1. “Systemic Lupus Erythematosus” Lab Data, Diagnosis and Management
Iraj Salehi-Abari
“Systemic Lupus Erythematosus” Lab Data, Diagnosis and Management
Iraj Salehi-Abari MD.,
Internist
Rheumatologist
SLE

2. Definition: Chronic disease Systemic/Multi-organ disease
Iraj Salehi-Abari
Definition:
Chronic disease
Systemic/Multi-organ disease
Inflammatory/Autoimmune disease
Autoantibody/Immune Complex
Skin, Joints, Kidneys, Lungs, Nervous system, Serous membranes,… y
SLE

3. Laboratory tests: Essential non-immunologic tests:
Iraj Salehi-Abari
Laboratory tests:
Essential non-immunologic tests:
CBC / diff / Plt / Ret
BUN/Cr, U/A, U24 Pr
AST/ALT, CK, serum Alb.
FBS, Lipid profile, Ca/P/Alk-Ph
ESR/CRP
PT/PTT /INR
SLE

4. Laboratory tests: Essential immunologic tests: ANA Anti-dsDNA/anti-Sm
Iraj Salehi-Abari
Laboratory tests:
Essential immunologic tests:
ANA
Anti-dsDNA/anti-Sm
aPL: aCl, LA, anti-B2 GPI
VDRL/TPI, LE cell (?)
C3/C4/CH50%
Commbs test
SLE

5. Laboratory tests: Other tests: Anti-RO (SS-A) /anti-La (SS-B)
Iraj Salehi-Abari
Laboratory tests:
Other tests:
Anti-RO (SS-A) /anti-La (SS-B)
Anti-u1-RNP, anti-Histone
RF/anti-CCP
ECG
TFT; T3/T4, freeT4 index, T3RP, TSH,
anti TPO
SLE

6. Anti-nuclear antibody (ANA):
Iraj Salehi-Abari
Anti-nuclear antibody (ANA):
The most sensitive antibody
Positive in 95-99% of cases
FANA:
Pattern: diffuse (homogen), Rim (peripheral) ++
>1/80
SLE

7. Anti-nuclear antibody (ANA):
Iraj Salehi-Abari
Anti-nuclear antibody (ANA):
FANA negative Lupus: anti-Ro/anti-La
Positive ANA: SLE, Sjogren’s, RA, JRA, Scl, MCTD, Vasculitis, DM/PM, IPF,IM,Lepra, Lupoid hepatitis, Aging, Drugs,…
SLE

8. Other antibodies: Anti-dsDNA: most specific Anti-Sm: most specific
Iraj Salehi-Abari
Other antibodies:
Anti-dsDNA: most specific
Anti-Sm: most specific
Anti-Histone: Drug-induced SLE
aPL (aCL, LA, anti-B2 GPI): APS
SLE

9. Other antibodies: Anti-u1RNP: MCTD
Iraj Salehi-Abari
Other antibodies:
Anti-u1RNP: MCTD
Anti-Ro/anti-La: Sjogren’s, SCLE, FANA negative SLE, Older SLE, Neonatal lupus, Congenital Heart Block, hereditary C deficiency
SLE

10. Imaging: Chest radiography (CXR-PA) Plain-X ray of involved joints
Iraj Salehi-Abari
Imaging:
Chest radiography (CXR-PA)
Plain-X ray of involved joints
Renal ultrasonography
Echocardiography
Abdominal CT
HRCT of lungs, DLCO
Brain MRI
SLE

11. Histology: Renal biopsy Skin biopsy: Lupus band Lymph node biopsy
Iraj Salehi-Abari
Histology:
Renal biopsy
Skin biopsy: Lupus band
Lymph node biopsy
Myocardial biopsy y
SLE

12. Diagnosis: “SLE Questionnaire”
Iraj Salehi-Abari
Diagnosis:
“SLE Questionnaire”
SLE can be established by “clinical/laboratory judgement “of an expert Rheumatologist
“1997 ACR classification criteria”
“2012 SLICC classification criteria”
“ 2015 ACR/SLICC revised criteria for diagnosis of SLE “: is the best way y
SLE

13. SLE Questionnaire: Arthritis > 3 months Raynaud phenomenon
Iraj Salehi-Abari
SLE Questionnaire:
Arthritis > 3 months
Raynaud phenomenon
Oral ulcer > 2 weeks
Cytopenia
Rash on cheeks > 1 month
Photosensitivity
Pleurisy > a few days
Protein in urine
Rapid loss of hair
Convulsion
[with > 3 positive answers possibility of SLE] y
SLE

