Serum immunoglobulin profiles in Chinese children with Henoch-Schönlein purpura.

The present study focuses on the associations of serum immunoglobulin with disease activity, relapse/recurrence and renal involvement in Henoch-Schönlein purpura (HSP) children. 1683 Chinese children with HSP were recruited from January 2015 to January 2021. Laboratory data of blood samples and urine tests were collected. Renal biopsy was performed by the percutaneous technique. Patients' histories associated with HSP onset were obtained by interviews and questionnaires. (1) IgA and IgE were dramatically elevated in HSP patients as compared to their normal values and subject to a 1.75-3.09 fold and a 1.97-2.61 fold increase, respectively. (2) No significant correlation of the disease activity with IgA and IgE was determined, respectively. (3) The serum IgA levels were significantly lower in patients with relapse/recurrence than that in patients without relapse/recurrence, which may be attributed to the transmission of IgA-mediated immune complexes from blood to vessel walls. (4) No significant correlation was found between serum IgA, IgE levels and the pathological classification. HSP children have marked disorders of serum immunoglobulin profiles, characterized by significant increases in IgA and IgE. The detection of serum IgA may be applicable to predict relapse/recurrence of HSP whereas not associated with disease activity and renal involvement.

A new scoring system for coronary artery abnormalities in Kawasaki disease.

BACKGROUND: In China, coronary artery abnormalities (CAAs) secondary to Kawasaki disease (KD) tend to have an increased occurrence. We hypothesize that Chinese children with KD may possess several unique CAA risks, and the predictive efficacy of multiple scoring systems in Chinese patients are still to be further studied. METHODS: Two hundred and three KD patients were recruited. Using multivariable analysis, independent predictors of CAAs were combined into a scoring system. Subsequently, CAA risks of our patients were evaluated by the newly established scoring system and eight other published scoring systems. RESULTS: Seventeen (8.37%) KD patients were identified as CAAs. The newly established scoring system contained the following 5 independent predictors: days of illness at initial treatment ≥7, redness and swelling of extremities, hematocrit ≤33%, percentage of monocytes ≥8.89%, and procalcitonin ≥0.5 ng/mL. The AUC value of newly established scoring system was 0.685 with a sensitivity of 41.18% and a specificity of 84.41%, higher than Harada score, Egami score, Kobayashi score, Sato score, San Diego score, Formosa score, and Tang score, whereas lower than Hua score. CONCLUSIONS: Days of illness at initial treatment ≥7 and procalcitonin are unique predictors of CAAs in newly established scoring system. Taking into account different identification criteria and analytical methodologies, there is still some heterogeneity among different scoring systems. IMPACT: The newly established scoring system contains the five independent predictors. Days of illness at initial treatment ≥7 and PCT are unique predictors of CAAs in our study, compared with 8 other systems. The AUC value of newly established scoring system is 0.685, similar to Hua score. There is some heterogeneity among different scoring systems.

Clinical implications of procalcitonin in Kawasaki disease: a useful candidate for differentiating from sepsis and evaluating IVIG responsiveness.

OBJECTIVE: Kawasaki disease (KD) is an acute systemic vasculitis and suspected to be triggered by several potential infections in which procalcitonin (PCT) experiences an increase to some extent. However, whether PCT can serve as a useful candidate for differentiating KD from sepsis, and even for predicting incomplete KD, intravenous immunoglobulin (IVIG) nonresponsiveness and coronary artery abnormalities (CAAs) remains unclear. METHODS: A total of 254 Chinese KD children were enrolled and divided into 6 subgroups, including complete KD, incomplete KD, IVIG-responsive KD, IVIG-nonresponsive KD, KD with CAAs and KD without CAAs. Blood samples were collected from all subjects within 24-h pre- and 48-h post-IVIG infusion, respectively. PCT, C-reactive protein, erythrocyte sedimentation rate and blood cell counts were detected. In addition, both 261 children with sepsis and 251 healthy children sex- and age-matched with KD children were enrolled in the same period. RESULTS: (1) PCT experienced the highest increase in sepsis patients before antibiotic therapy, followed by acute KD patients and the healthy controls. (2) The proportion of KD patients with a PCT concentration below 0.25 ng/ml was 11 folds higher than that of sepsis patients. (3) PCT had a sensitivity of 91.7% and a specificity of 30.3% at a cutoff value of > 0.15 ng/ml to predict IVIG nonresponsiveness, and the proportion of IVIG-nonresponders with a PCT concentration of 0.25-0.50 ng/ml was 2 folds higher than that of IVIG-responders. CONCLUSIONS: The PCT concentrations below 0.25 ng/ml may be useful for discriminating KD from sepsis, and moreover, the PCT concentrations of 0.25-0.50 ng/ml may be helpful in predicting IVIG nonresponsiveness.

Streptococcal infection in childhood Henoch-Schönlein purpura: a 5-year retrospective study from a single tertiary medical center in China, 2015-2019.

BACKGROUND: The present study focuses on the associations of streptococcal infection with the clinical phenotypes, relapse/recurrence and renal involvement in Henoch-Schönlein purpura (HSP) children. METHODS: Two thousand seventy-four Chinese children with HSP were recruited from January 2015 to December 2019. Patients' histories associated with HSP onset were obtained by interviews and questionnaires. Laboratory data of urine tests, blood sample and infectious agents were collected. Renal biopsy was performed by the percutaneous technique. RESULTS: (1) Streptococcal infection was identified in 393 (18.9%) HSP patients, and served as the most frequent infectious trigger. (2) Among the 393 cases with streptococcal infection, 43.0% of them had arthritis/arthralgia, 32.1% had abdominal pain and 29.3% had renal involvement. (3) 26.1% of HSP patients relapsed or recurred more than 1 time within a 5-year observational period, and the relapse/recurrence rate in streptococcal infectious group was subjected to a 0.4-fold decrease as compared with the non-infectious group. (4) No significant differences in renal pathological damage were identified among the streptococcal infectious group, the other infectious group and the non-infectious group. CONCLUSIONS: Streptococcal infection is the most frequent trigger for childhood HSP and does not aggravate renal pathological damage; the possible elimination of streptococcal infection helps relieve the relapse/recurrence of HSP.