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SYSTEMIC LUPUS ERYTHEMATOSUS

SYSTEMIC LUPUS ERYTHEMATOSUS. OUTLINE. Definition Epidemiology Pathophysiology Classification and diagnosis Clinical Features Lupus related syndromes Treatment Prognosis . DEFINITION.

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SYSTEMIC LUPUS ERYTHEMATOSUS

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  1. SYSTEMIC LUPUS ERYTHEMATOSUS

  2. OUTLINE • Definition • Epidemiology • Pathophysiology • Classification and diagnosis • Clinical Features • Lupus related syndromes • Treatment • Prognosis

  3. DEFINITION • Inflammatory autoimmune disorder affecting multiple organ systems characterized by the production of autoantibodies directed against cell nuclei”

  4. 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 • Severity is equal in male and female

  5. Etiology • Genetic (HLA DR3 association) • Abnormal immune response • Environmental • UV • Viruses • Hormones (Estrogen)

  6. PATHOPHYSIOLOGY

  7. Malar Rash (butterfly erythema) Discoid rash Photosensitive rash Subacute cutaneous LE Livedo reticularis Alopecia Raynaud’s Vasculitic ulceration Oral ulceration Nasal septal perforation Nailfold capillary changes CLINICAL FEATURES: Mucocutaneous

  8. MALAR RASH • Fixed erythema, flat or raised, over the malar eminences • Tending to spare the nasolabial folds

  9. DISCOID RASH • Erythematous raised patches with adherent keratotic scaling and follicular plugging; • Atrophic scarring may occur in older lesions

  10. Alopecia

  11. Subacute Cutaneous Lupus

  12. Acute Cutaneous: Malar Rash Note Sparing of Nasolabial Folds Chronic Cutaneous:Discoid Note Scarring, Hyperpigmentation

  13. Follicular Plugging Livedo Reticularis ACR

  14. ORAL ULCERS • Oral or nasopharyngeal ulceration • Usually painless, observed by a physician

  15. SLE - VASCULOPATHY • Small vessel vasculitis • Raynaud’s phenomenon • Antiphospholipid antibody syndrome

  16. CLINICAL FEATURES: Musculoskeletal • Arthritis is NONEROSIVE, transient, symmetrical, affecting small joints, seldom deforming, less severe than RA • Most common presenting feature of SLE

  17. Jaccoud’s Arthopathy: Nonerosive, Reducible Deformities

  18. CLINICAL FEATURES: Musculoskeletal • 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

  19. CLINICAL FEATURES: Ocular • Conjunctivitis • Photophobia • Monocular blindness-transient or permanent • Blurred vision • Cotton-Wool spots on retina-degeneration nerves fibers due to occlusion retinal blood vessels

  20. CLINICAL FEATURES: PLEUROPULMONAR • Pleuritis/Pleural effusion • Infiltrates/ Discoid Atelectasis • Acute lupus pneumonitis • Pulmonary hemorrhage • “Shrinking lung” - diaphragm dysfunction • Restrictive lung disease

  21. CLINICAL FEATURES: Cardiac • Pericarditis –in majority of patients • Libman Sacks endocarditis • Cardiac failure • Cardiac Arrythmias-common • Valvular heart disease • Coronary Artery Disease

  22. Lupus - Endocarditis Noninfective thrombotic endocarditis involving mitral valve in SLE. Note nodular vegetations along line of closure and extending onto chordae tendineae.

  23. CLINICAL FEATURES: HEMATOLOGIC DISORDER A) Hemolytic anemia - with reticulocytosis OR B) Leukopenia - less than 4,000/mm3 total on 2 or more occasions OR C) Lymphopenia - less than 1,500/mm3 on 2 or more occasions OR D) Thrombocytopenia - less than 100,000/mm3 in the absence of offending drugs

  24. CLINICAL FEATURES: Neurologic • Behavior/Personality changes, depression • Cognitive dysfunction • Psychosis • Seizures • Stroke • Chorea • Pseudotumor cerebri • Transverse myelitis • Peripheral neuropathy • Total of 19 manifestations described • May be difficult to distinguish from steroid psychosis or primary psychiatric disease

  25. CLINICAL FEATURES: Renal (Lupus Nephritis) • 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 Nephritis remains the most frequent cause of disease-related death.