14. ACR Classification criteria:
Iraj Salehi-Abari
ACR Classification criteria:
Malar rash [with at least 4 criteria out of 11 SLE]
DLE
Photosensitivity
Oral Ulcer
Arthritis
Psychosis/Seizure
Pleurisy/pericarditis
Renal
Hematologic
Positive ANA
Positive anti-dsDNA or Anti-Sm or aPL y
SLE

15. “SLICC” Classification criteria:
Iraj Salehi-Abari
“SLICC” Classification criteria:
Clinical criteria
ACLE : Malar rash/photosensitivity/SCLE
CCLE: DLE
Nonscarring alopecia
Oral/nasal ulcer
Joint disease
Pleurisy/pericarditis
Renal
Neurologic
Hemolytic anemia
Leukopenia/lymphopenia
Thrombocytopenia y
SLE

16. “SLICC” Classification criteria:
Iraj Salehi-Abari
“SLICC” Classification criteria:
Immunologic criteria
ANA
Anti-dsDNA
Anti-Sm
aPL antibodies
Low complement
Direct Coomb’s test
11 clinical + 6 immunologic = 17 criteria
[With 4 criteria out of 17 including at least one criteria from each group  SLE]; Lupus nephritis in biopsy along with ANA or antidsDNA  SLE y
SLE

17. Management of SLE: Pharmacologic + non-pharmacologic: .Minor SLE:
Iraj Salehi-Abari
Management of SLE:
Pharmacologic + non-pharmacologic:
.Minor SLE:
[Antimalaric (HCQ) + low dose steroid + others]
.Major SLE:
[Cytotoxics + high dose steroid + Antimalaric + others]
Cytotoxics: Cyclophosphamide, Mycophenolate mofetile (CellCept), Azathioprine, Leflunomide, MTX, Cyclosporine,… yM
SLE

18. Management of SLE: Cytotoxics: Biologic:
Iraj Salehi-Abari
Management of SLE:
Cytotoxics:
Cyclophosphamide, Mycophenolate mofetile (CellCept), Azathioprine, Leflunomide, MTX, Cyclosporine,…
Biologic:
Rituximab, Belimumab yM
SLE

19. Management of SLE: .Major SLE:
Iraj Salehi-Abari
Management of SLE:
.Major SLE:
Lupus nephritis class IV (may be III or V)
CNS lupus
Severe Hemolytic anemia/thrombocytopenia
Myocarditis
Necrotizing vasculitis (PAN)
Fulminant acute hemorrhagic lupus pneumonitis
Polymyositis y
SLE

20. Management of SLE: .Disease activity indices:
Iraj Salehi-Abari
Management of SLE:
.Disease activity indices:
SLE Disease Activity Index: SLEDAI
Systemic Lupus Activity Measure: SLAM
British Isles Lupus Assessment Group: BILAG
European Consensus Lupus Activity Measurement: ECLAM
Revised SLAM: SLAM-R y
SLE

21. Management of SLE: Sun protection: SPF > 55
Iraj Salehi-Abari
Management of SLE:
.Non-pharmacologic:
Sun protection: SPF > 55
Diet/nutrition: balanced diet, fish oil
Exercise
Smoking cessation y
SLE

22. Management of SLE: Immunizations:
Iraj Salehi-Abari
Management of SLE:
Immunizations:
Influenza and pneumococcal vaccines can be used
No live vaccines: measles, mumps, rubella, polio, varicella and vaccinia in patients with > 20 mg/day of prednisone for > 2 weeks y
SLE

23. Iraj Salehi-Abari
Management of SLE:
Comorbid condition: APS, PAH, atherosclerosis, osteoporosis,…
Avoidance of specific medications y
SLE

24. SLE and Pregnancy: Effects of SLE on pregnancy
Iraj Salehi-Abari
SLE and Pregnancy:
Effects of SLE on pregnancy
Effects of pregnancy on SLE
Flare: 50%, more better: 25%, No change: 25%
SLE + APS
Neonatal lupus y
SLE

25. Outcome and prognosis:
Iraj Salehi-Abari
Outcome and prognosis:
The 5 year survival rate:
Mid-20th century: 40%
Nowadays: > 90%
Causes of death:
Active SLE: CNS, Renal, Cardiovascular
Infection
Non-Hodgkin lymphoma, Lung cancer y
SLE

26. Poor prognostic factors:
Iraj Salehi-Abari
Poor prognostic factors:
Renal disease; class IV
HTN
Male sex
Young age
Older at presentation
Low socioeconomic status
Black race
APS and aPLs
Major SLE and high disease activity y
SLE