Cardiovascular risk factors among dermatomyositis patients with positive anti-Jo-1 antibody / 中国免疫学杂志

Objective:To assess the association between anti-Jo-1 antibody and cardiovascular risk factors among patients with dermatomyositis (DM).Methods:87 patients with DM were included from 2006 to 2013.Serum anti-Jo-1 antibody was measured by ELISA,and cardiovascular risk factors were evaluated.Multiple linear regression was used to assess the association between anti-Jo-1 antibody and cardiovascular risk factors in these patients.Results:The prevalence of hypertension,diabetes,hyperuricemia,and dyslipidemia were 16.09%,22.99%,10.34% and 67.82%,respectively.16.09% of DM patients had a positive anti-Jo-1 antibody and these patients had higher frequency of arthralgia/Raynaud's phenomenon/interstitial lung disease,and higher level of leukocyte and C reactive protein,while had lower level of serum uric acid (P<0.05).Multiple linear regression demonstrated that anti-Jo-1 antibody was closely associated with the level of C reactive protein and serum uric acid.Conclusion:Positive anti-Jo-1 antibody is associated with inflammation marker;however,the detailed mechanism remains further research.

Clinical features and imaging findings of interstitial lung disease in antisynthetase syndrome / 中华放射学杂志

Objective To investigate the clinical features and imaging findings of interstitial lung disease in antisynthetase syndrome(AS-ILD)and to compare the characteristics among AS specificities. Methods A total of 59 cases with AS-ILD at our hospital during the last 5 years were retrospectively reviewed, including anti-Jo1 positive in 37 cases and anti-PL7 positive in 6 cases and anti-PL12 positive in 6 cases and anti EJ positive in 10 cases. There were 14 males and 45 females aged (51 ± 12) years. The clinical features including myositis, arthritis, fever,"mechanic's hands", rash, proximal dysphagia, raynaud phenomenon were identified. Two radiologists evaluated the pattern, distribution and the ILD pattern of the lung abnormalities on HRCT findings. Based on the anti synthetase antibody positive subtype, 59 patients could be divided into four groups;then the differences of clinical features and HRCT findings between different subtypes were analyzed. X2 test was performed for the comparison of the differences between anti-Jo1 antibody positive and the other 3 group. Results (1)Myositis and arthritis were the most common AS manifestations, which were 61.02%(36/59)and 50.85%(30/59) respectively.(2)The lung abnormalities were predominantly basal(91.53%, 54/59) and peripheral(59.32%, 35/59). Ground-grass opacities(79.66%, 47/59)and reticulations(76.27%, 45/59) were found most frequently. Non-specific interstitial pneumonia (NSIP) was the most common HRCT pattern(64.41%, 38/59).(3)As compared with anti-Jo1 positive AS patients, anti-PL12 positive AS patients showed a high rate of traction bronchiectasis at diagnosis(83.33%, 5/6), while the difference was statistically significant(χ2=7.206, P=0.015). The prevalence of pericardial effusion(40.00%, 4/10) was significantly higher in the group of the anti-EJ positive AS patients than that with anti-Jo1 antibody AS(χ2=6.317, P=0.044). Conclusions Myositis and arthritis are the predominant clinical features. NSIP is the most common HRCT pattern in AS-ILD patients. There are some differences of signs among various subtypes, indicating that the difference of fibrosis in the lung and inflammatory reaction in the body being correlated with the AS specificities.

Clinical features and imaging findings of interstitial lung disease in antisynthetase syndrome / 中华放射学杂志

