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.

Systemic%20lupus%20erythematosus%20(pediatric) 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)

  • ≥4 criteria (at least 1 clinical & 1 immunologic criteria) or biopsy-proven lupus nephritis w/ positive antinuclear antibody (ANA) or anti-double stranded deoxyribonucleic acid (dsDNA)
  • Symptom/finding need not be present all at the same time

Clinical Criteria:

  • Acute cutaneous lupus, including:
    • Lupus malar rash (do not count if malar discoid)
    • Bullous lupus
    • Toxic epidermal necrolysis variant of systemic lupus erythematosus (SLE)
    • Maculopapular lupus rash
    • Photosensitive lupus rash (In the absence of dermatomyositis) or
    •  Subacute cutaneous lupus (nonindurated psoriaform &/or annular polycyclic lesions that resolve w/out scarring, although occasionally w/ post-inflammatory dyspigmentation or telangiectasias)
  • Chronic cutaneous lupus, including:
    • Classic discoid rash: localized (above the neck) or generalized (above & below the neck)
    • Hypertrophic (verrucous) lupus
    • Lupus panniculitis (profundus)
    • Mucosal lupus
    • Lupus erythematosus tumidus
    • Chilblains lupus
    • Discoid lupus/lichen planus overlap
  • Oral Ulcers or Nasal Ulcers
    • Oral: palate, buccal, tongue
    • In the absence of other causes, such as vasculitis, Behcet’s disease, infection (herpesvirus), inflammatory bowel disease, reactive arthritis, & acidic foods
  • 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
  • Synovitis involving ≥2 joints
    • Characterized by swelling or effusion
    • Or tenderness in ≥2 joints & at least 30 minutes of morning stiffness
  • 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
  • Renal
    • Urine protein–to-creatinine ratio (or 24-hour urine protein) representing 500 mg protein/24 hours or red blood cell casts
  • 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) 
  • 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 corticosteroids, drugs, & infection
  • Thrombocytopenia (<100,000/mm3)
    • At least once in the absence of other known causes such as drugs, portal hypertension, & thrombotic thrombocytopenic purpura

Immunologic Criteria:

  • Antinuclear antibodies (ANA) level above laboratory reference range
  • 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)]
  • Anti-Smith (Anti-Sm): presence of antibody to Smith (Sm) nuclear antigen
  • Antiphospholipid antibody positivity, as determined by:
    • Positive test for lupus anticoagulant
    • False-positive test result for rapid plasma reagin
    • Medium- or high-titer anticardiolipin antibody level  [Immunoglobulin A (IgA), immunoglobulin G (IgG) or immunoglobulin M (IgM)]
    • Positive test result for anti-B2-glycoprotein I [Immunoglobulin A (IgA), immunoglobulin G (IgG) or immunoglobulin M (IgM)]
  • Low complement (C3, C4, or CH50)
  • Direct Coombs’ test (in the absence of hemolytic anemia)

Expert Referral

  • Establish good working relationship amongst the primary care physician, pediatrician, rheumatologist, nurse, pharmacist, other healthcare providers, the patient & his/her family
    • Team approach will allow for earlier identification of disease flares & medication toxicity
  • Patient will initially present to a primary care physician/pediatrician who is responsible for:
    • Recognizing the signs & symptoms of systemic lupus erythematosus (SLE) in their patients
    • Making an initial diagnosis
    • Managing & monitoring patients w/ mild & stable systemic lupus erythematosus (SLE)
    • Referring patient to a rheumatologist & participating in further systemic lupus erythematosus (SLE) diagnosis, management & monitoring
  • A rheumatologist is an integral part of the medical team managing an systemic lupus erythematosus (SLE) patient
  • Referral to a rheumatologist is indicated for:
    • Confirmation of diagnosis
    • Management plan for patient
    • Periodic evaluation of disease activity & severity
    • Management of uncontrolled disease
    • Management of organ involvement or life-threatening disease
    • Prevention &/or management of treatment toxicities
    • Special situations eg pregnancy, antiphospholipid syndrome, life-threatening disease, concomitant infection & surgery 
  • Systemic lupus erythematosus (SLE) patients should be referred to psychologist, psychiatrist, physical &/or occupational therapist, ophthalmologist, dermatologist, nephrologist, cardiologist, orthopedic surgeon & other specialists when necessary
    • If there are signs of renal involvement, patient should be referred to a nephrologist for management
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