Objective: To explore the correlations between miR-125b, miR-200c, and the severity of interstitial lung disease associated with dermatomyositis/polymyositis (DM/PM-ILD). Methods: 30 consecutive patients with DM/PM an...Objective: To explore the correlations between miR-125b, miR-200c, and the severity of interstitial lung disease associated with dermatomyositis/polymyositis (DM/PM-ILD). Methods: 30 consecutive patients with DM/PM and 23 healthy controls were recruited into current study. Anti-JO-1, anti-SSA, muscle enzymes, the data of chest HRCT and pulmonary function test were collected. 9 consecutive DM/PM-ILD patients underwent bronchoalveolar lavage (BAL). TGF-β1 and surfactant protein D (SP-D) in BAL fluid (BALF) and plasma were detected by ELISA. miR-125b and miR-200c in PBMCs and bronchoalveolar cells were detected by QRT-PCR. All patients were classified into three groups: Mild or non-ILD group, moderate ILD group, and severe ILD group. The correlations between miRNAs and the severity of ILD, the lung damage markers, auto-antibodies, were analyzed. Results: The levels of miR-125b and miR-200c in bronchoalveolar cells were higher than in PBMCs, and the levels of TGF-β1 and SP-D were higher in BALF than in plasma in DM/PM-ILD patients. There were positive correlations between miR-125b, miR-200c in bronchoalveolar cells and SP-D in BALF. The levels of miR-125b and miR-200c in severe ILD group were higher than in mild or non-ILD and moderate ILD groups. There were negative correlations between miR-125b, miR-200c, and FEV1, and between miR-200c and DLCO. The patients with anti-JO-1 antibody had higher levels of miR-125b and miR-200c, and had more severe condition of ILD. Conclusion: miR-125b and miR-200c were positively correlated with the lung damage and severity of ILD in DM/PM, which could be important markers for judgement of disease condition in clinic.展开更多
BACKGROUND Anti-melanoma differentiation-associated gene 5 antibody-positive(anti-MDA5 Ab+)dermatomyositis complicated with rapidly progressive interstitial lung disease(anti-MDA5 Ab+DM-RP-ILD)has an unclear underlyin...BACKGROUND Anti-melanoma differentiation-associated gene 5 antibody-positive(anti-MDA5 Ab+)dermatomyositis complicated with rapidly progressive interstitial lung disease(anti-MDA5 Ab+DM-RP-ILD)has an unclear underlying mechanism with no recommended unified treatment plan.Herein,one of the cases that we report(Case 2)was successfully treated with tocilizumab despite having lung infection.CASE SUMMARY Case 1 was a 30-year-old woman who was admitted due to recurrent rash for 5 mo,fever and cough for 1 mo,and chest tightness for 3 d.She was diagnosed with non-myopathic dermatomyositis(anti-MDA5 Ab+)and interstitial pneumonia,and was treated with the combination of hormone therapy and cyclophosphamide followed by oral tacrolimus.Case 2 was a 31-year-old man admitted due to systemic rash accompanied by muscle weakness of limbs for more than 1 mo,and chest tightness and dry cough for 4 d.He was diagnosed with dermatomyositis(anti-MDA5 Ab+)and acute interstitial pneumonia with Pneumocystis jirovecii and Aspergillus fumigatus infections and was treated with hormone therapy(without cyclophosphamide)and the combination of tocilizumab and tacrolimus.The condition of both patients eventually improved and they were discharged and showed clinically stable condition at the latest follow-up.CONCLUSION Tocilizumab could be a salvage treatment for patients with anti-MDA5 Ab+DMRP-ILD who are refractory to intensive immunosuppression.展开更多
