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ERYTHEMATOSUS
INTRODUCTION
WHAT IS PAEDIATRIC SLE?
EPIDEMIOLOGY
Although SLE affects primarily women of childbearing age, approximately
5% of cases present in childhood, mainly around puberty. SLE is rare in
children younger than 9 years of age. Although there is a female
predominance of this disease in adolescence and adulthood, there is an
equal gender distribution in children. The overall prevalence of SLE in the
pediatric population is 10 to 25 cases per 100,000 children.
A LITTLE BIT OF HISTORY
Lupus is the Latin word for wolf. Erythematosus means red rashes. In 1851, Dr.
Cazenave discovered red rashes on a patient’s face that looked like wolf bites.
He named the rash Discoid Lupus Erythematosus (DLE).
In 1885, Sir William Osler recognized that many people with lupus had a disease
involving not only the skin but many other organs or systems. He named the
disease Systemic Lupus Erythematosus (SLE).
TYPES OF LUPUS
1. Systemic Lupus Erythematosus (SLE)
One that most people refer to when they say “lupus”. The symptoms of SLE
may be mild or serious. Although SLE usually first affects people between the
ages of 15 and 45, it can occur in childhood or later in life as well.
A rare disorder that can occur in newborn babies. Scientists suspect that
neonatal lupus is caused by auto-antibodies in the mother’s blood called
anti-Ro (SSA) and anti-La (SSB). Auto-antibodies (“auto” means “self”) are
blood proteins that act against the body’s own parts.
At birth, the babies have a skin rash, liver problems, and low blood counts.
These symptoms gradually go away over several months, although in rare
cases, babies with neonatal lupus may have a heart problem that slows down
the natural rhythm of the heart.
Some drugs may cause SLE-like features and hence this condition is called
“drug-induced lupus”. The features typically go away completely when the
drug is stopped. The kidneys and brain are rarely involved.
CLINICAL FEATURES
CARDIAC
Endocarditis
Myocarditis
Pericarditis
CONTITUTIONAL
Fatigue
Fever
Weight loss
GASTROINTESTINAL
Abdominal pain
Nausea & vomiting
CLINICAL FEATURES
DERMATOLOGICAL
Alopecia
Butterfly rash
Mucous membrane lesion
Photosensitivity
Purpura
Raynaud’s phenomenon
Urticaria
Vasculitis
CLINICAL FEATURES
HEMATOLOGIC
Anemia
Leukopenia
Thrombocytopenia
MUSCULOSKELETAL
Arthralgia
Arthritis
Myositis
PULM ONARY
Pleurisy
Pulmonary hypertension
Pulmonary parenchyma
CLINICAL FEATURES
NEUROPSYCHIATRIC
Cranial neuropathies
Organic brain syndrome
Peripheral neuropathies
Psychosis
Seizures
Transverse myelitis
RENAL
Casts
Hematuria
Nephrotic syndrome
Proteinuria
CLINICAL FEATURES
RETICULOENDOTHELIAL
Hepatomegaly
Lymphadenopathy
Splenomegaly
Clinical presentation varies in different patients & the disease activity varies
over time in a single patient
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 cell’s
nucleus (command centre).
WHAT CAUSES SLE?
The fact that SLE can run in families indicates that its development has a genetic
basis; however, no specific “lupus gene” has been identified yet.
Some of the other factors scientists are studying include sunlight, stress, certain
drugs, and agents such as viruses.
DIAGNOSIS
Diagnosis of systemic lupus erythematosus (SLE) is based on clinical symptoms &
lab findings
≥4 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.
11 common criteria, or measures that was developed by the American
College of Rheumatology (ACR):
1. Malar rash – a rash over the cheeks & nose, often in the shape of a butterfly
2. Discoid rash – a rash that appears red, raised, disk-shaped patches
3. Photosensitivity – a reaction to sun or light that causes a skin rash to appear
or get worse
4. Oral Ulcers – sores appearing in the mouth
5. Arthritis – joint pain & swelling of 2 or more joints in which the bones
around the joints do not become destroyed
DIAGNOSIS
6. Serositis – 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 – persistent protein or cellular casts in the
urine.
