| Objective: To investigate the clinical features, disease activity, treatment andprognosis of children with systemic lupus erythematosus for early diagnosis of thedisease and providing the basis for rational treatment.Methods: We retrospectively collected and summarized clinical data, includingthe first symptoms, clinical manifestations, laboratory tests, imaging examination,pathology, systemic lupus erythematosus disease activity index and treatment, the of65cases of children with SLE who received treatment in China-Japan Union Hospital fromJanuary2003to June2013. We also made the appropriate statistical analysis.Results:65cases of children with SLE, the male to female ratio was1:4.42. Theaverage age was11.28±3.12years. The peak incidence was10to15-year-old elderfemale children. The most common first symptom was fever73.8%(48/65), followedby rash50.8%(33/65), kidney damage43.1%(28/65), joint pain23.1%(15/65),anemic7.7%(5/65), headache, fatigue, abdominal pain each accounted for1.5%(1/65). The most common system organ damage was renal involvement(83.1%), Themost common renal involvement accounted for83.1%(54/65), followed by bloodsystem73.8%(48/65), respiratory system33.8percent (22/65), digestive system30.8%(20/65), nervous system23.1%(15/65), cardiovascular system18.5%(12/65).Laboratory test: hemoglobin decrease69.2%(45/65), reduction of red blood cellsaccounted for66.2%(43/65), thrombocytopenia49.2%(32/65),33.8%decrease inwhite blood cells (22/65), proteinuria49.2%(32/65), hematuria,46.2%(30/65),urinary tube accounted for12.3%(8/65), BUN increase accounted for33.8%(22/65),ALT increased40%(26/65), AST increased36.9%(24/65), albumin decreased26.2%(17/65), globulin increased18.5%(12/65), elevated blood lipids accounted for16.9%(11/65), ESR faster accounted for36.9%(24/65), CRP increases accounted for23.1%(15/65), IgG increases accounted for43.1%(28/65), IgM increased18.5%(12/65) C3reduce representing43.1%(28/65), C4reduced representing40%(26/65).Laboratory antinuclear antibodies tests showed that ANA-positive rate was89.2%(58/65), followed by anti-ds-DNA antibody64.6%(42/65), anti-Sm antibodies 33.8%(22/65), anti-SSA antibodies (+)18.5%(12/65), anti-SSB antibodies (+)12.3%(8/65), anti-RNP antibodies (+)9.2%(6/65), anti-Scl-70antibody (+)7.7%(5/65).22cases of children with respiratory involvement, there are18cases to checkthe lungs CT,4patients diagnosed with interstitial pneumonia,6cases diagnosed withpleural effusion.The head CT or MRI investigation of9cases with nervous systeminvolvement showed brain atrophy.5case showed type IV of10cases with renalbiopsy and pathology.32cases were assessed on the basis of SLEDAI. The averagescore was11.59±2.85points.The score was related to onset time to certain extent.26cases(81.3%) got10points or more showing the moderate to severe activity. Exceptfor ANA-positive rate, there was no significant difference between boys and girls inthe age of onset, laboratory test, clinical manifestations and SLEDAI scores. Thegroup misdiagnosis rate was23.1%(15/65). Follow-up from1to8years, with anaverage6.11±1.22years,5-year survival rate was81.5%(53/65). Completeremission rate was55.4%(36/65), partial remission rate was21.5%(14/65),ineffective was7.7%(5/65). The course of treatment,10died (15.4%), which died ofinfection in6cases (2cases of sepsis,3cases of tuberculosis,1case of cryptococcalmeningitis),2patients died of kidney failure,2patients died of neuropsychiatric lupus(neuropsychiatric lupus erythematosus, NPSLE).Conclusions: Children SLE were mainly the female children around puberty.The first symptoms of fever, rash, arthralgia and kidney damage were common. Themost common system organ damage was renal involvement, followed by bloodsystem, respiratory system and digestive system. In antinuclear autoantibodies test,the positive rate of ANA was the highest, followed by anti-ds-DNA antibodies,anti-Sm antibodies. Clinical manifestations of children SLE were complex and manysystem organs were involved. Clinical diagnosis was difficult and prone tomisdiagnosis. The activity of children with SLE were mostly moderate to severe.Diagnosed time, SLEDAI score, whether formal follow-up treatment and whetherco-infection were important prognostic factors. More attention should be paid to thechildren with the mentioned performances, especially female children aroundadolesce. Antinuclear autoantibody tests should be done as soon as possible, in orderto facilitate the early definite diagnosis. Refering SLEDAI score and insisting regularfollow-up treatment to improve prognosis. However, when the children combindedinfection, renal and nervous system involving would got poor prognosis and high mortality. |
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