Objective To investigate the clinical features and imaging findings of interstitial lung disease in antisynthetase syndrome(AS-ILD)and to compare the characteristics among AS specificities. Methods A total of 59 cases with AS-ILD at our hospital during the last 5 years were retrospectively reviewed, including anti-Jo1 positive in 37 cases and anti-PL7 positive in 6 cases and anti-PL12 positive in 6 cases and anti EJ positive in 10 cases. There were 14 males and 45 females aged (51 ± 12) years. The clinical features including myositis, arthritis, fever,"mechanic's hands", rash, proximal dysphagia, raynaud phenomenon were identified. Two radiologists evaluated the pattern, distribution and the ILD pattern of the lung abnormalities on HRCT findings. Based on the anti synthetase antibody positive subtype, 59 patients could be divided into four groups;then the differences of clinical features and HRCT findings between different subtypes were analyzed. X2 test was performed for the comparison of the differences between anti-Jo1 antibody positive and the other 3 group. Results (1)Myositis and arthritis were the most common AS manifestations, which were 61.02%(36/59)and 50.85%(30/59) respectively.(2)The lung abnormalities were predominantly basal(91.53%, 54/59) and peripheral(59.32%, 35/59). Ground-grass opacities(79.66%, 47/59)and reticulations(76.27%, 45/59) were found most frequently. Non-specific interstitial pneumonia (NSIP) was the most common HRCT pattern(64.41%, 38/59).(3)As compared with anti-Jo1 positive AS patients, anti-PL12 positive AS patients showed a high rate of traction bronchiectasis at diagnosis(83.33%, 5/6), while the difference was statistically significant(χ2=7.206, P=0.015). The prevalence of pericardial effusion(40.00%, 4/10) was significantly higher in the group of the anti-EJ positive AS patients than that with anti-Jo1 antibody AS(χ2=6.317, P=0.044). Conclusions Myositis and arthritis are the predominant clinical features. NSIP is the most common HRCT pattern in AS-ILD patients. There are some differences of signs among various subtypes, indicating that the difference of fibrosis in the lung and inflammatory reaction in the body being correlated with the AS specificities.

The levels and clinical significance of serum B cell activating factor in Chinese patients with polymyositis or dermatomyositis / 中华内科杂志

Objectives To investigate serum levels of B cell activating factor(BAFF)in Chinese patients with polymyositis(PM)or dermatomyositis(DM),and analyze the correlation of BAFF with autoantibodies and clinical phenotypes.Methods Serum BAFF levels of 28 PM patients and 30 DM patients(study group),and 25 matched healthy controls(control group)were measured by ELISA.Serum anti-Jo-1 antibody levels were also measured by ELISA in all the subjects.The results of the two groups were compared by unpaired t test and the relevance was analyzed by Pearson's correlation analysis.Results Serum levels of BAFF in PM/DM patients were significantly higher compared to healthy controls(P =0.000),but there was no statistically significant difference between the PM and DM patients(P ＞ 0.05).Patients with interstitial lung disease(ILD)had significantly higher serum BAFF level than the patients without ILD(P =0.000)or the controls(P =0.000).Serum BAFF levels of patients with positive antinuclear antibody(ANA)were significantly higher than those with negative ANA(P =0.003).For patients with anti-Jo-1 antibodies,the serum BAFF levels were correlated with the serum concentration of anti-Jo-1 antibodies(r =0.799,P =0.006).Conclusions Serum levels of BAFF are increased in Chinese PM/DM patients.These findings indicate that BAFF may be possibly enrolled in the pathogenesis of PM/DM.Detecting serum BAFF levels could have some implication for the diagnosis and treatment of PM/DM.

Serum anti-Jo-1 antibody level correlates with disease activity in polymyositis and dermatomyositis / 中华风湿病学杂志

ObjectiveTo evaluate the correlation between serum anti-Jo-1 antibody level and myositis disease activity in polymyositis/dermatomyositis.MethodsAnti-Jo-1 antibody levels in serum from 148 polymyositis/dermatomyositis patients and 130 healthy controls were measured by both ELISA and immunoblot assay.Disease activity of the anti-Jo-1 antibody positive patients was assessed by the global myositis disease activity score (visual analogue score,VAS) established by the International Myositis Assessment and Clinical Studies(IMACS) Group.The correlation between disease activity and level of serum antiJo-1 antibody was assessed.Comparisons between groups were performed by x2 test or t test.ResultsThe positive rate of ELISA and EUROLINE was 24.3％(36/148) and 27.0％(40/148) respectively.Fever,ILD,arthritis/arthralgia were found to be more predominant in anti-Jo-1 antibody positive patients than those who were anti-Jo-1 antibody negative patients.There was significant positive correlation between the global myositis disease activity score (VAS) and serum level of anti-Jo-1 antibody (r=0.874,P=9.000).Serum antiJo-1 antibody levels,together with global disease activity,were significantly decreased in the 7 patients after treatment.ConclusionAnti-Jo-1 antibody level measured by ELISA is associated with disease activity of PM/DM,and could be a marker of disease activity.

