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Sle
Sle
Sle
OUTLINE
Definition Epidemiology Pathophysiology Classification and diagnosis Clinical Features Lupus related syndromes Treatment Prognosis
DEFINITION
Inflammatory autoimmune disorder affecting multiple organ systems characterized by the production of autoantibodies directed against cell nuclei
EPIDEMIOLOGY
Prevalence influenced by age, gender, race, and genetics
Prevalence: 1:2000 Peak incidence 14-45 years Black > White (1:250 vs. 1:1000) Female predominance 10:1 HLA DR3 association, Family History
Etiology
Genetic (HLA DR3 association)
Abnormal immune response
Environmental
UV Viruses Hormones (Estrogen)
PATHOPHYSIOLOGY
Vasculitic ulceration Oral ulceration Nasal septal perforation Nailfold capillary changes
MALAR RASH
Fixed erythema, flat or raised, over the malar eminences Tending to spare the nasolabial folds
DISCOID RASH
Erythematous raised patches with adherent keratotic scaling and follicular plugging; Atrophic scarring may occur in older lesions
Alopecia
Follicular Plugging
Livedo Reticularis
ACR
ORAL ULCERS
SLE - VASCULOPATHY
Small vessel vasculitis Raynauds phenomenon Antiphospholipid antibody syndrome
Synovitis-90% patients, often the earliest sign Osteoporosis From SLE itself and therapy (usually steroids) Osteonecrosis (avascular necrosis) Can occur with & without history of steroid therapy
Lupus - Endocarditis
Noninfective thrombotic endocarditis involving mitral valve in SLE. Note nodular vegetations along line of closure and extending onto chordae tendineae.
Develops in up to 50% of patients 10% SLE patients go to dialysis or transplant Hallmark clinical finding is proteinuria Advancing renal failure complicates assessment of SLE disease activity
Usually asymptomatic Gross hematuria Nephrotic syndrome Acute renal failure Hypertension End stage renal failure
Uncommon SLE manifestations Severe abdominal pain syndromes in SLE often indicate mesenteric vasculitis, resembling medium vessel vasculitis (PAN) Diverticulitis may be masked by steroids Hepatic abnormalities more often due to therapy than to SLE itself
Laboratory Findings
Complete blood count
Anemia Leukopenia Lymphopenia Thrombocytopenia
Urine Analysis
Hematuria Proteinuria Granular casts
Immunological findings
ANA - 95-100%-sensitive but not specific for SLE Anti -ds DNA-specific(60%)-specific for SLE, but positive to other non lupus conditions 4 RNA associated antibodies
Anti-Sm (Smith) Anti Ro/SSA-antibody Anti La/SSB-antibody Anti-RNP Biologic false + RPR Lupus anticoagulant-antibodies tocoagulation factors. risk factor for venous and arterial thrombosis and miscarriage. Prolonged aPTT Anti-cardiolipin
Antiphospholipid antibody
CLASSIFICATION
THE 1982 REVISED CRITERIA FOR CLASSIFICATION OF SLE
1. Malar rash 2. Discoid rash 3. Photosensitivity 4. Oral ulcers 5. Arthritis 6. Serositis 7. Renal disease. > 0.5 g/d proteinuria 3+ dipstick proteinuria Cellular casts 8. Neurologic disease. Seizures Psychosis (without other cause)
9. Hematologic disorders. Hemolytic anemia Leukopenia (< 4000/uL) Lymphopenia (< 1500/uL) Thrombocytopenia (< 100,000/uL) 10. Immunologic abnormalities. Positive LE cell Anti-ds- DNA Anti- Sm Any antiphospholipid 11. Positive ANA ( 95-100% )
CLASSIFICATION CRITERIA
Must have 4 of 11 for Classification
Sensitivity 75% Specificity 96%
DIFFERENTIAL DIAGNOSIS
Almost too broad to consider given number of clinical manifestations Rheumatic: RA, Sjogrens syndrome, systemic sclerosis, dermatomyositis Nonrheumatic: HIV, endocarditis, viral infections, hematologic malignancies, vasculitis, ITP, other causes of nephritis Overlap Syndrome (UCTD, MCTD)
Raynauds Syndrome: -Not part of the diagnostic criteria for SLE - Does NOT warrant ANA if no other clinical evidence to suggest autoimmune disease
SLE treatment I.
Mild cases (mild skin or joint involvement): NSAID, local treatment, hydroxy-chloroquin Cases of intermediate severity (serositis, cytopenia, marked skin or joint involvement): corticosteroid (12-64 mg methylprednisolon), azathioprin, methotrexat
TREATMENT
Antiphospholipid Syndrome
Anticoagulation with warfarin (teratogenic) subcutaneous heparin and aspirin is usual approach in pregnancy
TREATMENT
ESR, CRP probably useful as general markers of disease activity Complement and anti-DNA antibodies may correlate to disease activity but often impractical turnaround time from lab Patient history
PROGNOSIS
Unpredictable course 10 year survival rates exceed 85% Most SLE patients die from infection, probably related to therapy which suppresses immune system Recommend smoking cessation, yearly flu shots, pneumovax q5years, and preventive cancer screening recommendations