BACKGROUND Clinically amyopathic deramatomyositis was manifested as the various cutaneous dermatomyositis(DM)manifestations without muscle weakness.Anti-melanoma differentiation-associated gene 5(anti-MDA5)and anti-Ro...BACKGROUND Clinically amyopathic deramatomyositis was manifested as the various cutaneous dermatomyositis(DM)manifestations without muscle weakness.Anti-melanoma differentiation-associated gene 5(anti-MDA5)and anti-Ro52 antibody-dual positive clinically amyopathic DM patients are at a high risk of developing rapidly progressive interstitial lung disease,and they exhibit an immensely high half-year mortality.CASE SUMMARY We presented three patients with anti-MDA5 and anti-Ro52 antibody-dual positive DM patients and we reviewed the previous studies on the link between anti-MDA5 and anti-Ro52 antibody-dual positive DM.Although we aggressively treated these patients similarly,but they all exhibited different prognoses.We reviewed the importance of clinical cutaneous rashes as well as the pathogenesis and treatment in the dual positive anti-MDA5 and anti-Ro52 associated DM.CONCLUSION Patients with anti-MDA5 anti-Ro52 antibody-dual positive DM should be accurately diagnosed at an early stage and should be treated aggressively,thus,the patient’s prognosis can be significantly modified.展开更多
Introduction:Anti-melanoma differentiation-associated gene 5-positive dermatomyositis(anti-MDA5+DM)is a distinct subtype of DM,which is characterized by typical cutaneous features,minimal or no muscle involvement and ...Introduction:Anti-melanoma differentiation-associated gene 5-positive dermatomyositis(anti-MDA5+DM)is a distinct subtype of DM,which is characterized by typical cutaneous features,minimal or no muscle involvement and notable interstitial lung disease,which typically progresses rapidly and has a high mortality.Spontaneous pneumomediastinum(PNM),a relatively unusual but serious complication of anti-MDA5+DM,further increases mortality.Currently,there is no generally accepted treatment regimen for anti-MDA5+DM-associated PNM.Case Description:A 53-year-old man with anti-MDA5+DM presented with rapidly progressive interstitial lung disease that progressed to diffuse subcutaneous emphysema and PNM despite aggressive immunosuppressive therapies.He responded well to combined anti-infection treatments,moderate immunotherapy,and continuous oxygen therapy.Conclusion:Comprehensive screening for potential infections,as well as close monitoring of the patient's immune status is essential for individualizing treatment and maximizing prognosis.展开更多
Background Interstitial lung disease （ILD） is a serious lung complication in polymyositis （PM） and dermatomyositis （DM） which affects prognosis and requires a more aggressive approach in therapy. This study inve...Background Interstitial lung disease （ILD） is a serious lung complication in polymyositis （PM） and dermatomyositis （DM） which affects prognosis and requires a more aggressive approach in therapy. This study investigated the prevalence, characteristics, predictive factors and unfavourable prognostic factors of ILD in newly diagnosed PM, DM and amyopathic DM （ADM）. Methods From January 2000 to December 2008, the medical records of 197 consecutive PM and DM patients at the Second Affiliated Hospital of Sun Yat-Sen University were reviewed excluding overlapping, juvenile, and malignancy-associated cases. The patients were assigned to an ILD （69 patients） and a non-lLD group （128 patients）. The clinical features, laboratory findings, and prognosis were compared. Results The multivariate analysis indicated that older age at onset （OR 1.033, 95%C/1.009-1.058, P=0.007）, fever （OR 4.109, 95%CI 1.926-8.767, P 〈0.001） and arthritis/arthralgia （OR 2.274, 95%C/1.101-4.695, P=0.026） were the independent predictive factors for developing ILD in PM/DM after excluding anti-Jo-1. Regarding anti-Jo-1, fever （OR 4.912, 95%CI 2.121-11.376, P 〈0.001） was associated with ILD. Poor survival in ILD patients was associated with ILD clinical subset （RR 0.122, 95%CI 0.049-0.399, P 〈0.001）, ADM/DM/PM-ILD （RR 0.140, 95%C/0.031-0.476, P=0.002）, cardiac involvement （RR 4.654, 95%CI 1.391-15.577, P=-0.013） and serum albumin level （RR 0.910, 95%CI 0.831-0.997, P=-0.042）. Conclusions Patients who presented with fever tended to have a higher frequency of PM/DM-associated ILD. A Hamman-Rich-like presentation, ADM-ILD, cardiac involvement and hypoalbuminemia were poor prognostic factors in ILD-PM/DM.展开更多
