slide1
Download
Skip this Video
Download Presentation
SYSTEMIC LUPUS ERYTHEMATOSUS

Loading in 2 Seconds...

play fullscreen
1 / 45

SYSTEMIC LUPUS ERYTHEMATOSUS - PowerPoint PPT Presentation


  • 591 Views
  • Uploaded on

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

loader
I am the owner, or an agent authorized to act on behalf of the owner, of the copyrighted work described.
capcha
Download Presentation

PowerPoint Slideshow about 'SYSTEMIC LUPUS ERYTHEMATOSUS' - heller


An Image/Link below is provided (as is) to download presentation

Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author.While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server.


- - - - - - - - - - - - - - - - - - - - - - - - - - E N D - - - - - - - - - - - - - - - - - - - - - - - - - -
Presentation Transcript
outline
OUTLINE
  • Definition
  • Epidemiology
  • Pathophysiology
  • Classification and diagnosis
  • Clinical Features
  • Lupus related syndromes
  • Treatment
  • Prognosis
definition
DEFINITION
  • Inflammatory autoimmune disorder affecting multiple organ systems characterized by the production of autoantibodies directed against cell nuclei”
epidemiology
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
etiology
Etiology
  • Genetic (HLA DR3 association)
    • Abnormal immune response
  • Environmental
    • UV
    • Viruses
    • Hormones (Estrogen)
clinical features mucocutaneous
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
malar rash
MALAR RASH
  • Fixed erythema, flat or raised, over the malar eminences
  • Tending to spare the nasolabial folds
discoid rash
DISCOID RASH
  • Erythematous raised patches with adherent keratotic scaling and follicular plugging;
  • Atrophic scarring may occur in older lesions
slide14

Acute Cutaneous: Malar Rash

Note Sparing of Nasolabial Folds

Chronic Cutaneous:Discoid

Note Scarring, Hyperpigmentation

oral ulcers
ORAL ULCERS
  • Oral or nasopharyngeal ulceration
  • Usually painless, observed by a physician
slide17

SLE - VASCULOPATHY

  • Small vessel vasculitis
  • Raynaud’s phenomenon
  • Antiphospholipid antibody syndrome
clinical features musculoskeletal
CLINICAL FEATURES: Musculoskeletal
  • Arthritis is NONEROSIVE, transient, symmetrical, affecting small joints, seldom deforming, less severe than RA
  • Most common presenting feature of SLE
clinical features musculoskeletal20
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
clinical features ocular
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
clinical features pleuropulmonar
CLINICAL FEATURES: PLEUROPULMONAR
  • Pleuritis/Pleural effusion
  • Infiltrates/ Discoid Atelectasis
  • Acute lupus pneumonitis
  • Pulmonary hemorrhage
  • “Shrinking lung” - diaphragm dysfunction
  • Restrictive lung disease
clinical features cardiac
CLINICAL FEATURES: Cardiac
  • Pericarditis –in majority of patients
  • Libman Sacks endocarditis
  • Cardiac failure
  • Cardiac Arrythmias-common
  • Valvular heart disease
  • Coronary Artery Disease
lupus endocarditis
Lupus - Endocarditis

Noninfective thrombotic endocarditis involving mitral valve in SLE.

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

clinical features hematologic disorder
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

clinical features neurologic
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
clinical features renal lupus nephritis
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.

clinical features renal lupus nephritis29
CLINICAL FEATURES: Renal (Lupus Nephritis)
  • Usually asymptomatic
  • Gross hematuria
  • Nephrotic syndrome
  • Acute renal failure
  • Hypertension
  • End stage renal failure
who classification of lupus nephritis
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

clinical features gastrointestinal hepatic
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
laboratory findings
Laboratory Findings
  • Complete blood count
    • Anemia
    • Leukopenia
    • Lymphopenia
    • Thrombocytopenia
  • Urine Analysis
    • Hematuria
    • Proteinuria
    • Granular casts
immunological findings
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
classification
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

classification criteria
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
differential diagnosis
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)
lupus related syndromes
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
lupus related syndromes38
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
lupus related syndromes39
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

sle treatment i
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
sle treatment ii
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
treatment
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
treatment44
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
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
ad