12. tbl. 97. árg. 2011
Cry wolf
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 OR 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 OR 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 OR Psychosis--in the absence of offending drugs or known metabolic derangements, e.g., uremia, ketoacidosis, or electrolyte imbalance |
| Hematologic Disorder |
Hemolytic anemia--with reticulocytosis OR Leukopenia--< 4,000/mm3 on ≥ 2 occasions OR Lyphopenia--< 1,500/ mm3 on ≥ 2 occasions OR Thrombocytopenia--<100,000/ mm3 in the absence of offending drugs |
| Immunologic Disorder |
Anti-DNA: antibody to native DNA in abnormal titer OR Anti-Sm: presence of antibody to Sm nuclear antigen OR 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 |