Systemic lupus erythematosus

• Rare disorder
• Multisystem 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/cumm
  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 Absbr>
  • 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

Long term 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

RCIAPCON 2005 - Conference Abstracts.Pediatric Oncall [serial online] 2006 [cited 15 June 2006(Supplement 6)];3. Available from:
http://www.pediatriconcall.com/fordoctor/Conference_abstracts/SLE.asp