Dermatomyositis(DM)is a heterogeneous autoimmune disease associated with numerous myositis specific antibodies(MSAs)in which DM with anti-melanoma differentiation-associated gene 5-positive(MDA5+DM)is a unique subtype...Dermatomyositis(DM)is a heterogeneous autoimmune disease associated with numerous myositis specific antibodies(MSAs)in which DM with anti-melanoma differentiation-associated gene 5-positive(MDA5+DM)is a unique subtype of DM with higher risk of developing varying degrees of Interstitial lung disease(ILD).Glycosylation is a complex posttranslational modification of proteins associated with many autoimmune diseases.However,the association of total plasma N-glycome(TPNG)and DM,especially MDA5+DM,is still unknown.TPNG of 94 DM patients and 168 controls were analyzed by mass spectrometry with in-house reliable quantitative method called Bionic Glycome method.Logistic regression with age and sex adjusted was used to reveal the aberrant glycosylation of DM and the association of TPNG and MDA5+DM with or without rapidly progressive ILD(RPILD).The elastic net model was used to evaluate performance of glycans in distinguishing RPLID from non-RPILD,and survival analysis was analyzed with N-glycoslyation score by Kaplan-Meier survival analysis.It was found that the plasma protein N-glycome in DM showed higher fucosylation and bisection,lower sialylation(α2,3-notα2,6-linked)and galactosylation than controls.In MDA5+DM,more severe disease condition was associated with decreased sialylation(specificallyα2,3-sialylation with fucosylation)while accompanying elevated H6N5S3 and H5N4FSx,decreased galactosylation and increased fucosylation and the complexity of N-glycans.Moreover,glycosylation traits have better discrimination ability to distinguish RPILD from non-RPILD with AUC 0.922 than clinical features and is MDA5-independent.Survival advantage accrued to MDA5+DM with lower N-glycosylation score(p=3e-04).Our study reveals the aberrant glycosylation of DM for the first time and indicated that glycosylation is associated with disease severity caused by ILD in MDA5+DM,which might be considered as the potential biomarker for early diagnosis of RPILD and survival evaluation of MDA5+DM.展开更多
Background:Clinically amyopathic dermatomyositis(CADM)is a unique sub-type of idiopathic inflammatory myopathies with a high prevalence of interstitial lung disease(ILD).Poor prognosis of the patients was strongly ass...Background:Clinically amyopathic dermatomyositis(CADM)is a unique sub-type of idiopathic inflammatory myopathies with a high prevalence of interstitial lung disease(ILD).Poor prognosis of the patients was strongly associated with rapid progressive ILD.The aim of this study was to identify risk factors for prediction of different types of ILD in CADM.Methods:In this study,data of 108 inpatients with CADM were collected,including 87 with ILD.The baseline clinical data and laboratory parameters,including myositis-specific and associated antibodies and tumor-associated antigens were analyzed to identify risk factors for acute or subacute interstitial pneumonitis(A/SIP)and chronic interstitial pneumonitis(CIP).Results:In 87 patients with CADM-ILD,39(36.1%)were A/SIP,and 48(44.4%)were CIP.There were 22(20.4%)patients with asymptomatic ILD who were detected by routine high resolution computed tomography.Cytokeratin-19 fragment(CYFRA21-1)was significantly higher in CADM-ILD than that in CADM patients without ILD;carcinoembryonic antigen and neuron-specific enolase were significantly elevated in A/SIP than that in CIP.Patients with A/SIP had a higher positive rate of anti-melanoma differentiation-associated gene 5(MDA5),while patients with CIP had a higher positive rate of anti PL-12 and anti-Ro-52.Logistic regression analysis indicated that elevation of CYFRA21-1 was a risk factor for ILD,higher titer of anti-MDA5 indicated increased likelihood for A/SIP,and higher titer of anti-Ro-52 was also clearly associated with CIP.Conclusions:This study indicated that the prevalence of ILD was high in CADM.Asymptomatic ILD has been previously underestimated.Anti-MDA5 was a risk factor for the presence of A/SIP,and CYFRA21-1 was a risk factor for ILD.展开更多