A Case of Dermatomyositis with Secondary Organizing Pneumonia / 영남의대학술지

Dermatomyositis is characterized by progressive, symmetric, proximal muscle weakness and a nonsuppurative inflammatory myopathy of unknown etiology involving predominantly skeletal muscles. It is also characterized by typical skin lesions. Interstitial lung disease has a poor prognosis when it is associated with dermatomyositis. Organizing pneumonia is a disease in which granulation tissue fills the lumina of terminal and respiratory bronchioles and extends into the distal airspaces. The cryptogenic nature of the process is appreciated in that organizing pneumonia patterns of injury can be seen in secondary forms of the disease (secondary organizing pneumonia). Organizing pneumonia has been reported to occur in 5~10% in dermatomyositis-polymyositis patients. Anti-histidyl tRNA synthetase antibody (anti-Jo-1) is a predictive disease marker that is reported to occur in up to 70% of patients. We describe a 49-year-old male dermatomyositis patient who presented with organizing pneumonia and was found to have negative anti-Jo-1 antibody.

Overlap Syndrome of Amyopathic Antisynthetase Syndrome and Systemic Lupus Erythematosus: A Case Report / 대한류마티스학회지

Interstitial lung disease (ILD) with autoantibodies against antisynthetase may occur in the absence of clinically apparent myositis. We report 58-year-old woman who initially presented with dyspnea. The patient showed positive immunologic markers reflecting systemic lupus erythematosus, destructive polyarthritis, Raynaud's phenomenon, and ILD. There is no proximal muscle weakness in her symptoms. This syndrome was confirmed by positive antibodies to Jo-1. For the early diagnosis of this syndrome, the possibility of this syndrome including other connective tissue disease should be considered in patients presenting with symptoms related to ILD and positive anti-nuclear antibody.

Extraction and purification of Jo-1 antigen / 基础医学与临床

Objective To improve the purifying method of Jo-1 antigen from rabbit thymus used for detection of anti-Jo-1 antibody by dot-blotting immunoassay(DB).Methods The rabbit thymus glands were cut into pieces,homogenized and extracted by PBS.Total protein was precipitated by acetone to get acetone powder(RTAP).The RTAP was solved in PBS and separated by an by anti-Jo-1 IgG affinity column.Results 5～7 g RTAP was obtained from 100g rabbit thymus glands.There was 19%～24% of protein in RTAP.Jo-1 antigen was enriched around 1900 folds through affinity chromatography,with 2.5% recovery of antigenic activity.In this preparation,there were several bands on SDS-PAGE,but only one band about 50 ku,reacted with anti-Jo-1 antisera on immunoblotting.Dot-blotting also showed that the antigen only reacted with Jo-1 antisera.The purified Jo-1 antigen was not stable for long time,but the antigenic activity could maintain for a long time when there was MgCl2 in the solution.Conclusion Affinity chromatography was a simple and easy method for purifying Jo-1 antigen from rabbit thymus.The antigen purified by affinity chromatography could meet the requirement for detecting Jo-1 antibody bydot-blotting.

Successful Combination Therapy of Cyclosporine and Methotrexate for Refractory Polymyositis with Anti-Jo-1 Antibody: A Case Report

Although corticosteroids have been the initial agent for the treatment of inflammatory myopathies (IM), immunosuppressive agents such as azathioprine, methotrexate, cyclophosphamide, or cyclosporine are commonly required to control the disease except mild cases. On the other hand, the efficacy of combination therapy of cyclosporine and methotrexate in severe rheumatoid arthritis has been proven without serious side effects. However, in treatment-resistant myositis, the experience of such a therapy is very limited, and has not been described in refractory polymyositis with anti-Jo-1 antibody. Here, we report a young female patient with recalcitrant polymyositis and anti-Jo-1 antibody who was successfully treated with the combination therapy of cyclosporine and methotrexate. At first, the myositis did not respond to several agents, such as corticosteroid, monthly pulse cyclophosphamide, azathioprine, or cyclosporine. Methotrexate was initially avoided as treatment regimen because of its potential pulmonary toxicity in the case with preexisting lung disease.

