The Presentation of some cases with “Systemic Lupus Erythematosus” Iraj Salehi-Abari The Presentation of some cases with “Systemic Lupus Erythematosus” Iraj Salehi-Abari MD., Internist Rheumatologist salehiabari@sina.tums.ac.ir SLE

In the name of God the merciful and compassionate

Iraj Salehi-Abari Case I: A 30 year-old woman one month after coming back from seaside has: Malar rash Frank non-scaring alopecia Oral superficial painless ulcer Normal Lab. Data y SLE

Case II: A 40 year-old woman with: Palpable purpura in legs Iraj Salehi-Abari Case II: A 40 year-old woman with: Palpable purpura in legs WBC: 2500/mm3, L: 20%, Hb: 10, Ret: 12%, Plt: 110,000 FANA; 1/640 (up to 1/40), rim pattern Skin Bx: Leukocytoclastic vasculitis y SLE

Case III: A 27 year-old woman hospitalized with: Iraj Salehi-Abari Case III: A 27 year-old woman hospitalized with: Generalized pitting edema Frank alopecia Proteinuria: 550 mg/24 hr, urine casts Positive high titer Anti-dsDNA Disagreement with renal Bx. y SLE

Case IV: A 39 year-old woman with: Malar rash, Good general condition Iraj Salehi-Abari Case IV: A 39 year-old woman with: Malar rash, Good general condition History of two episodes of convulsion Bilateral pleural effusion Hb: 11, MCV: 80, Ret: 1.3%, ESR: 80 No other abnormal Lab. data y SLE

Case V: A 20 year-old woman with: Frank alopecia Iraj Salehi-Abari Case V: A 20 year-old woman with: Frank alopecia Polyarthralgia/myalgia Positive FH of SLE in her mother High titer ANA and Anti-dsDNA positivity y SLE

Diagnosis: History & Physical examination Laboratory tests: CXR-PA y Iraj Salehi-Abari Diagnosis: History & Physical examination Laboratory tests: CBC, serum Cr, U/A, ESR, CRP FANA Anti-dsDNA, Anti-Sm, VDRL, LE, LA, aCL, Anti-B2GPI, C3/C4/CH50 CXR-PA y SLE

Diagnosis: Using a questionnaire: “SLE Questionnaire” Iraj Salehi-Abari Diagnosis: Using a questionnaire: “SLE Questionnaire” Diagnosis of SLE can be established by “clinical judgment “of an expert Rheumatologist There are two Classification criteria: ACR , SLICC The only Diagnostic criteria: 2015 ACR/SLICC revised Criteria for Diagnosis of SLE y SLE

Diagnosis: 1997 “ACR” classification criteria for SLE Iraj Salehi-Abari Diagnosis: 1997 “ACR” classification criteria for SLE 2012 “SLICC” classification criteria for SLE Above classification criteria have been developed for SLE as a means of categorizing patients for study purposes 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 y SLE

“SLICC” Classification criteria: Iraj Salehi-Abari “SLICC” Classification criteria: With 4 criteria out of 17 including at least one criteria from each group: definite SLE Lupus nephritis in biopsy along with positive ANA or anti-dsDNA : definite SLE y SLE

Iraj Salehi-Abari Case I: A 30 year-old woman one month after coming back from seaside has: Malar rash Frank non-scaring alopecia Oral superficial painless ulcer Normal Lab. Data ACR: No SLE SLICC: No SLE But upon clinical judgment: SLE 2015 ACR/SLICC revised criteria: SLE y SLE

Case II: A 40 year-old woman with: Palpable purpura in legs Iraj Salehi-Abari Case II: A 40 year-old woman with: Palpable purpura in legs WBC: 2500/mm3, L: 20%, Hb: 10, Ret: 12%, Plt: 110,000 FANA; 1/640 (up to 1/40), rim pattern Skin Bx: Leukocytoclastic vasculitis ACR: No SLE SLICC: No SLE But upon clinical judgment: SLE 2015 ACR/SLICC revised criteria: SLE y SLE

Case III: A 27 year-old woman hospitalized with: Iraj Salehi-Abari Case III: A 27 year-old woman hospitalized with: Generalized pitting edema Frank alopecia Proteinuria: 550 mg/24 hr, urine casts Positive high titer Anti-dsDNA Disagreement with renal Bx. ACR: No SLE SLICC: No SLE But upon clinical judgment: SLE 2015 ACR/SLICC revised criteria: SLE y SLE

Case IV: A 39 year-old woman with: Malar rash, Good general condition Iraj Salehi-Abari Case IV: A 39 year-old woman with: Malar rash, Good general condition History of two episodes of convulsion Bilateral pleural effusion Hb: 11, MCV: 80, Ret: 1.3%, ESR: 80 No other abnormal Lab. data ACR: No SLE SLICC: No SLE But upon clinical judgment: SLE 2015 ACR/SLICC revised criteria: SLE y SLE

Case V: A 20 year-old woman with: Frank alopecia Arthralgia/myalgia Iraj Salehi-Abari Case V: A 20 year-old woman with: Frank alopecia Arthralgia/myalgia Positive FH of SLE in her mother High titer ANA and Anti-dsDNA positivity ACR: No SLE SLICC: No SLE But upon clinical judgment: SLE 2015 ACR/SLICC revised criteria: SLE y SLE

Iraj Salehi-Abari Diagnosis: Right now in the absence of existing Diagnostic criteria for SLE, the general approach to the diagnosis of SLE is: Step I: fulfilling the 1997 ACR classification criteria Step II: fulfilling the 2012 SLICC classification criteria Step III: There are significant percent of the patients who do not fulfill the above classification criteria So, we need an accurate diagnostic criteria for early diagnosis and right now the main way of diagnosis is “clinical judgment” y SLE