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Systemic lupus erythematosus (SLE)is a prototypic autoimmune disease characterized by multisystemic organ involvement and production of multiple autoantibodies. The clinical manifestations of patients varied,ranging from mild joint pain and skin involvement to life-threatening internal organs involvement. In clinical practice,it is not common to have severe liver damage or even fulminant hepatic failure due to SLE disease itself. Liver is not the main organ to be involved in SLE,but abnormal elevation of liver enzyme is common in SLE. Liver biopsy is the gold standard for definite diagnosis. This article summarized the current reports of SLE with liver damage and analyzed the pathological changes of liver lesions due to SLE disease itself for improving the understanding of histopathology profile of SLE complicated with liver damage.
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Objective· To investigate the clinical features of macrophage activation syndrome (MAS) associated with adult-onset Still's disease (AOSD),and provide the basis for clinical diagnosis and treatment of the disease.Methods· The clinical data of 42 patients with AOSD,including 14 patients with AOSD-induced MAS (the MAS group) and 28 AOSD patients paired by age and sex (the non-MAS group),diagnosed in Department of Rheumatology,Renji Hospital,Shanghai Jiao Tong University School of Medicine from October 2013 to June 2016 were collected and then retrospectively analyzed.Results· There was no significant difference in age,sex and duration of AOSD between two groups.The mortality rate of patients in MAS group was significantly higher than that of patients in non-MAS group,as well as the rates of rash,splenomegaly and hemophagocytosis.The levels of ALT and serum ferritin in MAS group were higher than those in non-MAS group,while the level of FDP is lower.Glucocorticoids were used in all 42 patients,and the dosage of glucocorticoids was significantly higher in MAS group than non-MAS group.Only 1 patient with AOSD-induced MAS received MTX,the percentage of patients receiving MTX was significantly lower in MAS group than non-MAS group.Five patients with AOSD-induced MAS received IVIG,the percentage of patients receiving IVIG was significantly higher in MAS group than non-MAS group.Two patients with AOSD-induced MAS received VP-16.Conclusion · The mortality rate of patients in MAS group was significantly higher than that of patients in non-MAS group,as well as the rates of rash,splenomegaly and hemophagocytosis.The levels of ALT and serum ferritin in patients with AOSD-induced MAS were higher than patients without MAS,while the level of FDP was lower.Early diagnosis and active treatment is the key point to improve clinical outcome.
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Systemic lupus erythematosus is a kind of autoimmtne disease which may lead to multisystem involvement,with high mortality.As is well known,the prognosis of children is worse than that of adults.Many factors such as genetic factor,environmental factor and the self factors affect the survival of children with systemic lupus erythematosus.In order to improve the diagnosis,treatment and prognosis,this article reviews the recent research of the main factors affecting the prognosis of children with systemic lupus erythematosus.
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Objective To evaluate the influence of atopy on the prognosis of juvenile-onset systemic lupus erythematosus (JSLE).Methods The study was performed on 60 cases with JSLE diagnosed at the Department of Pediatrics,Renji Hospital Affiliated to School of Medicine of Shanghai Jiaotong University from October 2005 to April 2015.These patients were enrolled by mixed cohort study and subdivided into atopic group(26 cases) or non-atopic group(34 cases).The clinical and laboratory data of the disease onset,disease assessment scores,medications during follow-ups and remission/flare of the disease were recorded and analyzed to compare the difference between 2 groups.Results (1) The systemic lupus erythematosus disease activity index (SLEDAI) score [(17.080 ± 5.628) scores vs (12.590 ± 4.856) scores],anti-double-stranded DNA (anti-dsDNA) [(62.590 ± 43.602) IU/mL vs (40.230 ±30.189) IU/mL],erythrocyte sedimentation rate (ESR) [(59.150 ± 40.315) mm/1 h vs (40,350 ± 31.865)mm/1 h] were significantly elevated at onset in the atopic group compared with non-atopic controls (all P ＜ 0.05),while the complement C3[(0.450 ±0.218) g/L vs(0.640 ±0.333) g/L],C4 [(0.047 ±0.024) g/L vs(0.116 ±0.172) g/L] in atopic group was lower than those in the non-atopic group (all P ＜ 0.05).(2)During the follow ups of 1 and 6 months to 1 year,the JSLE patients with atopy always had higher SLEDA1 score compared with the non atopic controls(all P ＜ 0.05).(3)For medications,the daily cumulative glucocorticoid dose received by patients in the atopic group were larger than that of the non-atopic group,and the number of immunosuppressive agents used in the atopic group was more than that in the non-atopic controls (P ＜ 0.05).(4) During the 1-year follow-up,the rate of disease relapse in the atopic group was higher than that in the non-atopic group and the atopic group also needed much more time to reach disease remission (P ＜ 0.05).Conclusion JSLE patients combined with atopy may have an adverse influence on the prognosis of JSLE.