Clinical Features of Dermatomyositis/Polymyositis(DM/PM) with lung Involvement / 결핵

BACKGROUND: Although lung involvement has been reported in 5 to 46% of dermatomyositis/polymyositis (DM/PM) patients, reports of the condition in Korea are rare. This study evaluated the clinical features of lung involvement in DM/PM patients. METHODS: The medical records, laboratory results and radiologic findings of 79 DM/PM patients, who attended the Seoul National University Hospital (SNUH) between 1989 and 1999, were reviewed retrospectively. RESULTS: A total 79 patients of whom 24 patients(33%) showed lung involvement, were enrolled in this study. More patients with lung involvement were female(F:M=11:1), and older compared with those without lung involvement. Patients with lung involvement presented with dyspnea(79%), coughing(67%), an elevated ESR, and CK/LD. Anti-Jo 1 antibody test was positive in 30%, which is significantly higher in patients with lung involvement. A simple chest X-ray of the patients with lung involvement exhibited reticular opacity(50%), reticulonodular opacity(30%), patchy opacity(29%), nodular opacity(13%) and linear opacity(4%). HRCT(n=24) showed ground glass opacity(75%), linear or septal thickening(50%), patchy consolidation(42%), honey-combing(33%) and nodular opacity(17%). The pulmonary function test showed a restrictive ventilatory pattern(77%) and a lower diffusing capacity(62%). The patients were followed up during a mean duration of 30±28 months. They were treated with steroid only(50%) or a combination of steroids and cytotoxic agents(46%). Muscle symptoms were improved in 89% with treatment, but an improvement in the respiratory symptoms or in the pulmonary function test was rare. Patients with lung involvement had a higher mortality rate(21%) than those without lung involvement(10%) during the follow-up periods. CONCLUSION: DM/PM patients with lung involvement were mostly female, older and had a higher positive rate Anti-Jo 1 antibodies, but there was no significant difference in prognosis.

The Clinical Study On Inflammatory Myopathies / 대한류마티스학회지

OBJECTIVE: To determine possible similarities and differences in clinical and laboratory features and prognosis between patients with inflammatory myopathies diagnosed at Kangnam St. Mary's hospital and other reported series. METHODS: Medical records of 52 patients diagnosed as inflammatory myopathies by Bohan and Peter criteria who were admitted to Kangnam St. Mary's hospital between January 1989 and August 1994, were reviewed. The patients were further classified into 5 groups proposed by Bohan and Peter ; group I primary polymyositis(PM), group II primary dermatomyositis(DM), group III polymyositis/dermatomyositis(PM/DM) with neoplasia, group IV juvenile PM/DM, group V PM/DM associated with connective tissue disease. RESULTS: 1) There were 52 PM/DM cases with mean age at diagnosis of 34. 4 ranging from 6 to 67 years, and significantly more females(male:female = 9:43). 2) Of 52 patients, group II was the most frequent group(36.5%), and the next were as follows ; group I 28.8%, group V 25%, group IV 5.7%, group 3.8%. Systemic lupus erythematosus(SLE) was the commonest connective tissue disease, and malignancy was associated in 2 cases including breast cancer and small cell carcinoma of lung. 3) At presentation, 86.5% had proximal muscle weakness, 61.5% had arthralgia/arthritis and 44. 2% had Raynaud phenomenon. 4) The elevated serum LDH, aldolase and creatine kinase were found in 84. 6%, 84. 1%o, 71.2% of the patients respectively. Positive electromyography and muscle biopsy consistent with inflammatory myopathy were also found in 91.4%, 88.8% respectively. 5) Positive antinuclear antibody, rheumatoid factor, anti-Ro, anti-nRNP were found in 59.6%, 17.3%, 16.7%, 8.3% of patients respectively, mainly in group V. Anti-Jo-1 antibody was present in 25.8% of patients tested(8/31), including group II 4 cases, group V 3 cases and group I I case. 6) All 8 patients with anti-Jo-1 antibody had interstitial lung disease(ILD) and Raynaud phenomenon with statistical significance, compared with those without anti-Jo-1 antibody(P<0.01). 7) Among the 43 patients in whom follow up was available, 10 patients died(mortality rate 23.2%). The main cause of death were infection(4 cases), and respiratory muscle weakness, respiratory failure due to ILD, malignancy 2 cases respectively. CONCLUSION: The results of the study demonstrated that clinical expression of PM/DM were similar to previously reported series except relatively high frequency of primary dermatomyositis in this study. Anti-Jo-1 antibody was main autoantibody in inflammatory myopathies, which was associated with extramuscular manifestations, such as ILD and Raynaud's phnomenon.