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Diagnostic Approach for Suspected SLE1
Full abbreviations, accreditation, and disclosure information available at PeerView.com/TSR40
Suspected SLE
Test for ANA by immunofluorescence
Apply the EULAR/ACR 2019 criteria using the attribution algorithm
(best combination of sensitivity and specificity)
+
Classified SLE
Clinical SLEa
Clinical SLEa
Immunologic score = 0
+
Clinical score ≥8
+
ACR-97 photosensitivity
OR
See next page for
more info on clinical
domains
ANA +ve ANA -ve
EULAR/ACR score <10
EULAR/ACR score ≥10 with at
least one clinical criterion
Hypocomplementemia
and/or positive aPL
(alternate entry criterion)
EULAR/ACR score ≥10 with at least
one clinical criterion
Immunologic score ≥2
+
Clinical score ≥6
Diagnostic Approach for Suspected SLE1
Full abbreviations, accreditation, and disclosure information available at PeerView.com/TSR40
a
Also consider noncriteria features (eg, Raynaud phenomenon, myocarditis, atypical rashes).
1. Fanouriakis A et al. Ann Rheum Dis. 2021;80:14-25.
Clinical Domains Weight Clinical Domains Weight
Fever 2 2
6
6
4
8
10
2
3
4
4
2
3
5
2
2
4
6
Pleural or pericardial effusion
Hematologic
Neuropsychiatric
Mucocutaneous
Acute pericarditis
Leukopenia
Thrombocytopenia
Autoimmune hemolysis
Delirium
Psychosis
Seizure
Nonscarring alopecia
3
Complement proteins
SLE-specific antibodies
Low C3 OR low C4
4
6
Low C3 AND low C4
Anti-dsDNA OR anti-Sm antibody
Oral ulcers
SCLE or DLE
ACLE
Clinical score: 0-39
Joint involvement
Proteinuria >0.5 g/24 h
Renal biopsy class II or V LN
Renal biopsy class III or IV LN
Musculoskeletal
Renal
Anticardiolipin antibodies OR
Anti-β2GP1 antibodies OR
Lupus anticoagulant
Immunologic score: 0-2
Antiphospholipid antibiodies
Constitutional
Immunology Domains Weight Weight
Serosal
Immunology Domains
Within each domain, only the highest weight criterion is counted
EULAR Treatment Recommendations for SLE1
Full abbreviations, accreditation, and disclosure information available at PeerView.com/TSR40
1. Fanouriakis A et al. Ann Rheum Dis. 2021;80:14-25.
First line
Refractory
HCG
HCG
GC PO/IM
GC PO/IM MTX/AZA
Mild
First line
Refractory
HCG
HCG
GC PO/IM
GC PO/IM
MTX/AZA
BEL
CNI
CNI MMF
MMF
Moderate
First line
Refractory
HCG
HCG
GC PO/IM
GC PO/IM
MMF
CYC
CYC
RTX
Severe
• Sun protection
• Vaccinations
• Exercises
• No smoking
• Body weight
• Blood pressure
• Lipids
• Glucose
• Antiplatelets
Adjunct
Target
Remission Low-Disease Activity
SLEDAI = 0
HCQ; no GC
SLEDAI: ≤4; HCQ and prednisone ≤7.5 mg/d
Immunosuppressives, in stable doses and well tolerated
• Anticoagulants
(in aPL-positive
patients)
KDIGO 2021 Guidelines for Management of LN1
Full abbreviations, accreditation, and disclosure information available at PeerView.com/TSR40
Kidney biopsy showing class III/IV ± LN
Concomitant thrombotic
microangiopathy
Assess activity and chronicity items
Calcineurin inhibitors
• Tacrolimus (trough level approximately
5.5 ng/mL [6.8 nmol/L], data mainly
from Chinese patients) and reduced-
dose MPAA in patients with sCr <3
mg/dL (265 mcmol/L) as initial and
maintenance therapy for 24 months
• Voclosporin 23.7 mg BID and MPAA in
patients with eGFR >45 mL/min/1.73 m2
for 52 weeks
B-lymphocyte–targeting biologics
• Belimumab (IV 10 mg/kg Q2W for
3 doses then Q4W) and MPAA or IV
cyclophosphamide 500 mg Q2W x 6
• Rituximab IV 1 g on days 1 and 15,
as add-on therapy for refractory
cases or for corticosteroids
minimization
MPAA for at least
6 months
• MMF PO 1.0-1.5 g BID
or mycophenolic acid
sodium 0.72-1.08 g BID
Cyclophosphamide for
up to 6 months
• IV 500 mg Q2W x 6 or
0.5-1.0 g/m2
monthly x 6;
or PO 1.0-1.5 mg/kg/d
Active class III/IV ± LN
Corticosteroids
Methylprednisolone IV 0.25-0.50 g/d for 1 to 3 days, then prednisone PO 0.6-1.0 mg/kg/d
(not to exceed 80 mg/d) and taper over a few months to maintenance dose
Chronic class III/IV ± V LN
without activity
Supportive treatment
for CKD
If concomitant class V,
manage as class V
1. https://www.kidney-international.org/article/S0085-2538(21)00562-7/fulltext
And

