Systemic
Lupus
Erythematosu
s (SLE)
Yasmin Mahmoud
moustafa
Rofaida Ahmed
hasanain
GM21_02
Introduction to SLE
Definition:
Chronic, systemic autoimmune disorder
characterized by the production of autoantibodies
targeting multiple organ systems.
Epidemiology:
Affects 20–150 per 100,000 individuals globally.
10:1 female-to-male ratio, highest prevalence
among women of childbearing age.
Ethnic predisposition: Higher prevalence in
African, Hispanic, and Asian populations.
Etiology and Risk Factors
Environmental
Genetic
Adenovirus Factors:
is the UV radiation can trigger
Triggers:
leading cause in flares.
[Link]-DR2 and Infections (e.g., Epstein-
HLA-DR3 are strongly Barr virus) may initiate
associated. autoimmunity.
Family studies show a Medications like
5–12% prevalence hydralazine, procainamide,
among first-degree and isoniazid.
relatives. Hormonal
Influence:
Estrogen promotes B-cell
activation, while androgens
are protective.
Pathophysi
ology
Immune Dysregulation:
Overactive B cells produce autoantibodies (e.g., anti-
dsDNA, anti-Sm).
Immune Complex Formation:
Autoantibodies bind to nuclear antigens, forming
complexes deposited in tissues.
Inflammatory Cascade:
Complement activation leads to tissue damage.
Cytokine Imbalance:
Increased levels of IL-6, IL-10, and interferon-alpha (IFN-α).
Clinical Features
Systemic Symptoms:
Fatigue (most common),
fever (low-grade), and
unintentional weight loss.
Multi-Organ Involvement:
Dermatologic,
musculoskeletal, renal,
cardiopulmonary,
neurological, and
hematological systems.
Skin Manifestations Hematological Features
Malar Rash:
Butterfly-shaped rash over
Anemia: Normocytic,
the cheeks and nasal bridge,
normochromic (chronic
sparing the nasolabial folds.
disease).
Discoid Lupus:
Leukopenia: Low WBC count
Erythematous, scaly plaques
(<4,000/mm³).
leading to scarring.
Thrombocytopenia: Platelet
Photosensitivity:
count <100,000/mm³.
Exacerbation of lesions or
Antiphospholipid
rash with sun exposure.
Syndrome (APS): Risk of
Alopecia:
arterial and venous
Diffuse or localized hair loss.
thrombosis.
Musculoskeletal Symptoms
Arthritis:
Symmetrical, non-erosive polyarthritis.
Affects small joints of the hands, wrists, and knees.
Myalgia: Muscle pain without significant weakness.
Deformities: Jaccoud’s arthropathy (reversible joint
deformities).
Renal Involvement
Lupus Nephritis:
Occurs in up to 50% of patients.
Manifestations: Edema, hematuria, proteinuria, and hypertension.
ISN/RPS Classification:
Classes I–VI based on severity (I: minimal mesangial, VI: advanced
sclerotic).
Diagnosis: Renal biopsy is gold standard.
Cardiopulmonary
Manifestations
Cardiac:
Pericarditis: Chest pain relieved by leaning forward.
Myocarditis: Rare but severe.
Libman-Sacks Endocarditis: Sterile vegetations on heart valves.
Pulmonary:
Pleuritis, interstitial lung disease, pulmonary hypertension.
Neurological Symptoms
Central Nervous System (CNS):
Seizures (6–10%), psychosis (5%), cerebrovascular accidents.
Peripheral Nervous System (PNS):
Neuropathy, Guillain-Barré syndrome.
Cognitive dysfunction (lupus fog).
Diagnostic Criteria
2019 ACR/EULAR
Criteria:
ANA positivity is
mandatory.
Clinical domains:
Constitutional,
cutaneous,
musculoskeletal, renal,
neurological,
hematological.
Immunological domains:
Anti-dsDNA, anti-Sm,
low C3/C4.
Differential Diagnosis
Distinguishing SLE from:
Rheumatoid Arthritis: Erosive arthritis,
absence of ANA.
Sjögren’s Syndrome: Dominated by sicca
symptoms.
Dermatomyositis: Proximal muscle weakness
and Gottron’s papules.
Systemic Sclerosis: Skin tightening and
Raynaud’s phenomenon.
Lifestyle Modifications:
Avoid UV light, Overview
Management manage stress, maintain
vaccination schedules.
Medications:
Hydroxychloroquine: Reduces flares
and improves survival.
Corticosteroids: For acute
exacerbations.
Immunosuppressants:
Cyclophosphamide for severe organ
involvement.
Biologics: Belimumab for refractory
cases.
Complications
Renal Failure: Due to advanced lupus nephritis.
Infections: Opportunistic infections due to immunosuppressive
therapy.
Cardiovascular Disease: Accelerated atherosclerosis.
Thrombosis: Arterial or venous due to APS.
Prognosis
Survival: 10-year survival exceeds 90% in developed
countries.
Prognostic Factors:
Early diagnosis and treatment.
Severity of organ involvement.
Socioeconomic and healthcare access.
Thanks
!