2. SLE (systemic lupus erythematosus) or often known as a disease with a thousand
faces, is an autoimmune rheumatic disease that requires special attention, starting
from recognizing its clinical appearance to its management
Definition
3. Diagnosis SLE
• There are 2 SLE classification criteria that can be used 🡪 ACR 1997 and SLICC 2012
• ACR 1997 🡪 consists of 11 clinical and laboratory criteria. Patients are classified as SLE if they fulfill 4 of 11
criteria
• SLICC 2012 🡪 consists of 17 criteria. Patients are classified as SLE if they fulfill 4 of 17 criteria with at least 1
clinical criteria and 1 immunological criteria
• SLICC 2012 classification has a higher sensitivity and lower specificity than ACR 1997 classification
• EULAR/ACR 2018 🡪 this classification can be used if the ANA-IF titer is positive > 1:80 and there is no other
possible cause other than SLE (patients are included in SLE if the total score is > 10 with at least one clinical
criteria)
Introduction to SLE Patients
5. Organ involvement in the SLE
Introduction to SLE Patients
Constitutional symptoms
•Fever, fatigue, lymphadenopathy, malaise, and decreased appetite and weight
Musculosceletal symptoms
•Arthralgia, erosive arthritis, nonerosive arthritis, Jaccoud's arthropathy, muscle
weakness, myalgia, myositis, osteoporosis, rotator cuff syndrome, and tenosynovitis
Dermatology and mucosal symptoms
•Malar rash, discoid lesions, photosensitivity, oral ulcers, alopecia, subacute
cutaneous lupus erythematosus, profundus lupus
6. Organ involvement in the SLE
Introduction to SLE Patients
Renal symptoms
•Lupus nephritis
Neuropsychiatry symptoms
•Chorea, cognitive dysfunction, seizures, transverse myelitis, peripheral
neuropathy, headache, organic brain syndrome, depression, mood disorder and
psychosis
Cardiology symptoms
•Pericarditis, myocarditis, cardiomyopathy, heart valve disease, and abnormal
conduction system
7. Organ involvement in the SLE
Introduction to SLE Patients
Vascular symptoms
• Raynaud phenomenon, livedo reticularis, livedo racemosa, and vasculitis
Gastrointestinal and Hepatic symptoms
• Gastrointestinal 🡪 pharyngitis, dysphagia, esophagitis, anorexia, nausea, vomiting, diarrhea, acute abdominal
pain, peptic ulcer, IBD, protein-losing gastroenteropathy, malabsorption, ascites, peritonitis, pancreatitis,
mesenteric vasculitis, melena, gastrointestinal bleeding, gastrointestinal infarction, disorders motility, celiac
disease, intestinal cystoides pneumotosis and eosinophilic enteritis
• Hepatic 🡪 liver enzyme disorders, hepatomegaly, jaundice, hepatic arteritis, hepatitis lupus, and autoimmune
hepatitis
Ocular symptoms
• Episcleritis, keratitis, cyclic keratoconjunctivitis, orbital inflammation, dry eye, optic neuropathy, retinopathy
due to antiphospholipid antibodies, scleritis, uveitis, retinal vasculitis with cotton-wool spots and chloroquine-
induced toxicity
8. Organ involvement in the SLE
Introduction to SLE Patients
Obstetric symptoms
• SC birth, premature birth, LBW, small neonate for gestational age, spontaneous
abortion and stillbirth, preeclampsia
Endocrine symptoms
• Hyperprolactinaemia, vitamin D deficiency, and cushing syndrome
Hematologic symptoms
• Iron deficiency anemia, anemia of chronic disease, autoimmune hemolytic anemia,
drug-induced myelotoxicity and anemia due to chronic renal failure, thrombocytopenia,
platelet disorders, leukopenia, lymphopenia, increased erythrocyte sedimentation rate
9. SLE disease activity
• SLE disease activity 🡪 SLEDAI and MEX-SLEDAI
• SLEDAI 🡪 consists of 24 clinical and laboratory variables covering nine organ systems. Each variable has
an operational definition and a score of 1-8
• SLEDAI scores were grouped into no activity (score 0), mild disease activity (score 1-5), moderate disease
activity (score 6-10), severe disease activity (score 11-19), very severe disease activity (score > 20)
• SLE disease activity:
1. Mild SLE 🡪 SLEDAI <6 or MEX-SLEDAI 2-5
2. Moderate SLE 🡪 SLEDAI 6-12 or MEX-SLEDAI 6-9
3. Severe SLE 🡪 SLEDAI >12 or MEX-SLEDAI >10
Introduction to SLE Patients
12. Other Examination
Complemen
examination (C3 and
C4)
Vitamin D
examination
Bone mineral
density (BMD)
HIV, HBV and HCV
screening test
Sputum
examination
Chest X-ray
Electrocardiography
(ECG)
18. Treatment in SLE
Therapy targets SLE
• The target of SLE therapy is to achieve remission (SLEDAI 0 or MEX-SLEDAI 0-1) or minimal disease activity
(SLEDAI 1-5 or MEX SLEDAI 2-5).
• Another criteria that can be used to assess mild disease activity targets is the LLDAS with the following
criteria:
1. SLEDAI-2K score <4 without SLEDAI activity in major organ systems and gastrointestinal activity
2. No new manifestations of lupus disease activity were found compared to previous examinations
3. Physician Global Assessment (PGA) score <1 (scale 0-3)
4. Last dose of prednisolone <7.5 mg/day or equivalent
5. Immunosuppressant therapy and/or biologic agents at standard maintenance doses were well
tolerated, excluding investigational drugs
