2. INTRODUCTION
WHAT IS SLE?
Systemic lupus erythematosus is a chronic, multisystem,
inflammatory, autoimmune disorder characterized by formation of
autoantibodies directed against self-antigens and immune-complex
formation resulting in damage to essentially any organ.
4. WHAT CAUSES SLE?
SLE is an autoimmune disorder that develops when the bodyâs immune
system begins to attack its own tissues. Its cause is unknown, but it is
likely that a combination of genetic, environmental, and, possibly,
hormonal factors work together to cause SLE.
This occurs through the production of âauto-antibodiesâ that attack a
personâs own cells thus contributing to the inflammation of various
parts of the body, and may cause damage to organs and tissues.
The most common type of auto-antibody that develops in people
with SLE is called an antinuclear antibody (ANA) because it reacts
with parts of the cellâs nucleus (command centre).
5. WHAT CAUSES SLE?
The fact that SLE can run in families indicates that its development has a
genetic
basis; however, no specific âlupus geneâ has been identified yet.
Studies suggest that several different genes may be involved in
determining a personâs likelihood of developing the disorder, which
tissues and organs are affected, and the severity of disease. However, it
is believed that genes alone do not determine who gets SLE and that
other factors also play a role.
Some of the other factors scientists are studying include sunlight, stress,
certain drugs, and agents such as viruses.
6. CLINICAL FEATURES
C A R D I A C
ī§ Endocarditis
ī§ Myocarditis
ī§ Pericarditis
C O N T I T U T I O N A L
ī§ Fatigue
ī§ Fever
ī§ Weight
loss
G A S T R O I N T E S T I N A L
ī§ Abdominal pain
ī§ Nausea &
vomiting
7. CLINICAL FEATURES
D E R M A T O L O G I C A L
ī§ Alopecia
ī§ Butterfly rash
ī§ Mucous membrane
lesion
ī§ Photosensitivity
ī§ Purpura
ī§ Raynaudâs
phenomenon
ī§ Urticaria
ī§ Vasculitis
8. CLINICAL FEATURES
H E M A T O L O G I C
ī§ Anemia
ī§ Leukopenia
ī§ Thrombocytopeni
a
M U S C U L O S K E L E T A L
ī§ Arthralgi
a
ī§ Arthritis
ī§ Myositis
P U L M O N A R Y
ī§ Pleurisy
ī§ Pulmonary
hypertension
ī§ Pulmonary
parenchyma
9. CLINICAL FEATURES
N E U R O P S Y C H I A T R I C
ī§ Cranial neuropathies
ī§ Organic brain
syndrome
ī§ Peripheral
neuropathies
ī§ Psychosis
ī§ Seizures
ī§ Transverse myelitis
R E N A L
ī§ Casts
ī§ Hematuria
ī§ Nephrotic
syndrome
ī§ Proteinuria
10. CLINICAL FEATURES
R E T I C U L O E N D O T H E L I A L
ī§ Hepatomegaly
ī§ Lymphadenopathy
ī§ Splenomegaly
Clinical presentation varies in different patients & the disease
activity varies
over time in a single patient
1. Majority of patients have arthralgia of the hand
2. Most frequent manifestations in children include fever, rash,
alopecia,
arthritis & renal involvement
3. Compared with adults, children have a higher incidence of malar
rash, anemia, leukopenia, neurologic & renal involvement
11. DIAGNOSIS
Diagnosis of systemic lupus erythematosus (SLE) is based on clinical
symptoms &lab findings
Diagnosis based on the American College of Rheumatology
criteria for the diagnosis of definite lupus in children
ī§ âĨ4 criteria on the list either at the present time or at some time in the
past,there is a strong chance that you have lupus.
ī§ 11 common criteria, or measures that was developed by the
American College of Rheumatology (ACR):
1. Malar rash â a rash over the cheeks & nose, often in the shape of a
butterfly
2. Discoid rash â a rash that appears red, raised, disk-shaped patches
3. Photosensitivity â a reaction to sun or light that causes a skin rash to
appear or get worse
4. Oral Ulcers â sores appearing in the mouth
5. Arthritis â joint pain & swelling of 2 or more joints in which the bones
around the joints do not become destroyed
12. DIAGNOSIS
6. Serositis â inflammation of the lining around the lungs
(pleuritis) or inflammation of the lining around the heart
that causes chest pain which is worse with deep
breathing (pericarditis)
7. Kidney disorder â persistent protein or cellular casts
in the urine.
