| Objective: To analyze the clinical characteristics,treatment outcome and prognosis of cSLE complicated with different severity of thrombocytopenia,to provide the basis for clinical diagnosis,treatment and prognosis evaluation of this disease.Methods: The clinical data of cSLE patients with different severity thrombocytopenia diagnosed in the Department of Pediatric Rheumatic Immunology in our hospital from January 2008 to May 2019 were analyzed retrospectively,including demographic characteristics,clinical features,laboratory indicators,treatment and five-year follow-up mortality rate.Results: A total of 80 cSLE patients with thrombocytopenia were included in this study,accounting for 34.3% of the patients diagnosed with cSLE in the same period.General data: There were 57 patients with mild to moderate thrombocytopenia(71.25%)with the median platelet count 62,000/mm3,23 patients with severe thrombocytopenia(28.75%)with the median platelet count 7,000/mm3.The ratio of male to female was 1:3.7.The overall median age of onset was 10.8 years with a median duration of 51.5 days before the diagnosis Clinical manifestations and laboratory features: Compared to those in the mild to moderate thrombocytopenia group,the patients in the severe thrombocytopenia group had lower incidences of rash,hematuria and proteinuria,higher incidence of nervous system involvement and mucosal hemorrhage,lower SLEDI score,higher white blood cell count,lymphocyte count,C-reactive protein(CRP),Ig G,C3 and C4 levels,lower C4 reduction rate and anti-ds DNA antibody positive rate.All above were statistically significant(P<0.05).Bivariate correlation analysis showed that platelet count was negatively correlated with white blood cell count,neutrophil count,lymphocyte count,CRP,complement C3 and C4 at the time point of the lowest platelet count(P<0.05).Treatment and effect evaluation: Versus the mild to moderate thrombocytopenia group,the severe thrombocytopenia group was characterized by more frequent intravenous infusion of IG and platelet,lower rate of hydroxychloroquine application,lower post-treatment complete remission rate and higher recurrence rate after complete remission.The differences above were statistically significant(P <0.05).Univariate Cox regression analysis model showed that the five-year follow-up death risk was 6.436 times higher in patients with than that of patients without nervous system involvement(95%CI 2.327-17.801 P<0.001),and 6.546 times higher in patients with incomplete remission after thrombocytopenia treatment than that of patients with complete remission(95%CI 2.349-18.241 P<0.001).The high hemoglobin level at the time of the lowest platelet count was a protective factor for prognosis,HR=0.973(95%CI 0.955-0.992 P=0.005).Multivariate Cox regression analysis after adjustment of confounding factors demonstrated that neurological involvement(HR=4.070 95%CI 1.426-11.619 P=0.009)and incomplete remission of thrombocytopenia after treatment(HR=5.448 95%CI 1.789-16.592 P=0.003)were independent risk factors for death after five-year follow-up,whereas high hemoglobin level at the time of the lowest platelet count was an independent protective factor(HR=0.979 95%CI 0.963-0.997 P=0.019).Conclusions:(1)cSLE with severe thrombocytopenia often occurs in relatively inactive cSLE patients.(2)The treatment of cSLE patients with thrombocytopenia is complicated.The patients with severe thrombocytopenia are more likely to be given platelet transfusion plus IVIG therapy to relieve emergency and critical clinical symptoms.After treatment,the incomplete remission rate and relapse frequency after complete remission in patients with severe thrombocytopenia were significantly higher than those in patients with mild and moderate thrombocytopenia,suggesting that severe thrombocytopenia may be difficult to be treated.(3)The high hemoglobin level at the time of the lowest platelet count is an independent protective factor for the five-year follow-up death rate of cSLE with thrombocytopenia.Whereas,incomplete remission of thrombocytopenia after treatment and nervous system involvement,rather than the severity of thrombocytopenia,are independent risk factors. |
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