RESUMO
Objective:To investigate the clinical characters and prognosis of patients with systemic-onset juvenile idiopathic arthritis associated interstitial lung disease (SoJIA-ILD).Methods:Clinical manifestations, imaging features and prognosis were analyzed in 75 patients with SoJIA between October 2010 and December 2019 in the Second Affiliated Hospital of Wenzhou Medical University.Results:Seventy-five patients with SoJIA were enrolled. Among 12 children with SoJIA-ILD, 6 were male and 6 were female. The mean age of onset was (7.9±2.6) years. All of the 12 patients had fever. Ten patients had arthritis which mainly occurred in large joints. The incidence of arthritis was knee, hip and shoulder from high to low. Two patients had no joint involvement at the onset of the disease and had no joint symptoms during the follow-up. Nine patients (75%) had fever, rash and arthritis at the same time. The clinical features of ILD were mostly nonspecific, including cough in 8 cases (75%), shortness of breath in 7 cases (58%), chest pain in 3 cases (25%), velcro sound in 4 cases (33%) and pulmonary hypertension in 1 case (8%). Inflammatory indicators were all signifi-cantly elevated, among which was CRP (235±112) mg/L, ESR (39±25) mm/1 h, serum ferritin (SF) (1 312±384) ng/ml and serum amyloid A (SAA) (212±101) mg/L. High resolution computed tomography (HRCT) of the chest presented as reticular or line shadows in 12 patients, consolidation in 7 patients, ground interlobular septal thickening in 5 patients, glass opacity in 4 patients and honeycomb lung in 1 patient. ILD occurred in 4 cases (33%) in the early stage of SoJIA (disease course ≤6 months), and 8 cases (67%) in the medium and late stages of the disease course (>6 months), but all appeared in the active status of SoJIA. All of 12 patients received glucocorticoids therapy, 11 patients received high dose of glucocorticoid (>1 mg·kg -1·d -1) and 2 pa-tients received intravenous methylprednisolone pulse therapy. All of 12 patients were treated with glucocorti-coids combined with immunosuppressant or disease modifying antirheumatic drugs and 5 patients needed dual therapy or triple therapy. One case had been treated with biological agents before the occurrence of lung injury and the other 11 cases had not used biological agents before. After the diagnosis of SoJIA complicated with ILD, 4 cases were treated with tocilizumab. Macrophage activation syndrome (MAS) was found in 7 cases and 25% had MAS for two times or more. Ten patients had partial remission or complete remission and 2 patients died of respiratory failure. Conclusion:SoJIA-ILD maybe asymptomatic at the early stage of the disease. It is associated with disease activity of SoJIA. HRCT examination is very important for early diagnosis. Patients with SoJIA-ILD have higher rate of recurrence, death and MAS. It needs to arouse the clinicians' attention.
RESUMO
Objective To investigate the mechanism of epidermal growth factor receptor-forkhead transcription factor A2 (EGFR-FOXA2) pathway-involved high secretion of mucus in human bronchial epitheli-um (HBE) cells after respiratory syncytial virus (RSV) infection and to evaluate the effects of intervention using agonist ( rosiglitazone ) and antagonist ( GW9662 ) of peroxidase proliferation activated receptor γ( PPARγ) and EGFR inhibitor ( AG1478 ) . Methods HBE cells were randomly divided into six groups: A group ( AG1478+RSV) , B group ( rosiglitazone+RSV) , C group ( GW9662+RSV) , D group ( RSV) , E group (0. 1％ dimethyl sulfoxide DMSO) and F group (HBE cell control group). Two hours before RSV infection, A, B and C groups were respectively treated with 10 μmol/L of AG1478, rosiglitazone and GW9662. Expression of EGFR, PPARγ and FOXA2 at mRNA level in each group was detected by real-time fluorescence quantitative RT-PCR 12 h, 24 h and 48 h after HBE cells were infected with or without RSV. Expression of phosphorylated-EGFR ( p-EGFR) and EGFR at protein level was detected by Western blot. ELISA was performed to measure the expression of mucin-5AC (MUC5AC). Results Compared with F group, EGFR expression at mRNA lev-el, p-EGFR/EGFR protein ratio and MUC5AC expression at protein level were increased in a time-dependent manner in A, B, C and D groups at 12 h, 24 h and 48 h. Compared with group F, the expression of PPARγat mRNA level in A, B, and D groups increased at each time point. Moreover, PPARγ expression gradually in-creased over time in A and B groups, reaching the peaks at 48 h, but was in decline in D group. Expression of FOXA2 at mRNA level in RSV-infected HBE cells was declined at each time point compared with that in group F, especially in D group. Compared with group D, A and B groups showed significantly decreased EGFR ex-pression at mRNA level, p-EGFR/EGFR protein ratio and MUC5AC expression at protein level, but markedly increased FOXA2 expression at mRNA level. Conclusions RSV infection increased the expression of MUC5AC at protein level in HBE cells. PPARγand EGFR-FOXA2 signaling pathways were involved in the hypersecretion of airway mucus during RSV infection.