Fei Tong Kou Fu Ye (肺通口服液 Fei Tong Oral Liquid) was used to treat 30 cases of interstitial pneumopathy after radio- and/or chemotherapy.In comparison with the control group (15 cases) treated with hormones,the th...Fei Tong Kou Fu Ye (肺通口服液 Fei Tong Oral Liquid) was used to treat 30 cases of interstitial pneumopathy after radio- and/or chemotherapy.In comparison with the control group (15 cases) treated with hormones,the therapeutic effects in improving dyspnea,cough,respiratory rate,cyanosis,findings in X-films and CT examination,partial pressure of oxygen in artery,FVC and VC were found significantly better (P<0.05).The total effective rate obtained was 83.33%.展开更多
Objectives: To correlate the precise specificity of autoantibodies in Japanese dermatomyositis (DM) patients with their clinical phenotypes. Methods: Serum samples from 94 adult DM patients (67 with classical DM and 2...Objectives: To correlate the precise specificity of autoantibodies in Japanese dermatomyositis (DM) patients with their clinical phenotypes. Methods: Serum samples from 94 adult DM patients (67 with classical DM and 27 with clinically amyopathic dermatomyositis, CADM) were screened for autoantibodies using immunoprecipitation assays. Patients with antibodies against aminoacyl transfer RNA synthetase (ARS), Mi-2 or who had other autoantibodies were assessed for clinical symptoms and laboratory findings. Results: Sera from 27 of 94 DM patients (29%) were found to have anti-ARS antibodies. Nineteen (20%) had anti-CADM-140/MDA5, 5 (5%) had anti-Mi-2, and 8 (6%) had anti-p155/TIF1-γ. Anti-MJ/NXP-2 was not found in our series of adult DM. Seventeen patients with anti-ARS had fever and 22 had arthritis and interstitial lung disease (ILD), compatible with a diagnosis of anti-ARS syndrome. Seventeen of 19 (89%) with anti-CADM-140/MDA5 had ILD, 16 (84%) of whom developed rapidly progressive ILD (RP-ILD). Four of 5 (80%) with anti-Mi-2 had heliotrope rash and/or Gottron’s sign/papules, and 2 (40%) had V-sign and/or shawl-sign rash, whereas no ILD or malignancy was detected. As seen with anti-Mi-2-positive patients, a low frequency of ILD (13%) was found in patients with anti-p155/TIF1-γ but 6 of 8 (75%) had malignancy during their course. The frequency of ILD was significantly higher in patients with anti-ARS or anti-CADM-140/MDA5 compared with anti-Mi-2 or anti-p155/TIF1-γ (81% and 89%, respectively). It should be noted that anti-CADM-140/MDA5-positive patients suffered significantly more RP-ILD compared to patients with anti-ARS (84% vs. 7%, P < 0.0001). On the other hand, anti-p155/TIF1-γ positive patients had a significantly higher rate of malignancy compared with anti-ARS-, anti-CADM-140/MDA5-and anti-Mi-2-positive patients (75% vs. 7%: P = 0.0004, 5%: P = 0.0006, 0%: P = 0.02, respectively). Conclusions: These results indicate that in addition to antibodies previously identified as specific for DM, autoantibodies newly found in these patients are useful for stratifying them into clinical subgroups.展开更多
