1. Dr. Meg-angela Christi Amores
Rheumatology: SLE
Dr. Meg-angela Christi Amores

2. Systemic Lupus Erythematosus (SLE)
autoimmune disease in which organs and cells undergo damage mediated by tissue-binding autoantibodies and immune complexes
90% are women in childbearing years
all ethnic groups are susceptible

3. Pathogenesis
Interactions between susceptibility genes and environmental factors result in abnormal immune responses
(1) activation of innate immunity
(2) lowered activation thresholds of adaptive immunity cells
(3) ineffective regulatory and inhibitory CD4+ and CD8+ T cells
(4) reduced clearance of apoptotic cells and of immune complexes

4. result in abnormal immune responses
generate pathogenic autoantibodies and immune complexes that deposit in tissue, activate complement
cause inflammation
lead to irreversible organ damage

5. Pathogenesis
The result of these abnormalities is sustained production of pathogenic autoantibodies :
ANA (antinuclear antibodies)
Anti-dsDNA
Anti-Sm
Anti-RNP
Anti-Ro
Anti-La
Antihistone

6. Pathogenesis SLE is a multigenic disease
In most genetically susceptible individuals, normal alleles of multiple normal genes each contribute a small amount to abnormal immune responses
if enough variations accumulate, disease results

7. Pathogenesis Female sex is permissive for SLE
make higher antibody responses than males
Women exposed to estrogen-containing oral contraceptives or hormone replacement have an increased risk of developing SLE
environmental stimuli may influence SLE :
Exposure to ultraviolet light
some infections

8. Diagnosis based on characteristic clinical features and autoantibodies
Most patients experience exacerbations interspersed with periods of relative quiescence
Antinuclear antibodies (ANA) are positive in >98% of patients during the course of disease; repeated negative tests suggest that the diagnosis is not SLE

9. diagnosis Mnemonics: SOAP BRAIN MD
Serositis
Oral ulcers
Arthritis
Photosensitivity
Blood (all are low, anemia, etc)
Renal (protein, nephritis)
ANA
Immunologic (ds DNA)
Neurologic (seizures)
Malar rash
Discoid rash
> 4 out of 11 suggests SLE is highly likely

10. Manifestations Arthritis – polyarthritis
soft tissue swelling and tenderness in joints, most commonly in hands, wrists, and knees
Myositis with clinical muscle weakness

11. Manifestations Dermatitis
Discoid rash - roughly circular with slightly raised, scaly hyperpigmented erythematous rims and depigmented, atrophic centers
Malar rash - photosensitive, slightly raised erythema, occasionally scaly, on the face (particularly the cheeks and nose—the "butterfly" rash), ears, chin, V region of the neck, upper back, and extensor surfaces of the arms

12. Manifestations Neprhritis Nervous system manifestations
most serious manifestation of SLE
leading causes of mortality in the first decade of disease
Nervous system manifestations
cognitive dysfunction, including difficulties with memory and reasoning
seizures

13. Manifestations Vascular occlusions
Pleuritis with or without pleural effusion
Pericarditis
Anemia, Leukopenia, Lymphopenia, Thrombocytopenia

14. Treatment There is no cure
physician should plan to control acute, severe flares and then develop maintenance strategies

15. Treatment Conservative strategies: (for non-life threatening cases)
suppression of symptoms
Analgesics and antimalarials
NSAIDs are useful analgesics/anti-inflammatories, particularly for arthritis/arthralgias
Antimalarials (hydroxychloroquine, chloroquine, and quinacrine) often reduce dermatitis, arthritis, and fatigue

16. Treatment Life-threatening: systemic glucocorticoids
methylprednisolone sodium succinate
Prednisone
doses are tapered as rapidly as the clinical situation permits
Cytotoxic/immunosuppressive agents added to glucocorticoids