RESUMO
Objective To observe the expressions of peroxisome proliferator activatedγ (PPARγ),IL-17 and calcium activated family member 1 (hCLCA1) mRNA and protein in A549 cell infected with respiratory syncytial virus(RSV),and to explore the effect of rosiglitazone and anti-IL-17 Ab.Methods A549 cells were seeded in 6-well culture plates and randomly divided into six groups.DMSO/RSV group received medium containing 0.02％ DMSO 30min prior to RSV infection.Rosiglitazone/RSV group received rosiglitazone (101μmol/L) 30 min prior to RSV infection.GW9662/rosiglitazone/RSV group was first exposed to GW9662 (10μmol/L)for 30 min,prior to rosiglitazone and RSV infection.Anti-IL-17 Ab (0.15μmol/L) was administered to anti-IL-17 Ab/RSV group 2 hour prior to RSV infection.Supernatants were harvested at 6h,12h and 24h post-RSV infection,retrospectively.The expression of PPARγ,IL-17,hCLCA1 mRNA were measured by real-time quantitative PCR.Protein levels of hCLCA1 and IL-17 were measured by Western blot and ELISA,retrospectively.Results The levels of PPARγ,IL-17and hCLCA1 mRNA and protein were higher in DMSO/RSV group than those in cell control group (P ＜ 0.05) at three time points.The expression of IL-17,hCLCA1 mRNA and protein were lower both in rosiglitazone/RSV group and anti-IL-17 Ab/RSV group than those in DMSO/RSV group at the same time (P ＜0.05).While,both IL-17,hCLCA1 protein and mRNA expression in GW9662/Rosiglitazone/RSV group were similar to that in DMSO/RSV group (P＜ 0.05).Compared with that at 12h and 24h,rosiglitazone and anti-IL-17 Ab had the strongest inhibition effect onthe expression of IL-17 mRNA and protein at 6h (P ＜ 0.05).Conclusion Rosiglitazone can inhibit RSV-induced expression of IL-17,hCLCA1 and may inhibit airway mucus hypersecretion by increasing the activity of PPARγ.
RESUMO
Objective To observe the expressions of peroxisome proliferator activatedγ (PPARγ),IL-17 and calcium activated family member 1 (hCLCA1) mRNA and protein in A549 cell infected with respiratory syncytial virus(RSV),and to explore the effect of rosiglitazone and anti-IL-17 Ab.Methods A549 cells were seeded in 6-well culture plates and randomly divided into six groups.DMSO/RSV group received medium containing 0.02％ DMSO 30min prior to RSV infection.Rosiglitazone/RSV group received rosiglitazone (101μmol/L) 30 min prior to RSV infection.GW9662/rosiglitazone/RSV group was first exposed to GW9662 (10μmol/L)for 30 min,prior to rosiglitazone and RSV infection.Anti-IL-17 Ab (0.15μmol/L) was administered to anti-IL-17 Ab/RSV group 2 hour prior to RSV infection.Supernatants were harvested at 6h,12h and 24h post-RSV infection,retrospectively.The expression of PPARγ,IL-17,hCLCA1 mRNA were measured by real-time quantitative PCR.Protein levels of hCLCA1 and IL-17 were measured by Western blot and ELISA,retrospectively.Results The levels of PPARγ,IL-17and hCLCA1 mRNA and protein were higher in DMSO/RSV group than those in cell control group (P ＜ 0.05) at three time points.The expression of IL-17,hCLCA1 mRNA and protein were lower both in rosiglitazone/RSV group and anti-IL-17 Ab/RSV group than those in DMSO/RSV group at the same time (P ＜0.05).While,both IL-17,hCLCA1 protein and mRNA expression in GW9662/Rosiglitazone/RSV group were similar to that in DMSO/RSV group (P＜ 0.05).Compared with that at 12h and 24h,rosiglitazone and anti-IL-17 Ab had the strongest inhibition effect onthe expression of IL-17 mRNA and protein at 6h (P ＜ 0.05).Conclusion Rosiglitazone can inhibit RSV-induced expression of IL-17,hCLCA1 and may inhibit airway mucus hypersecretion by increasing the activity of PPARγ.
RESUMO
<p><b>OBJECTIVE</b>To investigate the etiology and clinical manifestation of hemoptysis in children.</p><p><b>METHOD</b>A retrospective analysis was performed for 106 cases of hemoptysis who were admitted to The Second Affiliated Hospital & Yuying Children's Hospital of Wenzhou Medical University from January 2005 to December 2014.The clinical information including laboratory tests and image data were collected and analyzed.</p><p><b>RESULT</b>A total of 106 patients (50 males and 56 females) were identified. The median age was 9.1 years (range 2 months to 18 years). Pneumonia (35, 31.1%) was the most common etiology of hemoptysis, which included bacterial pneumonia (27 cases), mycoplasmal pneumonia(4 cases), chlamydial pneumonia (3 cases), and influenza pneumonia(1 case). Other causes included bronchitis(15, 14.2%), pulmonary tuberculosis (11, 10.4%), bronchiectasis (11, 10.4%), diffuse alveolar hemorrhage (8, 7.5%), idiopathic pulmonary hemosiderosis(6, 5.7%), cardiovascular dysplasia(6, 5.7%), pulmonary contusion (4, 3.8%), foreign body in bronchus (2, 1.9%), allergic bronchopulmonary aspergillosis (2, 1.9%). Eighty-six patients manifested mild hemoptysis; moderate and massive hemoptysis were found in nine and eleven patients, respectively. Pneumonia accounted for 33.7% of mild hemoptysis and 45.5% of massive hemoptysis were due to bronchiectasis; 80.2% were treated with antibiotics and 41.5% were given hemostatic agents; 8.5% received lobectomy. Ninety-six patients (90.6%) were cured and parents gave up treatment in 4 cases (3.8%). Six patients (5.7%) suffered from recurrent hemoptysis.</p><p><b>CONCLUSION</b>Hemoptysis mainly occurred in children who were older than 6 years, the most common cause of hemoptysis was respiratory tract infection. In most cases, the amount of hemoptysis was small and the overall prognosis was good.</p>