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How I Think, How I Treat—Assessing, Managing, and Engaging Patients to Optimize Care in SLE and LN: Comparing Approaches With the Experts

  • 1. Diagnostic Approach for Suspected SLE1 Full abbreviations, accreditation, and disclosure information available at PeerView.com/TSR40 Suspected SLE Test for ANA by immunofluorescence Apply the EULAR/ACR 2019 criteria using the attribution algorithm (best combination of sensitivity and specificity) + Classified SLE Clinical SLEa Clinical SLEa Immunologic score = 0 + Clinical score ≥8 + ACR-97 photosensitivity OR See next page for more info on clinical domains ANA +ve ANA -ve EULAR/ACR score <10 EULAR/ACR score ≥10 with at least one clinical criterion Hypocomplementemia and/or positive aPL (alternate entry criterion) EULAR/ACR score ≥10 with at least one clinical criterion Immunologic score ≥2 + Clinical score ≥6
  • 2. Diagnostic Approach for Suspected SLE1 Full abbreviations, accreditation, and disclosure information available at PeerView.com/TSR40 a Also consider noncriteria features (eg, Raynaud phenomenon, myocarditis, atypical rashes). 1. Fanouriakis A et al. Ann Rheum Dis. 2021;80:14-25. Clinical Domains Weight Clinical Domains Weight Fever 2 2 6 6 4 8 10 2 3 4 4 2 3 5 2 2 4 6 Pleural or pericardial effusion Hematologic Neuropsychiatric Mucocutaneous Acute pericarditis Leukopenia Thrombocytopenia Autoimmune hemolysis Delirium Psychosis Seizure Nonscarring alopecia 3 Complement proteins SLE-specific antibodies Low C3 OR low C4 4 6 Low C3 AND low C4 Anti-dsDNA OR anti-Sm antibody Oral ulcers SCLE or DLE ACLE Clinical score: 0-39 Joint involvement Proteinuria >0.5 g/24 h Renal biopsy class II or V LN Renal biopsy class III or IV LN Musculoskeletal Renal Anticardiolipin antibodies OR Anti-β2GP1 antibodies OR Lupus anticoagulant Immunologic score: 0-2 Antiphospholipid antibiodies Constitutional Immunology Domains Weight Weight Serosal Immunology Domains Within each domain, only the highest weight criterion is counted
  • 3. EULAR Treatment Recommendations for SLE1 Full abbreviations, accreditation, and disclosure information available at PeerView.com/TSR40 1. Fanouriakis A et al. Ann Rheum Dis. 2021;80:14-25. First line Refractory HCG HCG GC PO/IM GC PO/IM MTX/AZA Mild First line Refractory HCG HCG GC PO/IM GC PO/IM MTX/AZA BEL CNI CNI MMF MMF Moderate First line Refractory HCG HCG GC PO/IM GC PO/IM MMF CYC CYC RTX Severe • Sun protection • Vaccinations • Exercises • No smoking • Body weight • Blood pressure • Lipids • Glucose • Antiplatelets Adjunct Target Remission Low-Disease Activity SLEDAI = 0 HCQ; no GC SLEDAI: ≤4; HCQ and prednisone ≤7.5 mg/d Immunosuppressives, in stable doses and well tolerated • Anticoagulants (in aPL-positive patients)
  • 4. KDIGO 2021 Guidelines for Management of LN1 Full abbreviations, accreditation, and disclosure information available at PeerView.com/TSR40 Kidney biopsy showing class III/IV ± LN Concomitant thrombotic microangiopathy Assess activity and chronicity items Calcineurin inhibitors • Tacrolimus (trough level approximately 5.5 ng/mL [6.8 nmol/L], data mainly from Chinese patients) and reduced- dose MPAA in patients with sCr <3 mg/dL (265 mcmol/L) as initial and maintenance therapy for 24 months • Voclosporin 23.7 mg BID and MPAA in patients with eGFR >45 mL/min/1.73 m2 for 52 weeks B-lymphocyte–targeting biologics • Belimumab (IV 10 mg/kg Q2W for 3 doses then Q4W) and MPAA or IV cyclophosphamide 500 mg Q2W x 6 • Rituximab IV 1 g on days 1 and 15, as add-on therapy for refractory cases or for corticosteroids minimization MPAA for at least 6 months • MMF PO 1.0-1.5 g BID or mycophenolic acid sodium 0.72-1.08 g BID Cyclophosphamide for up to 6 months • IV 500 mg Q2W x 6 or 0.5-1.0 g/m2 monthly x 6; or PO 1.0-1.5 mg/kg/d Active class III/IV ± LN Corticosteroids Methylprednisolone IV 0.25-0.50 g/d for 1 to 3 days, then prednisone PO 0.6-1.0 mg/kg/d (not to exceed 80 mg/d) and taper over a few months to maintenance dose Chronic class III/IV ± V LN without activity Supportive treatment for CKD If concomitant class V, manage as class V 1. https://www.kidney-international.org/article/S0085-2538(21)00562-7/fulltext And