文摘Objective: To explore the correlations between miR-125b, miR-200c, and the severity of interstitial lung disease associated with dermatomyositis/polymyositis (DM/PM-ILD). Methods: 30 consecutive patients with DM/PM and 23 healthy controls were recruited into current study. Anti-JO-1, anti-SSA, muscle enzymes, the data of chest HRCT and pulmonary function test were collected. 9 consecutive DM/PM-ILD patients underwent bronchoalveolar lavage (BAL). TGF-β1 and surfactant protein D (SP-D) in BAL fluid (BALF) and plasma were detected by ELISA. miR-125b and miR-200c in PBMCs and bronchoalveolar cells were detected by QRT-PCR. All patients were classified into three groups: Mild or non-ILD group, moderate ILD group, and severe ILD group. The correlations between miRNAs and the severity of ILD, the lung damage markers, auto-antibodies, were analyzed. Results: The levels of miR-125b and miR-200c in bronchoalveolar cells were higher than in PBMCs, and the levels of TGF-β1 and SP-D were higher in BALF than in plasma in DM/PM-ILD patients. There were positive correlations between miR-125b, miR-200c in bronchoalveolar cells and SP-D in BALF. The levels of miR-125b and miR-200c in severe ILD group were higher than in mild or non-ILD and moderate ILD groups. There were negative correlations between miR-125b, miR-200c, and FEV1, and between miR-200c and DLCO. The patients with anti-JO-1 antibody had higher levels of miR-125b and miR-200c, and had more severe condition of ILD. Conclusion: miR-125b and miR-200c were positively correlated with the lung damage and severity of ILD in DM/PM, which could be important markers for judgement of disease condition in clinic.
文摘BACKGROUND Anti-melanoma differentiation-associated gene 5 antibody-positive(anti-MDA5 Ab+)dermatomyositis complicated with rapidly progressive interstitial lung disease(anti-MDA5 Ab+DM-RP-ILD)has an unclear underlying mechanism with no recommended unified treatment plan.Herein,one of the cases that we report(Case 2)was successfully treated with tocilizumab despite having lung infection.CASE SUMMARY Case 1 was a 30-year-old woman who was admitted due to recurrent rash for 5 mo,fever and cough for 1 mo,and chest tightness for 3 d.She was diagnosed with non-myopathic dermatomyositis(anti-MDA5 Ab+)and interstitial pneumonia,and was treated with the combination of hormone therapy and cyclophosphamide followed by oral tacrolimus.Case 2 was a 31-year-old man admitted due to systemic rash accompanied by muscle weakness of limbs for more than 1 mo,and chest tightness and dry cough for 4 d.He was diagnosed with dermatomyositis(anti-MDA5 Ab+)and acute interstitial pneumonia with Pneumocystis jirovecii and Aspergillus fumigatus infections and was treated with hormone therapy(without cyclophosphamide)and the combination of tocilizumab and tacrolimus.The condition of both patients eventually improved and they were discharged and showed clinically stable condition at the latest follow-up.CONCLUSION Tocilizumab could be a salvage treatment for patients with anti-MDA5 Ab+DMRP-ILD who are refractory to intensive immunosuppression.
文摘BACKGROUND Clinically amyopathic deramatomyositis was manifested as the various cutaneous dermatomyositis(DM)manifestations without muscle weakness.Anti-melanoma differentiation-associated gene 5(anti-MDA5)and anti-Ro52 antibody-dual positive clinically amyopathic DM patients are at a high risk of developing rapidly progressive interstitial lung disease,and they exhibit an immensely high half-year mortality.CASE SUMMARY We presented three patients with anti-MDA5 and anti-Ro52 antibody-dual positive DM patients and we reviewed the previous studies on the link between anti-MDA5 and anti-Ro52 antibody-dual positive DM.Although we aggressively treated these patients similarly,but they all exhibited different prognoses.We reviewed the importance of clinical cutaneous rashes as well as the pathogenesis and treatment in the dual positive anti-MDA5 and anti-Ro52 associated DM.CONCLUSION Patients with anti-MDA5 anti-Ro52 antibody-dual positive DM should be accurately diagnosed at an early stage and should be treated aggressively,thus,the patient’s prognosis can be significantly modified.
