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PReS-FINAL-2293: An attempt to analyze variants of systemic lupus erythematosus onsets in childhood
Pediatric Rheumatology volume 11, Article number: P283 (2013)
Childhood-onset systemic lupus erythematosus (SLE), compared with adult-onset variants, is characterized by severity, variety of organ involvement, unpredictable history and diversity of onset variants.
To analyze and try to systemize variants of SLE onsets among patients of rheumatology department.
We collected and analyzed 27 cases of systemic lupus erythematosus in children (age up to 18 years) admitted to our department from 2006 to 2013.
Among 27 patients there were 21 (78%) females and 6 (22%) males; 11 years was an average age of onset. Variants of SLE onset can be systemized by different ways:
According to time from first manifestation to complete clinical presentation
«Acute» onset: time from first symptom to complete clinical presentation is less than 1 month - 9% (14 of 27) Among such cases were 4 (14,8%) with delayed (5-14 months) new organ involvement. 3 patients presented with renal involvement: 2 cases of microhematuria, proteinuria in patients without cytostatic therapy; 1 case of nephrotic syndrome in patient treated by chloroquine. 2 patients (treated by cytostatic drugs) presented with neuropsychiatric manifestations: seizures, focal cortical neurologic signs.
«Subacute» onset: time from first symptom to complete clinical presentation - from 1 to 3 months - 14,8% (4 of 27). This variant is characterized by progressive, step-by-step involvement of new organ systems without any regularity in priority.
«Prolonged» onset: slow evolution of clinical manifestations (from 8 to 24 months in this research) - 33,3% (9 of 27)
According to inflammatory activity of disease onset
55% of 27 patients presented fever (17 of 27). In 66% cases increased ESR/CRP rate was detected. Fever is more common for patients with «acute» or «subacute» onset variant (61% - 11 of 18), all of them also had increased ESR/CRP rate. Fever also presented in 4 patients of 9 «prolonged» onsets (44%), increased ESR/CRP rate in 7 of 9 (77%). Both fever and increased ESR/CRP rate were detected in different time from onset.
According to primary affected organ system
Dermatologic manifestation (malar rash, photosensitivity, non-specific rash) was the first symptom for majority of patients (44% -12 of 27), 3 of them had both dermatologic manifestation and oral ulcers. 7 patients (25,9%) started their history with different autoimmune cytopenias, including acute haemolytic anemia as the first symptom (2 cases), and acute thrombocytopenic purpura (3 cases). Frank arthritis or arthralgia presented in 7 cases (25,9%) as single symptom or in combination with another manifestations. 3 cases started with thrombosis as manifestation of antiphospholipid syndrome (later confirmed by laboratory tests). There was 1 case with episodes of severe headaches as single complaint for a long time, 1 case with vein thrombosis of leg; one patient with appendicitis and intestinal ulcers (as manifestation of mesenterial vein trombosis). 2 patients had prolonged history (up to 2 years) of non-specific manifestations (fatigue, weakness, weight loss, myalgia) before first clinical symptoms.
It's hard to systemize onsets of systemic lupus erythematosus because of their variability; and this variability causes variations of disease management and treatment tactics.
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Kuchinskaya, E., Dubko, M. PReS-FINAL-2293: An attempt to analyze variants of systemic lupus erythematosus onsets in childhood. Pediatr Rheumatol 11, P283 (2013) doi:10.1186/1546-0096-11-S2-P283
- Systemic Lupus Erythematosus
- Nephrotic Syndrome
- Malar Rash