“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 salehiabari@sina.tums.ac.ir SLE

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

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

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

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

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

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

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

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

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

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

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

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

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

“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

“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

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

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

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

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

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

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

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

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

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

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