Kenneth C. Kalunian, MD, Maureen A. McMahon, MD, and Joan T. Merrill, MD, prepared useful practice aids pertaining to systemic lupus erythematosus for this CME activity titled "Candid Conversations in Lupus: Navigating Advances in Diagnosis and Treatment to Provide Optimal Care for Each Patient." For the full presentation, monograph, complete CME information, and to apply for credit, please visit us at https://bit.ly/2zki5EQ. CME credit will be available until September 14, 2021.

1. Access the activity, “Candid Conversations in Lupus: Navigating Advances
in Diagnosis and Treatment to Provide Optimal Care for Each Patient,”
at PeerView.com/YHV40
Systemic Lupus Erythematosus
Classification Criteria Comparison
PRACTICE AID
1. Tan EM et al. Arthritis Rheum. 1982; 25:1271-1277. 2. Hochberg MC. Arthritis Rheum. 1997; 40:1725. 3. Petri M et al. Arthritis Rheum. 2012; 64:2677-2686. 4. Aringer M et al. Ann Rheum Dis. 2019;78:1151.
ACR 19971,2
Satisfy 4 of 11 criteria
SLICC 20123
Satisfy 4 of the criteria with at least 1 clinical criterion
and 1 immunologic criterion
OR
biopsy-proven LN with positive ANA
or anti-ds antibodies
ACR/EULAR 20194
At least one clinical criterion and 10 points
Criterion Points
Cutaneous
1. Malar rash
2. Photosensitivity
3. Discoid rash
4. Oral/nasopharyngeal ulceration
1. Acute cutaneous lupus (including malar and
photosensitive lupus rash) OR subacute cutaneous
lupus
Acute cutaneous lupus 6
2. Chronic cutaneous lupus
3. Oral or nasal ulcers
Subacute cutaneous lupus OR discoid lupus
Oral ulcers
4
2
4. Nonscarring alopecia Nonscarring alopecia 2
Joints
5. Nonerosive arthritis
(tenderness, swelling, or effusion
with ≥2 peripheral joints)
5. Synovitis (swelling or effusion with 2 joints
OR tenderness in 2 joints with 30 minutes of
morning stiffness)
Joint involvement 6
Serositis
6A. Pleuritis OR
6B. Pericarditis
6. Serositis (pleurisy, pleural effusions,
pleural rub, pericardial pain, pericardial effusion,
pericardial rub, pericarditis)
Pleural OR pericardial effusion 5
Acute pericarditis 6
Renal
7A. Persistent proteinuria
(>0.5 mg/24 h or >3+ dipstick) OR
7B. Cellular casts
7A. Urine protein-to-creatinine ratio (or 24-h urine
protein) >0.5 g/24 h OR
7B. Red blood cell casts
Proteinuria (>0.5 mg/24 h) 4
Renal biopsy Class II or V LN 8
Class III or IV LN 10
Neurologic
8A. Seizures OR
8B. Psychosis
8A. Seizures OR
8B. Psychosis OR
8C. Mononeuritis complex OR
8D. Myelitis OR
8E. Peripheral/cranial neuropathy OR
8F. Acute confusional state
Seizure 5
Psychosis 3
Delirium 2
Hematologic
9A. Hemolytic anemia OR
9B. Leukopenia (<4,000/mm3 on
2 occasions) OR
9C. Lymphophenia (<1,500 mm3
on 2 occasions) OR
9D. Thrombocytopenia
(<100,000/mm3
)
9. Hemolytic anemia Autoimmune hemolysis 4
10A. Leukopenia (<4,000/mm3) at least once OR
10B. Lymphopenia (<1,000/mm3) at least once
Leukopenia 3
11. Thrombocytopenia (<100,000/m3 at least once) Thrombocytopenia 4
Constitutional Fever 2
Immunologic
10A. Anti-DNA OR
10B. Anti-Smith OR
10C. Positive antiphospholipid
antibody
• Abnormal IgG/IgM
anticardiolipin antibodies
• Lupus anticoagulant
• False positive/positive syphilis
for 6 months
12. Anti-ds DNA Anti-ds DNA antibody OR
anti-Smith antibody
6
13. Anti-Smith antibody
14. Positive antiphospholipid antibody
• Lupus anticoagulant
• False positive for rapid plasma regain
• Medium-/high-titer IgA/IgG/IgM anticardiolipin
antibody level
• Positive anti- 2GP1
Antiphospholipid antibodies
• Anticardiolipin antibodies OR
• Anti- 2GP1 antibodies OR
• Lupus anticoagulant
2
15. Low complement
• C3, C4, or CH50
Low C3 OR low C4 3
16. Direct Coombs test Low C3 AND low C4 4
11. Positive ANA 17. Positive ANA Requires ANA at a titer of 1:80 on HEp-2 cells or an equivalent
positive test (ever)

2. Access the activity, “Candid Conversations in Lupus: Navigating Advances
in Diagnosis and Treatment to Provide Optimal Care for Each Patient,”
