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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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SYSTEMIC LUPUS ERYTHEMATOSUS


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OUTLINE

  • Definition

  • Epidemiology

  • Pathophysiology

  • Classification and diagnosis

  • Clinical Features

  • Lupus related syndromes

  • Treatment

  • Prognosis


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DEFINITION

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


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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


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Etiology

  • Genetic (HLA DR3 association)

    • Abnormal immune response

  • Environmental

    • UV

    • Viruses

    • Hormones (Estrogen)


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PATHOPHYSIOLOGY


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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


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MALAR RASH

  • Fixed erythema, flat or raised, over the malar eminences

  • Tending to spare the nasolabial folds


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DISCOID RASH

  • Erythematous raised patches with adherent keratotic scaling and follicular plugging;

  • Atrophic scarring may occur in older lesions


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Alopecia


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Subacute Cutaneous Lupus


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Acute Cutaneous: Malar Rash

Note Sparing of Nasolabial Folds

Chronic Cutaneous:Discoid

Note Scarring, Hyperpigmentation


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Follicular Plugging Livedo Reticularis

ACR


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ORAL ULCERS

  • Oral or nasopharyngeal ulceration

  • Usually painless, observed by a physician


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SLE - VASCULOPATHY

  • Small vessel vasculitis

  • Raynaud’s phenomenon

  • Antiphospholipid antibody syndrome


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CLINICAL FEATURES: Musculoskeletal

  • Arthritis is NONEROSIVE, transient, symmetrical, affecting small joints, seldom deforming, less severe than RA

  • Most common presenting feature of SLE


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Jaccoud’s Arthopathy: Nonerosive, Reducible Deformities


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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


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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


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CLINICAL FEATURES: PLEUROPULMONAR

  • Pleuritis/Pleural effusion

  • Infiltrates/ Discoid Atelectasis

  • Acute lupus pneumonitis

  • Pulmonary hemorrhage

  • “Shrinking lung” - diaphragm dysfunction

  • Restrictive lung disease


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CLINICAL FEATURES: Cardiac

  • Pericarditis –in majority of patients

  • Libman Sacks endocarditis

  • Cardiac failure

  • Cardiac Arrythmias-common

  • Valvular heart disease

  • Coronary Artery Disease


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Lupus - Endocarditis

Noninfective thrombotic endocarditis involving mitral valve in SLE.

Note nodular vegetations along line of closure and extending onto chordae tendineae.


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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


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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


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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.


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CLINICAL FEATURES: Renal (Lupus Nephritis)

  • Usually asymptomatic

  • Gross hematuria

  • Nephrotic syndrome

  • Acute renal failure

  • Hypertension

  • End stage renal failure


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WHO CLASSIFICATION OF LUPUS NEPHRITIS

Class INormal

Class IIMesangial

IIAMinimal alteration

IIBMesangial glomerulitis

Class IIIFocal and segmental proliferative glomerulonephritis

Class IVDiffuse proliferative glomerulonephritis

Class VMembranous glomerulonephritis

Class VIGlomerular sclerosis


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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


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Laboratory Findings

  • Complete blood count

    • Anemia

    • Leukopenia

    • Lymphopenia

    • Thrombocytopenia

  • Urine Analysis

    • Hematuria

    • Proteinuria

    • Granular casts


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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


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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


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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


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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)


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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


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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


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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


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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


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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


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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


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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


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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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