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Objective To explore the influence of allergic rhinitis (AR) on patients with systemic onset juvenile idiopathic arthritis (SoJIA).Methods The study involved 44 cases with SoJIA from Department of Pediatrics,Renji Hospital affiliated to School of Medicine of Shanghai Jiaotong University from July 2008 to November 2013.The Clinical and laboratory data of all patients were recorded respectively.This was a retrospective cohort study.According to the diagnosis of allergic rhinitis (AR),children were subdivided into AR group (16 cases) and Non-AR group (28 cases).ACR Pediatric criteria (ACR Pedi) 30/ 50/70 and related indicators of SoJIA between the two groups were compared.In the AR group,the correlation between AR scores and DAS28 was analyzed.When SoJIA of the two groups relapsed,the AR group (the treatment group) received anti-rheumatism for arthritis as well as nasal spray and oral antihistamines for AR.The non-AR group (control group) only received the anti-rheumatism for arthritis.The improvement of SoJIA between the two groups was analyzed.The continuous variables were analyzed by Student's t-test or the MannWhitney U-test as appropriate.Categorical data were compared between different groups by the Chi-square test.Correlations were determined by Pearson or Spearman's ranking.Results ① In the retrospective analysis:the physician's and patients'/parents' general assessment on a 10 cm visual-analogue scale (VAS),number of joints with res-triction of movement,number of swollen joints,ESR,serum ferritin(SF) and childhood health assessment questionnaire (CHAQ) score were significantly elevated in the AR group compared with Non-AR group at the disease onset [(6.7±1.0) cm vs (4.8±1.9) cm; (6.5±1.4) cm vs (3.2±1.5) cm; 4.1±2.7 vs 2.7± 1.7; 3.4±2.4 vs 1.4±1.5; (87±35) mm/1 h vs (61±40) mm/1 h; (888±1 043) μg/L vs (311±324) μg/L; 1.6±0.5 vs 0.7±0.3,respectively; all P＜0.05].At the 3 and 6 months follow-up after disease onset,the proportion of patients who reached ACR pedi 50,70 in AR group were lower than the Non-AR group,while the cumulative glucocorticoid dose was higher in the AR group than that of those without AR [at 3 months 38％ vs 71％; 13％ vs 46％; (76±35) mg/kg vs (43±36) mg/kg,respectively; at 6 months 25％ vs 71％; 19％ vs 64％; 127±57 vs 67±58,respectively,all P＜0.05]; In the AR group,at the disease onset,3 and 6 months follow-up after disease onset,the scores of AR was positively correlated with DAS28(r=0.741,0.703,0.680,respectively; all P＜0.05).② In the prospective study:when SoJIA was relapsed,systemic feature score,the physician's and patients' /parents' general assessment on a l0 cm VAS,number of joints with restriction of movement,number of swollen joints,ESR,SF and CHAQ score were significantly elevated in the treatment group compared with the control group [3.8±1.5 vs 2.1±1.1; (5.6±1.5) cm vs (4.5±1.6) cm; (4.6±1.9) cm vs (3.1±1.5)cm; 3.9±1.9 vs 2.5±1.4; 2.4±0.9 vs 1.5±1.2; 92±27 vs 53±37; 565(339,1 192) μg/L vs 171(85,284) μg/L; 13(0.8,1.6) vs 0.7(0.5,1); respectively; P＜0.05].The improvement rate of the physician's and patients'/parents' general assessment on a 10 cm VAS,number of swollen joints,number of joints with restriction of movement,ESR and CHAQ score at the follow-up 3 months were higher in treatment group than the control group [71(55,86)％ vs 46(0,75)％; 67(45,81)％ vs 28(-4,62)％; 92(77,96)％ vs 70(27,88)％; 65(48,81)％ vs 0(-17,67)％; 100(46,100)％ vs 42(0,100)％; 67(49,85)％ vs 37(0,75)％; P＜0.05].At the follow-up 6 months,the improvement rate of ESR,patients'/ parents' general assessment on a 10 cm VAS,number of joints with restriction of movement and CHAQ score were higher than control group [94(85,96)％ vs 73(33,85)％; 89(65,99)％ vs 63(5,85)％; 100(100,100)％ vs 100(0,100)％; 91(69,100)％ vs 72(11,91)％; respectively,P＜0.05].Conclusion AR may exert an adverse influence on SoJIA.SoJIA patients who are treated with combined with AR may have better outcome than those who are only treated for arthritis.