at PeerView.com/YHV40
Considerations in the Treatment
of Systemic Lupus Erythematosus
PRACTICE AID
Treatment Goals1,2
Ensure long-term survival
Achieve the lowest-possible disease activity
Prevent organ damage
Minimize drug toxicity
Improve quality of life
Educate patients about their role in disease management
Medicines Commonly Used to Treat Lupus3
Class Example(s) Comments
Corticosteroids
Prednisone,
prednisolone,
methylprednisolone,
and hydrocortisone
• Steroid dosage should be kept at lowest level possible; once lupus symptoms
respond to treatment, dose is gradually reduced
• Adverse effects of glucocorticoids are both dose- and duration-dependent; adverse
effects range from those that are not necessarily serious but are displeasing to
patients (eg, Cushingoid appearance) to those that are life-threatening (eg, serious
infections); some adverse effects, such as accelerated reductions in bone mineral
density or early cataracts, may be largely asymptomatic until later manifestations
develop that require medical attention (eg, acute vertebral collapse, cataract
requiring surgical extraction)
Antimalarials
Hydroxychloroquine
and chloroquine
• In general, all patients with SLE with any degree and type of disease activity should
be treated with hydroxychloroquine or chloroquine, unless these agents are
contraindicated; there is a tendency to avoid the use of chloroquine because
of increased risk of retinal toxicity
• Benefits of hydroxychloroquine or chloroquine in SLE are broad and include relief
of constitutional symptoms, fatigue, musculoskeletal manifestations, and
mucocutaneous manifestations; as an add-on to certain immunosuppressives,
it is thought that antimalarials may have additive benefits in more serious disease,
and they are also known to be helpful in anticoagulation
• Side effects from antimalarials are rare and usually mild and include upset stomach
and changes in skin color; these typically go away after body adjusts to the
medication; the possibility of retinal toxicity warrants clinician adherence to dosing
and screening recommendations
Biologics Belimumab, rituximab
• Belimumab, approved in 2011, was the first treatment approved for SLE in over
60 years
• Belimumab has been demonstrated to be effective in treatment of global lupus
disease activity and lupus nephritis; it has also been shown to reduce autoantibodies
Immunosuppressives
Cyclophosphamide,
azathioprine,
mycophenolate mofetil,
cyclosporine,
tacrolimus, leflunomide,
and methotrexate
• Used to control more serious lupus activity that affects major organs, including the
kidney, brain, cardiovascular system, and lungs
• May be given in addition to, or instead of, steroid therapy to lower the dose of steroids
needed and thus spare some of the undesirable side effects of steroid therapy
• Hydroxychloroquine has also been shown to be beneficial in decreasing the risk of
accelerated atherosclerosis, preeclampsia, and lupus nephritis
• Rituximab, a B-cell–depleting chimeric monoclonal antibody, may be useful in
patients with severely low platelet counts, hemolytic anemia, and refractory nephritis

3. Access the activity, “Candid Conversations in Lupus: Navigating Advances
in Diagnosis and Treatment to Provide Optimal Care for Each Patient,”
at PeerView.com/YHV40
Considerations in the Treatment
of Systemic Lupus Erythematosus
PRACTICE AID
a
Mild: constitutional symptoms/mild arthritis/rash ≤9% BSA/PLTs 50-100 x 103
/mm3
; SLEDAI ≤6; BILAG C or ≤1 BILAG B manifestations. b
Moderate: RA-like arthritis/rash 9%-18% BSA/cutaneous
vasculitis ≤18% BSA; PLTs 20-50 x 103
/mm3
/serositis; SLEDAI 7-12; ≥2 BILAG B manifestations. c
Severe: major organ-threatening disease (nephritis, cerebritis, myelitis, pneumonitis, mesenteric vasculitis;
thrombocytopenia with platelets <20 x 103
/mm3
; TTP-like disease or acute hemophagocytic syndrome; SLEDAI >12; ≥1 BILAG A manifestations.
