systemic%20lupus%20erythematosus%20(pediatric)
SYSTEMIC LUPUS ERYTHEMATOSUS (PEDIATRIC)
Systemic lupus erythematosus is a chronic, multisystem, inflammatory, autoimmune disorder characterized by formation of autoantibodies directed against self-antigens and immune-complex formation.
It can be suspected when ≥2 organ systems are involved.
It is predominantly diagnosed in females of childbearing age, rarely diagnosed before 8 years old.
Clinical presentation varies in different patients and the disease activity varies over time in a single patient. Majority of patients have arthralgia of the hand.

Monitoring

  • Monitoring during clinic visit should include:
    • History-taking
    • Physical exam
    • Lab tests
      • Complete blood count (CBC)
      • Creatinine measurement
      • Urinalysis
  • Results of lab tests that may precede a disease flare:
    • Decrease in serum complement levels
    • Increase in anti-double stranded deoxyribonucleic acid (dsDNA)
    • Increase in erythrocyte sedimentation rate (ESR)
    • Decrease in hemoglobin level, leukocyte or platelet counts
    • Increase in creatine phosphokinase (CPK) levels
    • Appearance of microscopic hematuria or proteinuria

Comorbidities & Complications of Systemic Lupus Erythematosus (SLE)

  • Comorbidities for which systemic lupus erythematosus (SLE) patients are at an increased risk include infections, hypertension, atherosclerosis, dyslipidemia, diabetes, osteoporosis, avascular necrosis & malignancies

Neuropsychiatric Systemic Lupus Erythematosus (NPSLE)

  • Monitor systemic lupus erythematosus (SLE) patients for neurological &/or psychiatric manifestations as in non-neuropsychiatric systemic lupus erythematosus (NPSLE) patients
  • Usually appears w/in 1 year from the time of diagnosis; may also appear before or at the time of diagnosis
  • Diagnostic work-up may include the following:
    • Lumbar puncture
    • Nerve conduction studies (NCS)
    • Neuropsychological assessment of cognitive function
    • Neuroimaging: T1/T2 magnetic resonance imaging (MRI), diffusion-weighted imaging, gadolinium-enhanced T1 sequences
    • Cerebrospinal fluid (CSF) analysis
    • Electroencephalography (EEG)
  • Patients found to have neuropsychiatric systemic lupus erythematosus (NPSLE) should be referred to a team of psychiatrists, psychologists, neurologist & rheumatologist

Toxicity

  • Patients on long-term glucocorticoids should be monitored for:
    • Electrolyte, glucose & lipid levels to identify metabolic conditions
    • Bone densitometry to identify osteoporosis & monitor response to treatment
  • Patients on Hydroxychloroquine should have ophthalmological exam every 6-12 months to detect retinal toxicity
  • Patients on immunosuppressants should be monitored for hematologic, liver & renal toxicity, & occurrence of infection

Lupus Nephritis

  • Patients suspected to have lupus nephritis should immediately undergo renal biopsy to confirm diagnosis, evaluate severity, determine prognosis & therapy
  • Indications for renal biopsy in systemic lupus erythematosus (SLE) patients
    • Unexplained increase in serum creatinine in the absence of alternative causes (eg sepsis, hypovolemia, medications)
    • Confirmed proteinuria (≥1.0 gm/24 hours)
    • Presence of the following (result of 2 tests done w/in a short period of time, w/o other causes):
      • Proteinuria of >5.0 gm/24 hours & hematuria [≥5 red blood cells/high-power field (RBCs/hpf)] or
      • Proteinuria of >5.0 gm/24 hours & cellular casts
  • Recommended tests used for monitoring of lupus nephritis:
  •  

      Active nephritis at onset of treatment Previous active nephritis, none currently No prior or current nephritis
    Blood pressure 1 3 3
    Urinalysis 1 3 6
    Protein/ Creatinine ratio 1 3 6
    PSerum creatinine 1 3 6
    C3/C4 levels 2 3 6
    Anti-DNA 1 3 3

    *Values are at monthly intervals indicating minimum time at which the test should be measured
    Modified from: 2012 American College of Rheumatology Guidelines for Screening, Treatment, and Management of Lupus Nephritis

Digital Edition
Asia's trusted medical magazine for healthcare professionals. Get your MIMS JPOG - Malaysia digital copy today!
Sign In To Download
Editor's Recommendations
Most Read Articles
Pearl Toh, 31 Dec 2019
Adding the neuraminidase inhibitor oseltamivir to usual care speeds up recovery from influenza-like illness by a day compared with usual care alone, with even greater benefits seen in older, sicker patients with comorbidities, according to the ALIC4E study.
23 Dec 2019
At a Menarini-sponsored symposium held during the Asian Pacific Society Congress, renowned cardiologist Prof John Camm provided the latest evidence for chronic stable angina with or without concomitant diseases, with a special focus on the antianginal agent ranolazine and combination therapies. The event was chaired and moderated by Dr Dante Morales from the University of the Philippines College of Medicine.
Pearl Toh, 6 days ago
Obeticholic acid significantly improves fibrosis and disease activity in patients with nonalcoholic steatohepatitis (NASH), a chronic liver disease currently with no approved therapy, according to an interim analysis of the landmark REGENERATE* study.
Stephen Padilla, 5 days ago
The Lancet Commission on Hypertension Group has recently released a position statement that contains a list of recommendations for the improvement of accuracy standards for devices that measure blood pressure (BP).