DIAGNOSIS AND CLINICAL FEATURES OF CHILDHOOD SYSTEMIC LUPUS ERYTHEMATOSUS
Amita Aggarwal*
Department of Pediatrics.*
Systemic Lupus Erythematosus
  • Rare disorder
  • Multi-system involvement
  • Most often affects teenage girls
  • Early diagnosis essential for good results

7 years old boy
  • Fever 6 months
  • No localizing symptoms
  • Examination: febrile rest WNL
  • Investigations
  • TLC 3800, lympho 20%
  • Urine normal
  • Chest X-ray

7 years old boy
  • Fever 6 months
  • No localizing symptoms
  • Examination: febrile rest WNL
  • Investigations - TLC 3800 lympho 20% - CXR - pericardial effusion - Pericardial fluid: exudative AFB neg. - ANA + 1:160 ; C3 C4 low

11 / girl
  • Fever low grade 3 mo
  • Rash all over the body
  • Arthralgia
  • Painful oral ulcers 1 mo
  • Difficulty in getting up from bed 10 d

-Dermatomyositis
-SLE

Examination
  • Pallor
  • Generalized lymphadenopathy
  • Oral ulcers on hard palate
  • Hepatomegaly
  • BP 110 / 70 mm
  • Neck and truncal weakness


Investigations
  • Hb 6.8 g/dl
  • TLC 1500
  • Platelet 22,000
  • Creatinine 1.5 mg
  • 24 hr Proteinuria 520 mg
  • FNAC LN: reactive hyperplasia
  • ANA> 1:320 rim
  • C3 <17.3 mg/dl
  • C4 <5.4 mg/dl
  • Anti-dsDNA 375 IU
  • Muscle enzymes
    - CPK 400 IU
    - AST 378 IU
    - ALT 140 IU

16 / girl
  • 6 months ago
    • Polyarthritis
    • ASO titres
    • Fever off & on
    • Dx acute RF
  • 2 months
    • Malar rash
    • Pericardial effusion
    • Alopecia
    • Anemia
    • ANA+ ? - anti-dsDNA ?C3, C4 Dx SLE
  • 5 days
    - Abnormal behaviour
    CNS lupus

3 week old child
  • Born to an SLE mother
  • Erythematous rash on exposure to light
  • Hb 10 G/dl
    Platelets 75,000
    TLC 3400/cu mm
    ALT/AST 84/92 IU

ACR Criteria for Diagnosis
  • Malar rash
    Photosensitivity
  • Recurrent oral ulcers
  • Discoid lupus
  • Serositis
  • CNS disease
    • Seizures
    • Psychosis
  • Arthritis
  • Hematological abn
  • Renal disease
  • ANA positivity
  • Autoantibodies
    • Anti-dsDNA Abs>
    • Anti-Sm Ab
    • Lupus anticoagulant
    • Anti-phospholipid antibodies

Three common early features

  • Fever
  • Fatigue
  • Arthralgia
  • Failure to thrive

ACR criteria do not represent the most common features
SGPGI experience (N = 90)
Median Age of onset 12 years
Median duration of symptoms 1 year
Girls: Boys 80:10

Neonatal Lupus 02 cases

Lupus: Clinical features
Renal involvement
When to suspect lupus
Child with
  • unexplained fever
  • glomerulonephritis
  • unexplained multisystem disease
  • serositis & arthritis
  • skin & joint disease
  • unexplained neurological symptoms and signs

What to do?
Test for ANA
  • High sensitivity> 98%
  • If negative, almost rules out diagnosis of SLE

What to do?
  • Assess degree of organ involvement
    • Urine examination, creatinine
    • Complete blood count
    • CXR, ECG
    • Coomb's test
    • CSF analysis & MRI scan

  • Assess disease activity
    • anti-dsDNA antibodies
    • complement levels

How to monitor disease activity?
  • Clinical Symptoms and Signs
  • Look for
    • TLC, Plat, Hb
    • Albuminuria, active sediment
    • Anti-dsDNA antibodies
    • Complement levels

  • Assess damage
    • Hypertension, Creatinine

Longterm complications:

Related to disease
Accelerated atherosclerosis
Premature CAD
Hypertension
Renal failure

Related to drugs
Corticosteroids
Growth retardation
Premature cataract
Diabetes
Hypertension
Avascular necrosis
Cytotoxics
Gonadal failure
Malignancy
NSAIDs
Analgesic nephropathy


Conclusion
  • SLE is a treatable condition
  • Early diagnosis key to better outcome
  • Suspect lupus when
    • Multisystem involvement
    • Unexplained symptoms
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