8. Neurological disorder â seizures or psychosis
9. Blood disorder â anemia, leukopenia,
lymphopenia, or thrombocytopenia
10.Immunologic disorder â anti-DNA or anti-Sm or
positive antiphospholipid antibodies
11.Abnormal antinuclear antibody (ANA)
13. DIAGNOSIS
Diagnosis of systemic lupus erythematosus (SLE) is based on clinical
symptoms &lab findings
Diagnosis based on the Systemic Lupus International
Collaborating Clinics (SLICC) classification criteria for systemic
lupus erythematosus (SLE)
ī§ âĨ4 criteria (at least 1 clinical & 1 immunologic
criteria)
or
Biopsy-proven lupus nephritis with positive antinuclear antibody
(ANA)
or
Anti-double stranded deoxyribonucleic acid (dsDNA)
ī§ Symptom/finding need not be present all at the same time
14. DIAGNOSIS
C L I N I C A L C R I T E R I A:
Acute cutaneous lupus, including:
ī§ Lupus malar rash (do not count if malar discoid)
ī§ Bullous lupus
ī§ Toxic epidermal necrolysis variant of systemic lupus erythematosus
(SLE)
ī§ Maculopapular lupus rash
ī§ Photosensitive lupus rash (In the absence of dermatomyositis) or
ī§ Subacute cutaneous lupus (nonindurated psoriaform &/or annular
polycyclic lesions that resolve w/out scarring, although occasionally
w/ post- inflammatory dyspigmentation or telangiectasias)
15. DIAGNOSIS
C L I N I C A L C R I T E R I A:
Chronic cutaneous lupus, including:
ī§ Classic discoid rash: localized (above the neck) or generalized (above
& below the neck)
ī§ Hypertrophic (verrucous) lupus
ī§ Lupus panniculitis (profundus)
ī§ Mucosal lupus
ī§ Lupus erythematosus tumidus
ī§ Chilblains lupus
ī§ Discoid lupus/lichen planus overlap
Oral Ulcers or Nasal Ulcers
ī§ Oral: palate, buccal, tongue
ī§ In the absence of other causes, such as vasculitis, Behcetâs disease,
infection (herpesvirus), inflammatory bowel disease, reactive arthritis,
& acidic foods
16. DIAGNOSIS
C L I N I C A L C R I T E R I A:
Nonscarring alopecia
ī§ Diffuse thinning or hair fragility w/ visible broken hairs
ī§ In the absence of other causes such as alopecia areata, drugs, iron
deficiency, & androgenic alopecia
Synovitis involving âĨ2 joints
ī§ Characterized by swelling or effusion
ī§ Or tenderness in âĨ2 joints & at least 30 minutes of morning stiffness
Renal
ī§ Urine proteinâto-creatinine ratio (or 24-hour urine protein) representing
500 mg protein/24 hours or red blood cell casts
17. DIAGNOSIS
C L I N I C
A L
C R I T E R I
A:
Serositis
ī§ Typical pleurisy for >1 day or pleural effusions or pleural rub
ī§ Typical pericardial pain (pain w/ recumbency improved by sitting
forward) for
>1 day or pericardial effusion or pericardial rub or pericarditis
by electrocardiography
ī§ In the absenceof other causes,such as infection, uremia, &
Dresslerâs pericarditis
18. DIAGNOSIS
C L I N I C
A L
C R I T E R I
A:
Neurologic
ī§ Seizures
ī§ Psychosis
ī§ Mononeuritis multiplex (in the absence of other known causes
such as primary vasculitis)
ī§ Myelitis
ī§ Peripheral or cranial neuropathy (in the absence of other known
causes such as primary vasculitis, infection, & diabetes mellitus)
ī§ Acute confusional state (in the absence of other causes,
including toxic/metabolic, uremia, drugs)
19. DIAGNOSIS
C L I N I C A L C R I T E R I A:
Hemolytic anemia
Leukopenia
(<4000/mm3)
ī§ at least once, in the absence of other known causes such as Feltyâs
syndrome, drugs, & portal hypertension or Lymphopenia (<1000/mm3)
at least once, in the absence of other known causes such as
Corticosteroids, drugs, &
infection Thrombocytopenia
(<100,000/mm3)
ī§ At least once in the absence of other known causes such as
drugs, portal hypertension, & thrombotic thrombocytopenic
20. DIAGNOSIS
I M M U N O L O G I C A L C R I T E R I A:
ī§ Antinuclear antibodies (ANA) level above laboratory reference
range
ī§ Anti-double stranded deoxyribonucleic acid (dsDNA) antibody level
above laboratory reference range [or >2-fold the reference range if
tested by enzyme-linked immunosorbent assay (ELISA)]
ī§ Anti-Smith (Anti-Sm): presence of antibody to Smith (Sm) nuclear
antigen
21. DIAGNOSIS
I M M U N O L O G I C A L C R I T E R I A:
ī§ Antiphospholipid antibody positivity, as determined by:
