12. tbl. 97. árg. 2011

Cry wolf

Úlfur, úlfur

Systemic Lupus Erythematosus (SLE) is an uncommon disease in children and adolescents but far from being unknown. The disease's symptoms are often non-specific and vague at first and clinicians must suspect SLE without the more specific symptoms. Treatment should be initiated as soon as possible to delay or prevent serious complications. This case demonstrates the history of a young boy who needed medical attention at the Children's Hospital of Iceland because of joint pain, swollen lymph nodes and fatigue. The epidemiology, diagnostic criteria, treatment and prognosis of children diagnosed with SLE are reviewed.

Table I

Diagnostic criteria for Systemic Lupus Erythematosus (SLE)

Criterion Definition
Malar Rash Fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds
Discoid rash Erythematous raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur in older lesions
Photosensitivity Skin rash as a result of unusual reaction to sunlight, by patient history or physician observation
Oral ulcers Oral or nasopharyngeal ulceration, usually painless, observed by physician
Nonerosive Arthritis Involving 2 or more peripheral joints, characterized by tenderness, swelling, or effusion
Pleuritis or Pericarditis

Pleuritis--convincing history of pleuritic pain or rubbing heard by a physician or evidence of pleural effusion


Pericarditis--documented by electrocardigram or rub or evidence of pericardial effusion

Renal Disorder

Persistent proteinuria > 0.5 grams per day or > than 3+ if quantitation not performed


Cellular casts--may be red cell, hemoglobin, granular, tubular, or mixed

Neurologic Disorder

Seizures--in the absence of offending drugs or known metabolic derangements; e.g., uremia, ketoacidosis, or electrolyte imbalance


Psychosis--in the absence of offending drugs or known metabolic derangements, e.g., uremia, ketoacidosis, or electrolyte imbalance

Hematologic Disorder

Hemolytic anemia--with reticulocytosis


Leukopenia--< 4,000/mm3 on ≥ 2 occasions


Lyphopenia--< 1,500/ mm3 on ≥ 2 occasions


Thrombocytopenia--<100,000/ mm3 in the absence of offending drugs

Immunologic Disorder

Anti-DNA: antibody to native DNA in abnormal titer


Anti-Sm: presence of antibody to Sm nuclear antigen


Positive finding of antiphospholipid antibodies on:

1. an abnormal serum level of IgG or IgM anticardiolipin antibodies

2. a positive test result for lupus anticoagulant using a standard method

3. a false-positive test result for at least 6 months confirmed by Treponema pallidum immobilization or fluorescent treponemal antibody absorption test

Positive Antinuclear Antibody An abnormal titer of antinuclear antibody by immunofluorescence or an equivalent assay at any point in time and in the absence of drugs


Þetta vefsvæði byggir á Eplica