Diagnosis and treatment

Systemic Lupus
Erythematosus

DEFINITION OF
LUPUS

Lupus is an inflammatory auto

immune disorder that may affect
multiple organ systems
Its clinical manifestations are
secondary to the trapping of antigenantibody complex or to auto antibodymediated destruction of host cells

WHAT CAUSES
LUPUS?

Geneti
cs

Hormone
s

Environme
nt

ENVIRONMENTAL
TRIGGERS

Ultraviolet light
Stress
Medications
Infection
Foods

Chemical agents
Surgery
Vaccines
Hormones

LUPUS IS
Different for each person.
The great imitator
A disease that ranges from mild to
life threatening.
Characterized by flares and
remissions.

ORGAN
INVOLVEMENT
WITH LUPUS

Kidneys
Lungs
Central nervous
system

Blood vessels
Blood
Heart
Skin

1997 Update of the 1982 American College of

Rheumatology
classification criteria systemic lupus erythematosus

Criterion

Definiton

1. Malar
rash

Fixed erythema, flat or raised, over the

malar eminences, tending to spare the
nasolabial folds

2. Discoid
rash

Erythematous raised patches with adherent

keratotic scaling and follicular plugging :
atrophic scarring may occur in older lesions

3.

Photose
nsitivity

Skin rash as a result of unusual reaction to

sunlight, by patient history or physician
observation

Criterion Definiton

4. Oral
ulcers

Oral or nasopharyngeal ulceration, usually

painless, observed by a physician

5. Arthritis

Nonerosive arthritis involving 2

peripheral joints, characterized by
tenderness, swelling, or effusion

6. Serositis

(A) Pleuritis : convincing history of

pleuritic pain or rub heard by a physician
or evidence of leural effusion
Or
(B) Pericarditis : documented by ECH or
rub or evidence of pericardial effusion

Criterion Definiton

7. Renal
disorder

(A) Persistent proteinuria > 0.5 g/d or >

3+ if quantitation not performed
or
(B) Cellular casts: may be red blood cell,
hemoglobin, granular, tubular, or mixed

8.
(A) Seizures: in the absence of offending
Neurologi drugs or known metabolic derangements
c disorder (eg, uremia, ketoacidosis, or electrolyte
imbalance)
or
(B) Psychosis: in the absence of offending
drugs or known metabolic derangements
(eg, uremia, ketoacidosis, or electrolyte
imbalance)

1997 Update of the 1982 American College of Rheu

classification criteria systemic lupus erythematosu

Criterion

Definiton

(A) Hemolytic anemia: with

9.Hematolo reticulocytosis
or
gic
(B) Leukopenia: < 4000/mm total
disorder on 2
occasions
or
(C) Lymphopenia: < 1500/mm total
on 2
occasions

Criterion

10.
Immunologic
disorder

11.
Antinuclear
antibody

Definiton
(A) Positive LE cell preparation or
(B) Anti-DNA: antibody to native DNA in
abnormal titer or
(C) Anti-Sm: presence of antibody to Sm
nuclear antigen or
(D) False positive serologic test for
syphilis known to be positive for at least
6 months and confirmed by T pallidum
immobilization or fluorescent treponemal
antibody absorption test
An abnormal titer of anticuclear antibody
by immunofluorescence or an equivalent
assay at any poit in time and in the
absence of drugs known to be associated
with drug-induced lupus syndrome

(Hochberg, 1997; Gladman et al, 1999; Lockshin,

2001)

ACR classification criteria for LUPUS

(1997)
For the purpose of identifying patients in clinical
studies, a person has SLE if 4 or more of the 11
criteria are present, serially or simultaneously,
during any interval of observation.
It is important to remember that a patient may
have SLE and not have 4 criteria.

Malar Rash

Discoid
rash

Photosensitivity

Oral ulcer

Arthritis (with swelling)

SEBELUM PENGOBATAN

KESAN : WANITA 27 THN DGN VARIAN

LUPUS ERITEMATOSUS SISTEMIK
Gambar Setelah Terapi

Arthritis
-arthralgia
-symmetrical, non-erosive
synovitis
-Jacoubs arthropathy
(reducible deformities)
-Nodules possible

Cardiac manifestations
Pericarditis commonest
Myocarditis
Libman-Sacks endocarditis

Coronary artery disease (premature)

Renal Disorder

Proteinuria
(>500
mg/24hrs)

Cellular casts

Neurologic:
Psychosis

A) Seizure
B)

OTHER
LUPUS SYMPTOMS

Chest pain

Hair loss

Anemia

Vasculitis

Raynauds phenomenon

Headache
Dizziness
Depression
Seizures
Cognitive dysfunction
Memory disturbances

Alopecia areata

Vasculitis (livedo reticularis)

Vasculitic rashes

Raynauds
phenomenon

Livedo reticularis

Alopecia
diffuse or patchy

HISTOPATOLOGI
Folikular
plugging

Band
like

Epidermis
atrofi

Degenerasi
sel basal

Kelenja
r
sudorif
era Lupus Eritematosus
Sesuai gambaran
Sistemik

HASIL BIOPSI KULIT

GAMBARAN HISTOPATOLOGI PENDERITA

LUPUS BAND (+)

COMMON LABORATORY
TESTS

Antinuclear Antibody (ANA)

Anti DNA
Anti-Sm
Anti-RNP
Anti-Ro (SSA)
Anti-La (SSB)

ANA test
Anti-nuclear antibodies

Present in the sera of almost all

patients
ANA titres reflect disease activity
Specific antinuclear antibodies
anti-dsDNA, anti-Ro, anti-La, antiSm, anti-histone

LE Cell

The LE cell is a
neutrophil that has
engulfed the
antibody-coated
nucleus of another
neutrophil.
LE cells may appear
in rosettes where
there are several
neutrophils vying for
an individual
complement covered
protein.

Other autoantibodies

Anti-phospholipid antibody (1997

modification)

Anti-cardiolipin antibody (IgG or IgM)

Biologic false positive VDRL (> 6
months)
Lupus anticoagulant

Rheumatoid factor

Other laboratory test

Complement levels

C3 and C4 levels
Abnormally low in active SLE
Indicate immune activation

Renal involvement

Urinalysis
Serum Creatinine
Renal Biopsy

EFFECT OF LABORATORY TESTS

WITH INCREASED LUPUS
ACTIVITY
C reactive protein (CRP)
Sedimentation rate
(ESR)
Anti DNA
Liver and Kidney
Function tests
Urine protein or cell
casts

CBC (WBC, RBC,

platelets)
Complement
Serum albumin

Drug Therapy

Anti-inflammatory drugs
Corticosteroids
Anti-malarial drugs
Immunosuppressive RX
Symptomatic therapy?

Corticosteroids

For more active disease with arthritis,

pericarditis, pleuritis or serositis.
IV or orally
Maintenance in UK of oral enteric
coated pred.
Acute flares IV methylpred
(dont forget side effects,
osteoporosis, atherosclerosis)

Anti-malarial Drugs

Particularly effective against arthritis,

skin rashes & mouth ulcers
Hydroxychlorquine (Plaquenil) may
take 4-6 months before a benefit is
seen
Side effects = ? Harm foetus, gastric
symptoms,rash, darkening of skin,
muscle weakness, retinal damage
Eye exam before then 6 monthly

Immunosuppressive
Agents

Azathioprine
Cyclophosphamide in severe
cases, (especially in lupus nephritis &
cerebritis)

Mycophenolate Mofetil

TERIMAKASIH