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Background and Aims

Whether immunosuppressive therapy (IS) may be safely withdrawn in lupus nephritis (LN) is still unclear. We assessed rate and predictors of flare after IS withdrawal in patients with class III/IV LN in remission.

Methods

Patients with class III/IV biopsy-proven LN treated with IS between 1980 and 2020 were considered. Remission was defined as normal serum creatinine, proteinuria <0.5 g/24h, inactive urine sediment, and no extra-renal SLE activity on stable immunosuppressive and/or antimalarial therapy and/or prednisone ≤5mg/day. IS discontinuation was defined as the complete withdrawal of immunosuppressants, flares according to SLEDAI-Flare-Index. Predictors of flare were analyzed by multivariate logistic regression.

Results

Among 513 SLE patients included in our database, 270 had LN. Sixty-eight patients with class III/IV LN (28.5%) discontinued IS, 49±28 months after remission achievement. During a mean±SD follow-up of 119±72 months, 19 patients (27.9%) developed a flare (8 renal), meaning a flare rate of 2.7/100patients/year. No difference in flare rate between patients with normal or low C3/C4 was observed, nor between anti-dsDNA positive or negative patients; 7/8 renal flares occurred in patients with class IV LN. At multivariate analysis, antimalarial therapy (OR 0.121, 95%CI 0.017-0.854, p=0.034), and remission longer than 3 years before IS discontinuation (OR 0.244, 95%CI 0.060-0.997, p=0.050) protected against disease flares. The 19 patients with flares were re-treated, and 14/19 (73.7%) re-achieved remission after restarting therapy (12 resumed IS (63.2%), 4 started belimumab (21%) and 3 (15.8%) glucocorticoids alone).

Conclusions

Withdrawal of IS seems feasible in patients with proliferative LN in remission for at least 3 years and on antimalarials.

Background and Aims

The MSK components of the BILAG and SLEDAI have limited sensitivity, specificity and responsiveness. We aimed to develop a better disease activity tool for MSK lupus.

Methods

“LAMDA” was derived using data from the multicentre USEFUL study (PMID:33792659); 133 patients with inflammatory MSK pain received intramuscular depomedrone then were assessed for 66/68 SJC/TJC, BILAG-2004, SLEDAI-2K, physician MSK-VAS, inflammatory markers, patient pain and disease-activity-VAS and MSK-ultrasound. Baseline variables were modelled against US using penalized (Lasso) regression. For validation we evaluated: (i) responsiveness at week 6 in USEFUL and (ii) association with quality of life and treatment decision in an independent study (n=100).

Results

LAMDA was a composite of SJC, patient-MSK-pain-VAS, physician-MSK-disease-activity-VAS and ESR ranging from 0 to 26.5. Response effect size was greater for the LAMDA (0.37) than the BILAG-MSK (0.31), SLEDAI-MSK (0.27) and total US score (0.33). With active US at baseline, LAMDA’s effect size was 0.42.

In the validation study LAMDA score correlated with better physical function (R = -0.49, p<0.001), pain (R = -0.44, p=0.002) and Burden to Others (R = -0.38, p=0.009). LAMDA was higher when therapy was increased (mean (95% CI) difference 12.9 (5.8, 19.9), p<0.001).

Conclusions

LAMDA is a continuous disease activity instrument for MSK lupus that is sensitive to imaging-synovitis without swelling, more responsive than other instruments and correlates with quality of life and treatment decision. LAMDA may improve the ability of clinicians to monitor and treat MSK lupus, and determine the efficacy of therapies in clinical trials.

Background and Aims

BILAG-2004 is a comprehensive disease activity instrument for SLE but administrative burden and frequency of errors limits its use in routine practice. We aimed to develop a new tool, known as “Easy-BILAG”, for accurate, time-efficient scoring of BILAG-2004 in routine practice, with full fidelity to the original instrument.

Methods

Frequency of SLE manifestations was collated from the UK BILAG-biologics registry dataset. Easy-BILAG prototype formats were drafted to address known issues affecting speed and accuracy of completion. Accuracy and usability of the finalised, expert-verified Easy-BILAG was tested against standard format BILAG-2004 in a validation workbook exercise of case vignettes. 33 rheumatology professionals from 14 UK centres and ranging in expertise, completed the workbook.

Results

Easy-BILAG incorporates the most frequently encountered features of SLE, plus complete constitutional and renal domains into a rapid single page assessment. An embedded glossary and colour-coding assists scoring each organ domain. An optional second page captures all rarer items if needed. In the validation exercise, Easy-BILAG yielded significantly higher scoring accuracy (96.7%) than standard BILAG-2004 documentation (87.8%, p≤0.001), with better inter-rater agreement. Gains in accuracy with Easy-BILAG were most apparent among general hospital rheumatologists, leading to equivalent accuracy as tertiary centre rheumatologists (93.3% vs 96.6%, p=0.17). Easy-BILAG was completed significantly faster than the standard format and was rated as intuitive and well adapted for routine practice by clinicians.

Conclusions

Easy-BILAG facilitates more rapid and accurate scoring of BILAG-2004 and could assist professionals in all settings integrate SLE disease activity recording into routine care.