8. Neurological disorder – seizures or psychosis
9. Blood disorder – anemia, leukopenia, lymphopenia, or
thrombocytopenia
10. Immunologic disorder – anti-DNA or anti-Sm or positive
antiphospholipid antibodies
11. Abnormal antinuclear antibody (ANA)
DIAGNOSIS
Diagnosis of systemic lupus erythematosus (SLE) is based on clinical symptoms &
lab findings
Nonscarring alopecia
Diffuse thinning or hair fragility w/ visible broken hairs
In the absence of other causes such as alopecia areata, drugs, iron deficiency,
& androgenic alopecia
Renal
Urine protein–to-creatinine ratio (or 24-hour urine protein) representing 500
mg protein/24 hours or red blood cell casts
DIAGNOSIS
C L I N I C A L C R I T E R I A:
Serositis
Typical pleurisy for >1 day or pleural effusions or pleural rub
Typical pericardial pain (pain w/ recumbency improved by sitting forward) for
>1 day or pericardial effusion or pericardial rub or pericarditis by
electrocardiography
In the absence of other causes, such as infection, uremia, & Dressler’s
pericarditis
DIAGNOSIS
C L I N I C A L C R I T E R I A:
Neurologic
Seizures
Psychosis
Mononeuritis multiplex (in the absence of other known causes such as
primary vasculitis)
Myelitis
Peripheral or cranial neuropathy (in the absence of other known causes such
as primary vasculitis, infection, & diabetes mellitus)
Acute confusional state (in the absence of other causes, including
toxic/metabolic, uremia, drugs)
DIAGNOSIS
C L I N I C A L C R I T E R I A:
Hemolytic anemia
Leukopenia (<4000/mm3)
at least once, in the absence of other known causes such as Felty’s syndrome,
drugs, & portal hypertension or Lymphopenia (<1000/mm3) at least once, in
the absence of other known causes such as
Thrombocytopenia (<100,000/mm3)
At least once in the absence of other known causes such as drugs, portal
hypertension, & thrombotic thrombocytopenic purpura
DIAGNOSIS
I M M U N O L O G I C A L C R I T E R I A:
PHARMACOTHERAPY
Oral corticosteroids
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
Lowest possible dose should be used for maintenance therapy
High-dose corticosteroids are necessary for refractory manifestations of SLE
& for severe organ systems’ manifestations especially CNS, renal &
hematologic manifestations
Decreases inflammation by suppression of the immune system
Topical corticosteroids
Helpful for discoid lesions especially on the scalp
Use a less potent steroid on the face because it is more prone to atrophy
TREATMENT
CORTICOSTEROIDS
Parenteral corticosteroids
Pulse therapy with IV corticosteroids in combination with
immunosuppressive therapy is recommended for Class III and IV SLE patients
with confirmed glomerulonephritis
TREATMENT
HYDROXYCHLOROQUINE
Recommended as background treatment for Class III and IV SLE patients with
nephritis
TREATMENT
IMMUNOSUPPRESSANTS
1. Azathioprine
2. Belimumab
3. Cyclophosphamide
4. IV Immune Globulin (IVIg)
5. Methotraxate
TREATMENT
NSAIDS
These drugs provide symptomatic relief of fever, arthritis & mild serositis
SUNSCREEN
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
COMPLICATIONS
Some degree of long term and often permanent organ dysfunction from
either SLE or its treatment has been found in 88% of patients.
Hypertension
Growth retardation
Chronic pulmonary impairment
Ocular abnormalities
Permanent renal damage
Neuropsychiatric symptoms
Musculoskeletal damage
Gonadal impairment
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.