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
Dermatomyositis(DM)is an idiopathic inflammatory myopathy characterized by prominent skin lesions,muscle weakness,and clinically heterogeneous systemic manifestations.Among patients with DM,the male-to-female ratio is...Dermatomyositis(DM)is an idiopathic inflammatory myopathy characterized by prominent skin lesions,muscle weakness,and clinically heterogeneous systemic manifestations.Among patients with DM,the male-to-female ratio is 2:1.Juvenile dermatomyositis(JDM)is most prevalent between the ages of 4 and 14 years,while adult-onset dermatomyositis typically occurs between the ages of 40 and 60 years.In a population-based study conducted in Olmsted County,Minnesota,USA,the risk of DM was found to increase with age across different age groups stratified by decade.The incidence rate of DM in individuals aged≥80 years was 3.2 per 100,000 person-years.Dermatomyositis in elderly patients is characterized by unique clinical manifestations,pathogenic mechanisms,and therapeutic approaches.However,discussions regarding geriatric dermatomyositis are currently limited.Therefore,this article aims to review the epidemiology,clinical features,histopathology,and pathogenesis of dermatomyositis,with a particular focus on the unique clinical characteristics of geriatric dermatomyositis.展开更多
In this manuscript, we present a case report of a child with 16-year-old previously healthy Hispanic male who presented for progressive proximal muscle weakness, rash, and dysphagia. He was admitted to the acute care ...In this manuscript, we present a case report of a child with 16-year-old previously healthy Hispanic male who presented for progressive proximal muscle weakness, rash, and dysphagia. He was admitted to the acute care floor and diagnosed with juvenile dermatomyositis and found to be positive for anti-Mi-2 alpha, anti-Mi-2 beta, and anti-MDA-5 antibodies. He gradually improved with a combination of steroid, immunomodulatory treatment, and physical therapy. This case outlines the clinical course of a patient with this rare disorder as well as the importance of understanding the role of associated antibodies to manage potential long-term sequelae.展开更多
Objective:The skin manifestations of dermatomyositis(DM)are complex and diverse,with some rashes having diagnostic significance,while others have a certain suggestive effect on the patient’s condition,visceral damage...Objective:The skin manifestations of dermatomyositis(DM)are complex and diverse,with some rashes having diagnostic significance,while others have a certain suggestive effect on the patient’s condition,visceral damage,and prognosis.This case-cohort study was performed to investigate the skin manifestations of 205 adult inpatients with DM.We then analyzed the association between the clinical characteristics and panniculitis in adult patients with DM.Methods:This study was conducted at the First Medical Center of PLA General Hospital,Beijing,China from March 1,2016 to December 30,2020 with follow-up until December 31,2021.This case-cohort study involved 205 eligible participants with DM who met the criteria for idiopathic inflammatory myopathy.Clinical data,cutaneous manifestations,and lesion locations were screened and analyzed.Serum samples were collected,and immunoblotting was used to detect 16 types of myositis-specific antibodies and myositis-associated antibodies.Results:The results showed that 16(7.8%)of the 205 adult patients with DM had panniculitis.The panniculitis was mainly distributed in the subcutaneous fat of the abdomen,buttocks,and thighs.This condition was often accompanied by skin ulcers,atrophic scars,and calcinosis.Moreover,9(72.7%)of the 16 patients with panniculitis were positive for antibodies to nuclear matrix protein 2(anti-NXP2 antibodies).Conclusion:The data showed that panniculitis is an uncommon skin manifestation in adult patients with DM,with a positivity rate of 7.8%.The appearance of panniculitis is likely associated with anti-NXP2 antibodies.展开更多
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Dermatomyositis(DM)is an idiopathic inflammatory myopathy characterized by prominent skin lesions,muscle weakness,and clinically heterogeneous systemic manifestations.Among patients with DM,the male-to-female ratio is 2:1.Juvenile dermatomyositis(JDM)is most prevalent between the ages of 4 and 14 years,while adult-onset dermatomyositis typically occurs between the ages of 40 and 60 years.In a population-based study conducted in Olmsted County,Minnesota,USA,the risk of DM was found to increase with age across different age groups stratified by decade.The incidence rate of DM in individuals aged≥80 years was 3.2 per 100,000 person-years.Dermatomyositis in elderly patients is characterized by unique clinical manifestations,pathogenic mechanisms,and therapeutic approaches.However,discussions regarding geriatric dermatomyositis are currently limited.Therefore,this article aims to review the epidemiology,clinical features,histopathology,and pathogenesis of dermatomyositis,with a particular focus on the unique clinical characteristics of geriatric dermatomyositis.
文摘In this manuscript, we present a case report of a child with 16-year-old previously healthy Hispanic male who presented for progressive proximal muscle weakness, rash, and dysphagia. He was admitted to the acute care floor and diagnosed with juvenile dermatomyositis and found to be positive for anti-Mi-2 alpha, anti-Mi-2 beta, and anti-MDA-5 antibodies. He gradually improved with a combination of steroid, immunomodulatory treatment, and physical therapy. This case outlines the clinical course of a patient with this rare disorder as well as the importance of understanding the role of associated antibodies to manage potential long-term sequelae.
文摘Objective:The skin manifestations of dermatomyositis(DM)are complex and diverse,with some rashes having diagnostic significance,while others have a certain suggestive effect on the patient’s condition,visceral damage,and prognosis.This case-cohort study was performed to investigate the skin manifestations of 205 adult inpatients with DM.We then analyzed the association between the clinical characteristics and panniculitis in adult patients with DM.Methods:This study was conducted at the First Medical Center of PLA General Hospital,Beijing,China from March 1,2016 to December 30,2020 with follow-up until December 31,2021.This case-cohort study involved 205 eligible participants with DM who met the criteria for idiopathic inflammatory myopathy.Clinical data,cutaneous manifestations,and lesion locations were screened and analyzed.Serum samples were collected,and immunoblotting was used to detect 16 types of myositis-specific antibodies and myositis-associated antibodies.Results:The results showed that 16(7.8%)of the 205 adult patients with DM had panniculitis.The panniculitis was mainly distributed in the subcutaneous fat of the abdomen,buttocks,and thighs.This condition was often accompanied by skin ulcers,atrophic scars,and calcinosis.Moreover,9(72.7%)of the 16 patients with panniculitis were positive for antibodies to nuclear matrix protein 2(anti-NXP2 antibodies).Conclusion:The data showed that panniculitis is an uncommon skin manifestation in adult patients with DM,with a positivity rate of 7.8%.The appearance of panniculitis is likely associated with anti-NXP2 antibodies.