基金Natural Science Foundation of Jiangxi Province,Grant/Award Number:20202ACBL206011Jiangxi Provincial Clinical Research Center for Rheumatic and Immunologic Diseases,Grant/Award Number:20192BCD42005Jiangxi Province Medical Leading Discipline Construction Project。
文摘Introduction:Anti-melanoma differentiation-associated gene 5-positive dermatomyositis(anti-MDA5+DM)is a distinct subtype of DM,which is characterized by typical cutaneous features,minimal or no muscle involvement and notable interstitial lung disease,which typically progresses rapidly and has a high mortality.Spontaneous pneumomediastinum(PNM),a relatively unusual but serious complication of anti-MDA5+DM,further increases mortality.Currently,there is no generally accepted treatment regimen for anti-MDA5+DM-associated PNM.Case Description:A 53-year-old man with anti-MDA5+DM presented with rapidly progressive interstitial lung disease that progressed to diffuse subcutaneous emphysema and PNM despite aggressive immunosuppressive therapies.He responded well to combined anti-infection treatments,moderate immunotherapy,and continuous oxygen therapy.Conclusion:Comprehensive screening for potential infections,as well as close monitoring of the patient's immune status is essential for individualizing treatment and maximizing prognosis.
文摘Background Interstitial lung disease （ILD） is a serious lung complication in polymyositis （PM） and dermatomyositis （DM） which affects prognosis and requires a more aggressive approach in therapy. This study investigated the prevalence, characteristics, predictive factors and unfavourable prognostic factors of ILD in newly diagnosed PM, DM and amyopathic DM （ADM）. Methods From January 2000 to December 2008, the medical records of 197 consecutive PM and DM patients at the Second Affiliated Hospital of Sun Yat-Sen University were reviewed excluding overlapping, juvenile, and malignancy-associated cases. The patients were assigned to an ILD （69 patients） and a non-lLD group （128 patients）. The clinical features, laboratory findings, and prognosis were compared. Results The multivariate analysis indicated that older age at onset （OR 1.033, 95%C/1.009-1.058, P=0.007）, fever （OR 4.109, 95%CI 1.926-8.767, P 〈0.001） and arthritis/arthralgia （OR 2.274, 95%C/1.101-4.695, P=0.026） were the independent predictive factors for developing ILD in PM/DM after excluding anti-Jo-1. Regarding anti-Jo-1, fever （OR 4.912, 95%CI 2.121-11.376, P 〈0.001） was associated with ILD. Poor survival in ILD patients was associated with ILD clinical subset （RR 0.122, 95%CI 0.049-0.399, P 〈0.001）, ADM/DM/PM-ILD （RR 0.140, 95%C/0.031-0.476, P=0.002）, cardiac involvement （RR 4.654, 95%CI 1.391-15.577, P=-0.013） and serum albumin level （RR 0.910, 95%CI 0.831-0.997, P=-0.042）. Conclusions Patients who presented with fever tended to have a higher frequency of PM/DM-associated ILD. A Hamman-Rich-like presentation, ADM-ILD, cardiac involvement and hypoalbuminemia were poor prognostic factors in ILD-PM/DM.
基金supported by grants from The National Key R&D Program of China(2022YFC3400803)the National Natural Science Foundation of China(32071276)+1 种基金Greater Bay Area Institute of Precision Medicine(IPM2021C005)National Postdoctoral Program for Innovative Talents(BX20190076).
