and the abnormal anti-dsDNA levels in combination with the decrease in serum C3 and/or C4 complement (НR 9.99, 95%CI 2.10-47.25) were independently associated with FNGL.

anti-dsDNA, anti-double-stranded DNA antibodies; eGFR, estimated glomerular filtration rate; RBC, red blood cell; data presented as mean±SD or median (interquartile range)

Conclusions

FNGL are associated with unfavorable clinical phenotype. This subset of LN requires further studies targeting its outcomes and optimal treatment.