2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus

Aringer, Martin; Costenbader, Karen H.; Daikh, David I.; Brinks, Ralph; Mosca, Marta; Ramsey‐Goldman, Rosalind; Smolen, Josef S.; Wofsy, David; Boumpas, Dimitrios T.; Kamen, Diane L.; Jayne, David; Cervera, Ricard; Costédoat-Chalumeau, Nathalie; Diamond, Betty; Gladman, Dafna D.; Hahn, Bevra H.; Hiepe, Falk; Jacobsen, Søren; Khanna, Dinesh; Lerstrøm, Kirsten; Massarotti, Elena; McCune, Joseph M.; Ruiz‐Irastorza, Guillermo; Sánchez‐Guerrero, Jorge; Schneider, Matthias; Urowitz, Murray B.; Βertsias, George; Hoyer, Bimba F.; Leuchten, Nicolai; Tani, Chiara; Tedeschi, Sara K.; Touma, Zahi; Schmajuk, Gabriela; Anić, Branimir; Assan, Florence; Chan, Tak Mao; Clarke, Ann E.; Crow, Mary K.; Czirják, László; Doria, Andrea; Graninger, Winfried; Halda-Kiss, Bernadett; Hasni, Sarfaraz; Izmirly, Peter; Jung, Michelle; Kumánovics, Gabór; Mariette, Xavier; Padjen, Ivan; Pego-Reigosa, José María; Romero-Díaz, Juanita; Fernández, Íñigo Rúa-Figueroa; Séror, Raphaèle; Stummvoll, Georg; Tanaka, Yoshiya; Tektonidou, Maria G.; Vasconcelos, Carlos; Vital, Edward M; Wallace, Daniel J.; Yavuz, Şule; Meroni, Pier Luigi; Fritzler, Marvin J.; Naden, Ray; Dörner, Thomas; Johnson, Sindhu R.

doi:10.1136/annrheumdis-2018-214819

Cited by 1,006 publications

(521 citation statements)

References 32 publications

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“…124 The development of new classification criteria for SLE with increased weighting for kidney disease will facilitate the inclusion of more patients in LN trials. 125 New drugs in development for LN, including novel CNI, B-cell inhibiting and depleting agents, kinase inhibitors, inhibitors of costimulation, inhibitors of complement activation, in combination with improved trial designs, may provide additional agents in the near future.…”

Section: Recommendationmentioning

confidence: 99%

2019 Update of the Joint European League Against Rheumatism and European Renal Association–European Dialysis and Transplant Association (EULAR/ERA–EDTA) recommendations for the management of lupus nephritis

et al. 2020

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ObjectiveTo update the 2012 EULAR/ERA–EDTA recommendations for the management of lupus nephritis (LN).MethodsFollowing the EULAR standardised operating procedures, a systematic literature review was performed. Members of a multidisciplinary Task Force voted independently on their level of agreeement with the formed statements.ResultsThe changes include recommendations for treatment targets, use of glucocorticoids and calcineurin inhibitors (CNIs) and management of end-stage kidney disease (ESKD). The target of therapy is complete response (proteinuria <0.5–0.7 g/24 hours with (near-)normal glomerular filtration rate) by 12 months, but this can be extended in patients with baseline nephrotic-range proteinuria. Hydroxychloroquine is recommended with regular ophthalmological monitoring. In active proliferative LN, initial (induction) treatment with mycophenolate mofetil (MMF 2–3 g/day or mycophenolic acid (MPA) at equivalent dose) or low-dose intravenous cyclophosphamide (CY; 500 mg × 6 biweekly doses), both combined with glucocorticoids (pulses of intravenous methylprednisolone, then oral prednisone 0.3–0.5 mg/kg/day) is recommended. MMF/CNI (especially tacrolimus) combination and high-dose CY are alternatives, for patients with nephrotic-range proteinuria and adverse prognostic factors. Subsequent long-term maintenance treatment with MMF or azathioprine should follow, with no or low-dose (<7.5 mg/day) glucocorticoids. The choice of agent depends on the initial regimen and plans for pregnancy. In non-responding disease, switch of induction regimens or rituximab are recommended. In pure membranous LN with nephrotic-range proteinuria or proteinuria >1 g/24 hours despite renin–angiotensin–aldosterone blockade, MMF in combination with glucocorticoids is preferred. Assessment for kidney and extra-renal disease activity, and management of comorbidities is lifelong with repeat kidney biopsy in cases of incomplete response or nephritic flares. In ESKD, transplantation is the preferred kidney replacement option with immunosuppression guided by transplant protocols and/or extra-renal manifestations. Treatment of LN in children follows the same principles as adult disease.ConclusionsWe have updated the EULAR recommendations for the management of LN to facilitate homogenization of patient care.

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Section: Recommendationmentioning

confidence: 99%

2019 Update of the Joint European League Against Rheumatism and European Renal Association–European Dialysis and Transplant Association (EULAR/ERA–EDTA) recommendations for the management of lupus nephritis

et al. 2020

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“…The classification criteria for SLE by the American College of Rheumatology (ACR) (2) and more recently by the Systemic Lupus International Collaborating Clinics (SLICC) (3)-both developed | 79 for research purposes (3,4)-recognize this clinical and laboratory heterogeneity. Low levels of serum complement protein (C3 and C4) are included in the SLICC criteria as well as the classification criteria newly developed by the European League Against Rheumatism (EULAR) and the ACR (5), due to the relatively high specificity of complement activation leading to low serum complement in SLE (6).…”

Section: Introductionmentioning

confidence: 99%

Complement Activation in Patients With Probable Systemic Lupus Erythematosus and Ability to Predict Progression to American College of Rheumatology–Classified Systemic Lupus Erythematosus

