Clinical and laboratory features suggestive of childhood-onset systemic lupus erythematosus (SLE)

Clinical manifestations

Persistent fever
Fatigue
Anorexia
Weight loss
Arthralgia and arthritis
Myositis
Serositis (pleuritis, pericarditis, peritonitis)
Malar rash
Discoid rash
Alopecia
Oral and nasal ulcers
Photosensitivity
Lymphadenopathy
Peripheral edema
Central nervous system (CNS) features^*

Laboratory findings

Persistent thrombocytopenia (platelets <150,000/mL)
Hemolytic anemia (Hgb ≤12 g/dL)^¶
Lymphopenia (lymphocytes <1500/mL)
Leukopenia (WBC <4000/mL)
Hematuria
Proteinuria
Hypocomplementemia (low C3 and C4)
Autoantibodies (ANA, anti-dsDNA, anti-Smith, anti-RNP, anti-Ro/SSA, anti-La/SSB, lupus anticoagulant, aCL, anti-beta2GPI)
Elevated liver enzymes/hepatitis (not necessarily specific for SLE)
Increased ESR (but normal CRP, except in a few scenarios)
Other supportive features:
- Hypergammaglobulinemia (IgG)
- Hypoalbuminemia

SLE should be suspected in a child with any of these clinical or laboratory features.

aCL: anticardiolipin; ANA: antinuclear antibody; anti-B2GPI: anti-beta2 glycoprotein I; anti-dsDNA: anti-double-stranded DNA; anti-RNP: anti-ribonucleoprotein; CRP: C-reactive protein; ESR: erythrocyte sedimentation rate; Hgb: hemoglobin; IgG: immunoglobulin G; WBC: white blood cell.

* CNS features include headaches, cognitive impairment, psychosis (visual and auditory hallucinations, distortions), acute confusional state (resembling delirium), seizures, stroke, chorea, demyelination, idiopathic intracranial hypertension, mood disorders, anxiety disorders, and aseptic meningitis.^[1]

¶ Especially direct antiglobulin test (DAT) positive.

Reference:

The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes. Arthritis Rheum 1999; 42:599.

Graphic 64565 Version 6.0

خرید پکیج

Clinical and laboratory features suggestive of childhood-onset systemic lupus erythematosus (SLE)