文摘Dermatomyositis(DM)is a heterogeneous autoimmune disease associated with numerous myositis specific antibodies(MSAs)in which DM with anti-melanoma differentiation-associated gene 5-positive(MDA5+DM)is a unique subtype of DM with higher risk of developing varying degrees of Interstitial lung disease(ILD).Glycosylation is a complex posttranslational modification of proteins associated with many autoimmune diseases.However,the association of total plasma N-glycome(TPNG)and DM,especially MDA5+DM,is still unknown.TPNG of 94 DM patients and 168 controls were analyzed by mass spectrometry with in-house reliable quantitative method called Bionic Glycome method.Logistic regression with age and sex adjusted was used to reveal the aberrant glycosylation of DM and the association of TPNG and MDA5+DM with or without rapidly progressive ILD(RPILD).The elastic net model was used to evaluate performance of glycans in distinguishing RPLID from non-RPILD,and survival analysis was analyzed with N-glycoslyation score by Kaplan-Meier survival analysis.It was found that the plasma protein N-glycome in DM showed higher fucosylation and bisection,lower sialylation(α2,3-notα2,6-linked)and galactosylation than controls.In MDA5+DM,more severe disease condition was associated with decreased sialylation(specificallyα2,3-sialylation with fucosylation)while accompanying elevated H6N5S3 and H5N4FSx,decreased galactosylation and increased fucosylation and the complexity of N-glycans.Moreover,glycosylation traits have better discrimination ability to distinguish RPILD from non-RPILD with AUC 0.922 than clinical features and is MDA5-independent.Survival advantage accrued to MDA5+DM with lower N-glycosylation score(p=3e-04).Our study reveals the aberrant glycosylation of DM for the first time and indicated that glycosylation is associated with disease severity caused by ILD in MDA5+DM,which might be considered as the potential biomarker for early diagnosis of RPILD and survival evaluation of MDA5+DM.
基金This work was supported by grants from the National Natural Science Foundation of China(Nos.81801615 and 81871289).
文摘Background:Clinically amyopathic dermatomyositis(CADM)is a unique sub-type of idiopathic inflammatory myopathies with a high prevalence of interstitial lung disease(ILD).Poor prognosis of the patients was strongly associated with rapid progressive ILD.The aim of this study was to identify risk factors for prediction of different types of ILD in CADM.Methods:In this study,data of 108 inpatients with CADM were collected,including 87 with ILD.The baseline clinical data and laboratory parameters,including myositis-specific and associated antibodies and tumor-associated antigens were analyzed to identify risk factors for acute or subacute interstitial pneumonitis(A/SIP)and chronic interstitial pneumonitis(CIP).Results:In 87 patients with CADM-ILD,39(36.1%)were A/SIP,and 48(44.4%)were CIP.There were 22(20.4%)patients with asymptomatic ILD who were detected by routine high resolution computed tomography.Cytokeratin-19 fragment(CYFRA21-1)was significantly higher in CADM-ILD than that in CADM patients without ILD;carcinoembryonic antigen and neuron-specific enolase were significantly elevated in A/SIP than that in CIP.Patients with A/SIP had a higher positive rate of anti-melanoma differentiation-associated gene 5(MDA5),while patients with CIP had a higher positive rate of anti PL-12 and anti-Ro-52.Logistic regression analysis indicated that elevation of CYFRA21-1 was a risk factor for ILD,higher titer of anti-MDA5 indicated increased likelihood for A/SIP,and higher titer of anti-Ro-52 was also clearly associated with CIP.Conclusions:This study indicated that the prevalence of ILD was high in CADM.Asymptomatic ILD has been previously underestimated.Anti-MDA5 was a risk factor for the presence of A/SIP,and CYFRA21-1 was a risk factor for ILD.
文摘Fei Tong Kou Fu Ye (肺通口服液 Fei Tong Oral Liquid) was used to treat 30 cases of interstitial pneumopathy after radio- and/or chemotherapy.In comparison with the control group (15 cases) treated with hormones,the therapeutic effects in improving dyspnea,cough,respiratory rate,cyanosis,findings in X-films and CT examination,partial pressure of oxygen in artery,FVC and VC were found significantly better (P<0.05).The total effective rate obtained was 83.33%.