Ramsey‐Goldman

Alexander²,

Massarotti

et al. 2019

Arthritis & Rheumatology

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Objective To evaluate the frequency of cell‐bound complement activation products (CB‐CAPs) as a marker of complement activation in patients with suspected systemic lupus erythematosus (SLE) and the usefulness of this biomarker as a predictor of the evolution of probable SLE into SLE as classified by the American College of Rheumatology (ACR) criteria. Methods Patients in whom SLE was suspected by lupus experts and who fulfilled 3 ACR classification criteria for SLE (probable SLE) were enrolled, along with patients with established SLE as classified by both the ACR and the Systemic Lupus International Collaborating Clinics (SLICC) criteria, patients with primary Sjögren's syndrome (SS), and patients with other rheumatic diseases. Individual CB‐CAPs were measured by flow cytometry, and positivity rates were compared to those of commonly assessed biomarkers, including serum complement proteins (C3 and C4) and autoantibodies. The frequency of a positive multianalyte assay panel (MAP), which includes CB‐CAPs, was also evaluated. Probable SLE cases were followed up prospectively. Results The 92 patients with probable SLE were diagnosed more recently than the 53 patients with established SLE, and their use of antirheumatic medications was lower. At the enrollment visit, more patients with probable SLE were positive for CB‐CAPs (28%) or MAP (40%) than had low complement levels (9%) (P = 0.0001 for each). In probable SLE, MAP scores of >0.8 at enrollment predicted fulfillment of a fourth ACR criterion within 18 months (hazard ratio 3.11, P < 0.01). Conclusion Complement activation occurs in some patients with probable SLE and can be detected with higher frequency by evaluating CB‐CAPs and MAP than by assessing traditional serum complement protein levels. A MAP score above 0.8 predicts transition to classifiable SLE according to ACR criteria.

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“…More recently, lupus-related autoantibodies (≥ 1 immunological criterion needed) were ascribed EDITOR'S CHOICE increased importance in the 2012 Systemic Lupus International Collaborating Clinics (SLICC) criteria set, where the presence of ANA and/or antibodies to doublestranded (ds) DNA, combined with biopsy-proven lupus nephritis, was enough to classify as SLE [6]. In the 2019 SLE classification criteria from the European League Against Rheumatism (EULAR) and ACR, the presence of IF-ANA has a pivotal role and serves as an entry criterion with a stipulated titre of ≥ 1 : 80 [7,8].…”

Section: Introductionmentioning

confidence: 99%

Longitudinal anti-nuclear antibody (ANA) seroconversion in systemic lupus erythematosus: a prospective study of Swedish cases with recent-onset disease

Frodlund

Wetterö

Dahle

et al. 2019

Clinical &Amp; Experimental Immunology

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Summary Serum immunoglobulin (Ig)G anti‐nuclear antibodies (ANA) detected by indirect immunofluorescence (IF) microscopy remains a hallmark of systemic lupus erythematosus (SLE). Whether or not IF‐ANA status varies over time is controversial. We therefore designed a prospective study with longitudinal follow‐up of patients with recent‐onset SLE. The study population consisted of 54 recently diagnosed SLE cases, all meeting the 1982 American College of Rheumatology (ACR) and/or the 2012 Systemic Lupus International Collaborating Clinics (SLICC) criteria. Clinical follow‐up data, including disease activity, organ damage and sera, were collected from clinical onset of SLE and onwards, in most cases yearly (0‒96 months). IF‐ANA was analysed on human epithelial cells‐2 (HEp‐2) cells and categorized regarding staining patterns. Using an addressable laser bead assay (FIDIS™ Connective profile), we measured IgG‐ANA fine specificities against Ro52/SSA, Ro60/SSA, Sjögren’s syndrome type B antigen (La/SSB), Smith antigen (Sm), Smith antigen/ribonucleoprotein (Sm/RNP), U1 RNP (U1RNP), dsDNA, ribosomal‐P protein and histone. At baseline, all patients were judged ANA‐positive at an abnormal titre corresponding to the 95th percentile of healthy blood donors, but seven of 54 patients (13%) lost ANA‐positivity over time. Homogeneous (AC‐1; 46%) and speckled (AC‐4 or 5; 31%) were the most frequently observed patterns at inclusion, whereas 7% switched pattern at least once during follow‐up. Established associations between ANA fine specificities and clinical data were confirmed. Levels of anti‐Sm/RNP, but not of anti‐dsDNA, correlated with clinical disease activity [modified SLE disease activity 2000 (mSLEDAI‐2K)]. Our data indicate that a considerable proportion of Swedish patients with SLE lose ANA‐positivity over time, whereas consistent staining patterns were frequent. The clinical and mechanistic relevance of ANA seroconversion remains uncertain. Further prospective evaluations in larger SLE populations with more diverse ethnicities are warranted.

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2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus

Cited by 1,006 publications

References 32 publications

2019 Update of the Joint European League Against Rheumatism and European Renal Association–European Dialysis and Transplant Association (EULAR/ERA–EDTA) recommendations for the management of lupus nephritis

2019 Update of the Joint European League Against Rheumatism and European Renal Association–European Dialysis and Transplant Association (EULAR/ERA–EDTA) recommendations for the management of lupus nephritis

Complement Activation in Patients With Probable Systemic Lupus Erythematosus and Ability to Predict Progression to American College of Rheumatology–Classified Systemic Lupus Erythematosus

Longitudinal anti-nuclear antibody (ANA) seroconversion in systemic lupus erythematosus: a prospective study of Swedish cases with recent-onset disease

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