DNA immunoadsorption for childhood-onset systemic lupus erythematosus

We present a retrospective review of DNA immunoadsorption (DNA-IA) therapy on clinical symptoms as well as indicators in pediatric cases with systemic lupus erythematosus (SLE), and follow up the short-term curative effects. 16 SLE cases were treated by DNA-IA for 3 times every other day. We observed the changes on clinical manifestations and immunological indicators, in order to compare the alteration of these indicators including clinical manifestations, Systemic Lupus Erythematosus Disease Active Index (SLEDAI) scores, 24 hurinary protein excretion, autoantibodies, serum IgG and complement C3. 13 cases were followed up regularly, within 3 months after DNA-IA therapy, 12 cases of clinical manifestations improved (92.3%). SLEDAI scores in 10 cases decreased from (16.20 ± 12.54) to less than 5 (76.9%), 8 cases of ANA, anti-DNA antibodies were negative (61.5%), 13 cases with IgG level in serum recovered to normal (10.39 ± 4.38) g/L, C3 level rose to normal (1.06 ± 0.23) g/L. 3 to 6 months after IA, clinical manifestations and laboratory examinations in all cases got maximum improved. 9 months after IA, SLEDAI score in 2 cases (15.4%) rose to more than 5, anti-DNA antibody in 2 cases (15.4%) became positive, and 1case (7.7%) with serum C3 decreased again. 2 cases died from multiple organs dysfunction within 3 to 6 months after IA. No serious complications were found during DNA-IA. We recommend that DNA immunoadsorption is a safe and effective therapy for active childhood-onset SLE, which could improve clinical symptoms, eliminate ANA and anti-DNA antibodies. Combining with corticosteroids and immunosuppressive drugs, DNA-IA could significantly reduce the activity of disease and protect vital organs function in the short term.

Anti-nuclear matrix protein 2+ juvenile dermatomyositis with severe skin ulcer and infection: A case report and literature review

BACKGROUND Juvenile dermatomyositis(JDM)is an idiopathic inflammatory myopathy that occurs in childhood.It is characterized by muscle weakness and a characteristic rash.Previous literature reports have rarely described JDM with severe skin ulcers and infections.CASE SUMMARY Herein,we describe a case of a 2-year-old female patient who suffered from JDM,whose myositis-specific autoantibodies were positive for anti-nuclear matrix protein 2 antibody,with progressively worsening skin ulcers and severe infections.The patient was treated with glucocorticoids and various immunosuppressants.Nevertheless,further progression of the disease and the combination of primary disease and severe infection in the later period were fatal.CONCLUSION In children,anti-nuclear matrix protein 2+JDM combined with skin ulcers often indicates severe disease.In such cases,personalized treatment for the primary disease and infection prevention and control are essential.

Infliximab therapy and outcomes in patients with polyarticular juvenile idiopathic arthritis:a single-center study in China

Background Juvenile idiopathic arthritis(JIA)is a chronic inflammatory disease that includes seven heterogeneous subgroups with different prognoses.In particular,polyarticular JIA(pJIA)has a longer period of active disease and a poorer prognosis.Tumor necrosis factor(TNF)-alpha inhibitors are effective in patients with pJIA,but the therapeutic regimen remains controversial.Here,we performed a single-center study to determine the potential correlation between TNF-alpha inhibitor(infliximab)therapy and outcomes in these patients.Methods Clinical data of 40 pJIA patients were collected at our center from January 1,2010 to January 1,2018,and patients were grouped according to the timing of infliximab therapy.The erythrocyte sedimentation rate(ESR),the number of joints with active disease,and the 27-point juvenile arthritis disease activity score(JADAS-27)were analyzed.Results The ESR,the active joint count,and the JADAS-27 decreased significantly in all groups after 3 months(P=0.041/0.415/0.008,0.022/0.030/<0.001,and 0.05/0.012/<0.001,respectively)and 6 months(P=0.036/0.045/0.041,0.076/0.037/<0.001,and 0.096/0.006/<0.001,respectively)of infliximab treatment,although the rates of change of these parameters were similar.However,after 12 months,only patients treated with infliximab within 3 months of disease onset had a stable ESR,active joint count,and JADAS-27,while these parameters increased sharply when infliximab was administered 3 months and especially 1 year after disease onset.Conclusions TNF-alpha is a pleiotropic pro-inflammatory cytokine of crucial importance in the pathogenesis of JIA.Infliximab can improve the outcomes of patients with pJIA significantly,and should be introduced early during the clinical course.