  26. CLINICAL FEATURES: Renal (Lupus Nephritis) • Usually asymptomatic • Gross hematuria • Nephrotic syndrome • Acute renal failure • Hypertension • End stage renal failure

  27. WHO CLASSIFICATION OF LUPUS NEPHRITIS Class I Normal Class II Mesangial IIA Minimal alteration IIB Mesangial glomerulitis Class III Focal and segmental proliferative glomerulonephritis Class IV Diffuse proliferative glomerulonephritis Class V Membranous glomerulonephritis Class VI Glomerular sclerosis

  28. CLINICAL FEATURES: Gastrointestinal & Hepatic • 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

  29. Laboratory Findings • Complete blood count • Anemia • Leukopenia • Lymphopenia • Thrombocytopenia • Urine Analysis • Hematuria • Proteinuria • Granular casts

  30. 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 • Antiphospholipid antibody • Biologic false + RPR • Lupus anticoagulant-antibodies tocoagulation factors. risk factor for venous and arterial thrombosis and miscarriage. Prolonged aPTT • Anti-cardiolipin • Depressed serum complement • Anti hystones antibodies

  31. 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 THE 1982 REVISED CRITERIA FOR CLASSIFICATION OF SLE

  32. CLASSIFICATION CRITERIA • Must have 4 of 11 for Classification • Sensitivity 96% • Specificity 96% • Like RA, diagnosis is ultimately clinical • Not all “Lupus” is SLE • Discoid Lupus • Overlap syndrome • Drug induced lupus • Subacute Cutaneous Lupus

  33. DIFFERENTIAL DIAGNOSIS • Almost too broad to consider given number of clinical manifestations • Rheumatic: RA, Sjogren’s syndrome, systemic sclerosis, dermatomyositis • Nonrheumatic: HIV, endocarditis, viral infections, hematologic malignancies, vasculitis, ITP, other causes of nephritis • “Overlap Syndrome” (UCTD, MCTD)

  34. LUPUS RELATED SYNDROMES • Drug Induced Lupus • Classically associated with hydralazine, isoniazid, procainamide • Male:Female ratio is equal • Nephritis and CNS abnormalities rare • Normal complement and no anti-DNA antibodies • Symptoms usually resolve with stopping drug

  35. LUPUS RELATED SYNDROMES • Antiphospholipid Syndrome (APS) • Hypercoagulability with recurrent thrombosis of either venous or arterial circulation • Thrombocytopenia-common • Pregnancy complication-miscarriage in first trimester • Lifelong anticoagulation warfarin is currently recommended for patients with serious complications due to common recurrence of thrombosis • Antiphospholipid Antibodies • Primary when present without other SLE feature. • Secondary when usual SLE features present

  36. LUPUS RELATED SYNDROMES Raynaud’s Syndrome: -Not part of the diagnostic criteria for SLE - Does NOT warrant ANA if no other clinical evidence to suggest autoimmune disease

  37. 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

  38. SLE – treatment II. • Severe, life-threatening organ involvements (carditis, nephritis, systemic vasculitis, cerebral manifestations): high-dose intravenous corticosteroid + iv. cyclophosphamide + in some cases: plasmapheresis or iv. immunoglobulin, or, instead of cyclophosphamide: mycophenolate mofetil (not registered in the EU) • Some cases of nephritis (especially membranous), myositis, thrombocytopenia: cyclosporine

  39. TREATMENT • Antiphospholipid Syndrome • Anticoagulation with warfarin (teratogenic) • subcutaneous heparin and aspirin is usual approach in pregnancy • Lupus and Pregnancy • No longer “contraindicated” • No changes in therapy other than avoiding fetal toxic drugs • Complications related to renal failure, antiphospholipid antibodies, SSA/SSB

  40. 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

  41. 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

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