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Objective To evaluate the inlluence of atopy on juvenile idiopathic arthritis (JIA).Methods The study involved 117 cases with JIA from Department of Pediatrics,Renji Hospital Affiliated to School of Medicine of Shanghai Jiaotong University from Jul.2008 to Jul.2013.These patients were enrolled for retrospective cohort study,and subdivided into JIA and atopic group or JIA and non-atopic group.There were 34 cases combined with atopy,83 cases without atopy.Based on the diagnosis of allergic rhinitis (AR),those JIA children in the atopic group were organized into AR group (19 cases) and non-AR group (15 cases).The clinical and laboratory data were recorded and analyzed to compare the differences of the remission of American College of Rheumatology Pediatric (ACR Pedi) 30/50/70 between atopic group and non-atopic group,AR group and non-AR group.In AR group,the correlation between AR scores and disease activity score (DAS28) was analyzed.Results 1.The physician's and patients'/parents' general assessment on a 100 mm visual-analogue scale (VAS),number of joints with restriction of movement and childhood health assessment questionnaire (CHAQ) were significantly elevated in atopic group compared with controls at the beginning (all P ＜ 0.05).In the follow-up 3 months after disease onset,the proportion of reaching ACR Pedi 30,50 and the proportion of reaching ACR Pedi 50,70 in 6 months later in JIA with atopy were lower than JIA children without atopy (all P ＜ 0.05) ; In the follow-up 3 and 6 months,the cumulative glucocorticoid dose was higher in atopy group compared with Non-atopy,which showed a statistical significance (all P ＜ 0.05).2.Among the AR group,at the disease onset,the physician's and patients'/parents' VAS,number of joints with restriction of movement and CHAQ were elevated in AR group compared with controls with statistical significance (all P ＜ 0.05).In the follow-up 3 months,the proportion of reaching ACR Pedi 30 and 50 was lower in AR group compared with non-AR group.In the follow-up 6 months,the cumulative glucocorticoid dose was higher in AR group compared with non-AR,which showed a statistical significance.But the ratio of ACR Pedi 30,50 and 70 were lower in AR group compared with non-AR group (all P ＜ 0.05).Among JIA combined with AR,at the beginning,follow-up 3 and 6 months after disease onset,the scores of AR positively correlated with DAS28 (r =0.671,0.518,0.496,all P ＜ 0.05).Conclusion Atopy or AR may exert an adverse influence on JIA.
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Allergic rhinitis and bronchial asthma are two common respiratory allergic diseases in children and the incidence of the two diseases is increasing.They are regarded as mutually separate diseases,but recent studies suggest that there is a strong link between the two diseases,which is represented at the etiology,mechanisms,pathologic and therapeutic levels.So combined allergic rhinitis and asthma syndrome (CARAS),a new terminology has formally introduced to illustrate concurrent symptoms of allergic rhinitis and bronchial asthma.The diagnosis and treatment of CARAS need to be considered collectively.Therefore the incidence of misdiagnosis and repeated use of drugs can be significantly reduced,then improving the clinical efficacy.This article reviews progress of the diagnosis and treatment of CARAS in children.
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Children's rheumatic diseases,characterized with long disease course,systemic involvement,high morbidity and mortality rate and poor prognosis,deserve further attention in long-term treatment and follow-up.For now,there are many factors involved in the pathogenesis of rheumatic diseases,particularly the role of atopy atracted controversy.According to the conventional theory,Th1-type autoimmune diseases and Th2-mediated allergic diseases may have mutual antagonism in the pathogenesis,or that both kinds of diseases can co-exist and may contribute to a reduced disease severity.However,recent studies show that atopy may exert an adverse influence on children's rheumatic diseases,including higher severity,relapse-susceptibility,protracted remission and poorer outcomes.Recent researches have found that the underlying mechanisms of atopy on the development of rheumatic diseases can not be explained by the conventional Thl/Th2 theory.A complicated regulatory network of immune system,including Th17,Treg,NK,macrophagocyte,etc,may play a significant role in the development of the two kinds of diseases.This article provides up-to-date review on the immunological influence of atopy on children's rheumatic diseases at home and abroad.