1. van Vollenhoven RF et al. Ann Rheum Dis. 2014;73:958-967. 2. Bertsias G et al. Ann Rheum Dis. 2008;67:195-205. 3. https://www.lupus.org/resources/medications-used-to-treat-lupus.
4. Fanouriakis A et al. Ann Rheum Dis. 2019;78:736-745.
Treatment of Nonrenal SLE: EULAR Recommendations4
Sun protection
Vaccinations
Exercise
No smoking
Body weight
Blood pressure
Lipids
Glucose
Antiplatelet
anti-coagulants
(in aPL-positive
patients)
Remission
SLEDAI = 0
HCQ
No GC
OR
Low disease
activity
SLEDAI 4
HCQ
Pre 7.5 mg/d
Immunosuppressives
(in stable doses and
well tolerated)
Adjunct Target
HCQ
GC PO/IM GC PO/IV
MTX/AZA
BEL
CYC
RTX
Milda
First
line
Refractory
Moderateb
First
line
Refractory
Severec
First
line
Refractory
CNI
MMF
Grade A Grade B Grade C Grade D
Given the clinical heterogeneity of SLE and the unpredictable disease course,
the therapeutic approach is highly variable and is generally guided by the
predominant disease manifestations.
In general, all patients with SLE with any degree and type of disease activity should
be treated with an antimalarial (eg, hydroxychloroquine); additional therapy is
based upon the severity of disease and the combination of manifestations.

4. Access the activity, “Candid Conversations in Lupus: Navigating Advances
in Diagnosis and Treatment to Provide Optimal Care for Each Patient,”
at PeerView.com/YHV40
Management of Reproductive Health in SLE:
2020 Recommendations and Good Practice
Statements from the ACR1
PRACTICE AID
a
Recommendation for IUD use includes women receiving immunosuppression therapy.
1. Sammaritano LR et al. Arthritis Care Res (Hoboken). 2020;72:461-488.
Use of Contraception
SLE: Moderate-high disease activity
IUDsa, progestin implant, DMPA, or
progestin-only pill over combined
estrogen–progestin contraceptives
AVOID estrogen patch
SLE: Low disease activity
IUDsa, progestin implant, combined estrogen
and progesterone pill, progestin-only pill (less
effective), vaginal ring, or DMPA
IUDs or progestin implant preferred over other
hormonal contraceptives
AVOID estrogen patch
Special Circumstances
• Use of mycophenolate medications requires an IUD or combination of two other forms of contraception
• Avoid DMPA in patients at risk for osteoporosis
Strongly recommend
Conditionally recommend
Conditionally recommend against
• Discuss contraception and pregnancy planning at initial or early visit with women of reproductive age and
counsel regarding efficacy and safety
• Recommend barrier methods if more effective methods are contraindicated
• Recommend emergency (post-coital) contraception when necessary
Pregnancy Counseling, Assessment, and Management
• Counsel RMD patients regarding improved maternal and pregnancy outcomes when disease is
quiescent/low activity before pregnancy
• Comanagement with rheumatology and other specialists preferred
Assess patients considering pregnancy
High disease activity
Treat to control disease
activity and reassess
when quiescent/low
disease activity
Low disease activity
Change to
pregnancy-compatible
medications and
observe for efficacy
and tolerance
Assess patients beginning early in pregnancy
SLE
Continue HCQ
(if on)
Start HCQ
(if not on and no
contraindication)
Low-dose aspirin
Laboratory
assessment of
disease activity at
least once per
trimester
Strongly recommend
Conditionally recommend
Conditionally recommend against