文摘Objectives: To correlate the precise specificity of autoantibodies in Japanese dermatomyositis (DM) patients with their clinical phenotypes. Methods: Serum samples from 94 adult DM patients (67 with classical DM and 27 with clinically amyopathic dermatomyositis, CADM) were screened for autoantibodies using immunoprecipitation assays. Patients with antibodies against aminoacyl transfer RNA synthetase (ARS), Mi-2 or who had other autoantibodies were assessed for clinical symptoms and laboratory findings. Results: Sera from 27 of 94 DM patients (29%) were found to have anti-ARS antibodies. Nineteen (20%) had anti-CADM-140/MDA5, 5 (5%) had anti-Mi-2, and 8 (6%) had anti-p155/TIF1-γ. Anti-MJ/NXP-2 was not found in our series of adult DM. Seventeen patients with anti-ARS had fever and 22 had arthritis and interstitial lung disease (ILD), compatible with a diagnosis of anti-ARS syndrome. Seventeen of 19 (89%) with anti-CADM-140/MDA5 had ILD, 16 (84%) of whom developed rapidly progressive ILD (RP-ILD). Four of 5 (80%) with anti-Mi-2 had heliotrope rash and/or Gottron’s sign/papules, and 2 (40%) had V-sign and/or shawl-sign rash, whereas no ILD or malignancy was detected. As seen with anti-Mi-2-positive patients, a low frequency of ILD (13%) was found in patients with anti-p155/TIF1-γ but 6 of 8 (75%) had malignancy during their course. The frequency of ILD was significantly higher in patients with anti-ARS or anti-CADM-140/MDA5 compared with anti-Mi-2 or anti-p155/TIF1-γ (81% and 89%, respectively). It should be noted that anti-CADM-140/MDA5-positive patients suffered significantly more RP-ILD compared to patients with anti-ARS (84% vs. 7%, P < 0.0001). On the other hand, anti-p155/TIF1-γ positive patients had a significantly higher rate of malignancy compared with anti-ARS-, anti-CADM-140/MDA5-and anti-Mi-2-positive patients (75% vs. 7%: P = 0.0004, 5%: P = 0.0006, 0%: P = 0.02, respectively). Conclusions: These results indicate that in addition to antibodies previously identified as specific for DM, autoantibodies newly found in these patients are useful for stratifying them into clinical subgroups.
文摘目的:采用CT定量分析多发性肌炎/皮肌炎相关间质性肺病(Polymyositis/dermatomyositis-associated interstitial lung disease,PM/DM-ILD)患者的肺部改变,探究其评价PM/DM-ILD患者胸部改变的应用价值。方法:收集301例PM/DM-ILD及76例非ILD患者胸部CT图像纳入数字肺分析平台,测量两组研究对象全肺及各肺叶的肺容积、平均肺密度及肺血管体积百分比。利用定量CT肺密度直方图计算高衰减区百分比(Percentage of high attenuation area,HAA%)。采用多元Logistic回归分析筛选与PM/DM-ILD诊断相关的定量参数并构建复合模型。使用受试者工作特征曲线(ROC)及曲线下面积(AUC)确定鉴别诊断PM/DM-ILD组和非ILD组的最佳定量参数及其阈值、并评价复合模型的诊断效能。结果:与非ILD组相比,ILD组全肺及各肺叶的肺容积均明显减小、平均肺密度及HAA%均明显增高,双肺上叶肺血管体积百分比明显增大、而双肺下叶肺血管体积百分比明显减小,差异均具有统计学意义(P<0.05)。多元Logistic回归分析显示右下肺平均密度(OR=1.015,95%CI:1.003~1.028,P=0.019)、右下肺血管体积百分比(OR=0.763,95%CI:0.584~0.997,P=0.047)及年龄(OR=1.025,95%CI:1.003~1.048,P=0.026)对诊断ILD具有统计学意义。ROC曲线分析显示,右下肺平均密度为鉴别诊断PM/DM-ILD组和非ILD组的最佳CT定量参数,AUC为0.84(95%CI:0.80~0.89),以-770.7 HU为阈值,敏感度为78.1%,特异度为77.8%。基于CT定量参数及年龄构建的复合诊断模型具有更佳的诊断效能,AUC为0.87(95%CI:0.83~0.91)。结论:定量CT能够客观准确评估PM/DM-ILD患者肺容积、平均肺密度及肺血管体积改变。基于CT定量参数及年龄构建的复合模型对ILD的识别显示出良好的诊断效能,为PM/DM-ILD患者的病情评估和诊断提供了便捷的新途径。
