| Objective: To study the relationship between the clinical manifestations and pathological characteristics of lupus nephritis in children and to analyse the factors related to its prognosis.Methods: A retrospectively research was designed to study the clinical and pathological medical records in children with lupus nephritis from Children's Hospital of Chongqing Medical University from January 2000 to April 2010. And the patients were followed up for the further prognosis. All the data was analysed by statistical software of SPSS 16.0.Result: (1) 104 patients were diagnosed with LN from January 2000 to April 2010. There were 20 males and 84 females, the male to female ratio was 1: 4.2. The mean age of onset was(11.22±2.35)years old ,and the peak age of morbidity ranged from 9 to 15 years old. (2) The signs of renal damages was dominant 50% in all the primary symptoms while the rash was the predominant symptom of the non-renal performance. There were 98 children suffered from the urinary or renal disfunction when primary diagnosed as systemic lupus erythematosus (SLE) and the proportion was 94.23%. (3) Proteinuria was the most common urine change (87.50%), and 27 cases (34.18%) achieved nephrotic level proteinuria. The following change was hematuria (63.46%). (4)The proportion of the positive autoantibody test followed as ssDNA antibody(94.90%), ANA(80.61%), dsDNA antibody(77.50%). The outcome of immunoglobulin assay found IgG , IgA and IgM all increased, which occupied 71.43%, 65.31% and 55.10% respectively, but C3 decreased, which proportion was 80.61%. (5)Among the 40 specimen from patients with renal biopsy, 23 specimen (57.5%) was ClassⅣnephritis, which was the prominant histopathology on initial renal biopsy, 9 specimen (22.50%) was classⅡand 3 (7.50%) wasⅤ+Ⅲ, but ClassⅡ+Ⅲ,ⅤandⅤ﹢Ⅳwere much rare. In clinical manifestations, 12 children (30%) had nephrotic syndrome, 11(27.50%) had hematuria or(and) proteinuria, 9(22.50%) were acute nephritis but chronic nephritis and subclinical types were rare, which were both 2 cases (5.00%). There was no statistical significant distinction between the pathological classifications and various clinical types (χ2=37.365, P=0.139). (6) There were statistical significant differences between the level of urine protein and the various pathological classifications (F=6.822, P=0.04). Nephrotic proteinuria was common in ClassⅣandⅤLN while the urine protein varied from 0.16 to 1.00g/24h in classⅡ,ⅢandⅡ﹢Ⅲ. (7) The activity of the LN disease was correlated to the renal activity index(AI) (r=0.384). As the disease aggravated, the AI increased but the SLEDAI scores had no significant difference in various pathological classifications (F=1.62, P=0.212). There was statistical significant difference between AI and CI in LN with different pathological classification(sF=16.595, P=0.00; F=4.038, P=0.026, AI and CI of classⅣwas highest than others. (8) Among the 40 followed up children, 18(45.00%)were significantly remitted, 9 (22.50%) were partially remitted, 5 (12.50%) had effective treatment, while 8 (20.00%) were accepted ineffective therapy. (9) The risk factors connected to prognosis were hypertension, severe proteinuria prior and corticosteroids only (P﹤0.10). The longer course before diagnosed, the worse outcome it would be(r=0.347, P=0.028).However, the prognosis of lupus nephritis was not related with the factors were as follows, sex, the age of onset, serum creatinine and C3 levels, SLEDAI points and pathological types.Conclusion: (1) Lupus nephritis was more often appeared in female children, the onset age was 9 to 15 years old. (2) Renal damages happened early in the disease course and it consisted one half of all the chief complaints. (3) Essentially all the pathologic types can appear but we found classⅣhistopathology was dominant and the most common urine change was proteinuria. There was no correlation between pathological classifications and clinical types. (4) The level of urine protein was related to the various pathological classifications. (5) Systemic disease activity accompanied with renal activity but there was no correlation between them. (6) The prognosis of LN was influenced by many factors, in this study, we found hypertention, severe proteinuria prior and corticosteroids only were the risk factors. The longer course before diagnosed, the worse prognosis it would be. |
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