o Positive test for lupus anticoagulant
o False-positive test result for rapid plasma reagin
o Medium- or high-titer anticardiolipin antibody level
[Immunoglobulin A (IgA), immunoglobulin G (IgG) or
immunoglobulin M (IgM)]
o Positive test result for anti-B2-glycoprotein I [Immunoglobulin A
(IgA), immunoglobulin G (IgG) or immunoglobulin M (IgM)]
ī§ Low complement (C3, C4, or CH50)
ī§ Direct Coombsâ test (in the absence of hemolytic anemia)
23. MANAGEMENT
MONITORING
Results of lab tests that may precede a disease flare:
1. Decrease in serum complement levels
2. Increase in anti-double stranded deoxyribonucleic acid
(dsDNA)
3. Increase in erythrocyte sedimentation rate (ESR)
4. Decrease in hemoglobin level, leukocyte or platelet counts
5. Increase in creatine phosphokinase (CPK) levels
6. Appearance of microscopic hematuria or proteinuria
24. TREATMENT
G O A L S O F T H E R A P Y :
ī§ Control disease manifestation
ī§ Allow child to have a good quality of life without major
exacerbations
ī§ Prevent serious organ damage that adversely affects function or
lifespan
ī§ Prevent adverse effects of the drugs used
PHARMACOTHERAPY
Corticosteroi
ds
Immunosuppressa
nts
NSAI
Ds
Sunscre
en
25. TREATMENT
C O R T I C O S T E R O I D S
Oral corticosteroids
ī§ Patients w/ mild SLE do not normally require use of systemic
corticosteroids but there are patients who has low quality of life if not
given low-dose corticosteroids
ī§ Lowest possible dose should be used for maintenance therapy
ī§ High-dose corticosteroids are necessary for refractory manifestations
of SLE & for severe organ systemsâ manifestations especially CNS,
renal & hematologic manifestations
ī§ Decreases inflammation by suppression of the immune system
Topical corticosteroids
ī§ Helpful for discoid lesions especially on the scalp
ī§ Use a less potent steroid on the face because it is more prone to
atrophy
26. TREATMENT
C O R T I C O S T E R O I D S
Parenteral corticosteroids
ī§ Pulse therapy with IV corticosteroids in combination with
immunosuppressive therapy is recommended for Class III and IV SLE
patients with confirmed glomerulonephritis
27. TREATMENT
H Y D R O X Y C H L O R O Q U I N E
ī§ Used for skin & joint manifestations
ī§ Also used for preventing flares & other constitutional symptoms
ī§ Inhibits chemotaxis of eosinophils & locomotion of neutrophils &
impairs complement-dependent antigen-antibody reactions
ī§ Recommended as background treatment for Class III and IV SLE
patients with nephritis
28. TREATMENT
I M M U N O S U P P R E S S A N T S
These agents act as immunosuppressive, cytotoxic & anti-
inflammatory agents
ī§ In the treatment of severe CNS & severe glomerulonephritis,
thrombocytopenia & hemolytic anemia, high dose
glucocorticoids & immunosuppressantS are used
ī§ Concomitant use with corticosteroids allows lower
doses of immunosuppressants
1. Azathioprine
2. Belimumab
3. Cyclophosphamide
4. IV Immune Globulin (IVIg)
5. Methotraxate
29. TREATMENT
N S A I D S
ī§ These drugs provide symptomatic relief of fever, arthritis & mild
serositis
ī§ Inhibit inflammatory reactions & pain by decreasing prostaglandin
synthesis
ī§ SLE patients have a high incidence of NSAID-induced hepatotoxicity
S U N S C R E E N
Patients with SLE should apply sunscreen with at least an SPF of 15 to
prevent dermal or systemic disease flares upon exposure to ultraviolet
light
30. COMPLICATIONS
Some degree of long term and often permanent organ dysfunction
from
either SLE or its treatment has been found in 88% of patients.
ī§ Hypertension
ī§ Growth retardation
ī§ Chronic pulmonary impairment
ī§ Ocular abnormalities
ī§ Permanent renal damage
ī§ Neuropsychiatric symptoms
ī§ Musculoskeletal damage
ī§ Gonadal impairment
31. PROGNOSIS
Outcomes for SLE have improved significantly over the past several
decades and depend largely on the organ systems that are involved.
Worse prognoses are seen in patients with severe lupus nephritis or
cerebritis, with risk of chronic disability or progression to renal failure.
With current therapy for the disease and the success of renal
transplantation, however, most patients live well into adulthood.