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*For reference only for medical professionals, what is the disease of IIM? At the first academic annual meeting of the National Clinical Research Center for Skin and Immune Diseases and the 11th CSTAR/CRDC/CRCA annual meeting, Dr.
Wu Chanyuan from Peking Union Medical College Hospital reported on inflammatory diseases based on the National Rheumatism Data Center (CRDC) The progress in 2020 introduces the current status of my country's idiopathic inflammatory myopathy (IIM), analysis of IIM organ involvement based on CRDC, research on the pathogenesis of IIM and progress in treatment.
IIM is a group of systemic autoimmune connective tissue diseases that can cause chronic damage to muscles and other organs.
Based on clinical and immunopathological characteristics, IIM is mainly divided into polymyositis (PM), dermatomyositis (DM) and inclusion body muscles.
Three subtypes of inflammatory disease (IBM).
At present, a total of 193 centers and 4620 patients have entered CRDC.
However, due to the insufficient completeness of the cases, the overall completeness is only 55%.
Figure 1: Current status of IIM in CRDC What are the characteristics of my country's native IIM patients? The team of Dr.
Wu Chanyuan conducted a case analysis of 2334 IIM patients who met the criteria for direct reporting.
The patients were mainly female, the male to female ratio was 1:3, the average age of onset was 46.
8 years, and the course of disease was about half a year at the time of diagnosis.
A low body mass index (BMI) indicates that inflammatory diseases consume more nutrition for the body.
Patients with a history of smoking accounted for 11.
7%.
Figure 2: General situation of IIM IIM is dominated by dermatomyositis, accounting for more than 70%, and the rest are polymyositis and amyopathy dermatomyositis (CADM).
In terms of organ involvement, muscle (78.
4%) and skin (77.
2%) were mainly involved.
The other more common ones have respiratory system involvement.
With the collection of multicenter cases, cardiovascular and gastrointestinal involvement has also increased.
In terms of muscle involvement, the team of Dr.
Wu Chanyuan's manual muscle strength test (MMT8) found that the main muscle groups involved were the biceps and quadriceps.
Figure 3: The MMT8 score included imaging data of more than 1,000 patients for lung involvement.
The results showed that non-specific interstitial pneumonia (NSIP) was the main one, followed by ordinary interstitial pneumonia (UIP).
Because UIP and f-NSIP have poor response to treatment, imaging analysis is meaningful for later treatment guidance.
Figure 4: CRDC imaging classification of lung involvement shows that the most important myositis-specific antibodies are anti-Jo-1 and anti-MDA5 antibodies.
Special attention should be paid to the fact that the anti-Ro-52 antibody in myositis-related antibodies is related to poor treatment response and has certain clinical value.
In terms of disease activity, the disease evaluation-activity score (DAS) of skin and muscle involvement is around 2~2.
5, which is mainly mild activity.
In terms of organ involvement, muscle involvement and lung involvement are mainly involved, and some other chronic injury lesions also mainly involve these two systems.
What is the involvement of organs in IIM patients? Dr.
Wu Chanyuan’s team performed a multivariate logistic regression analysis on different organ involvements, and summarized the independent prevalence factors related to IIM.
1 The independent risk factors for myocardial involvement are increased creatine kinase (CK), increased γ-glutamyl transpeptidase (GGT), increased C-reactive protein (CRP), and positive anti-mitochondrial antibodies (AMA).
Figure 5: Independent risk factors for myocardial involvement 2 Gastrointestinal tract involvement Although it is not a common manifestation of organ involvement, it is closely related to clinical diagnosis and treatment and clinical prognosis.
Dr.
Wu Chanyuan’s team performed a multivariate logistic regression analysis on the involvement of the digestive tract and found that independent risk factors include male, skin ulceration, Raynaud’s phenomenon, swallowing muscle involvement, and myocardial involvement; independent protective factors for disease include: IgG rise high. Figure 6: Multivariate logistic regression analysis of digestive tract involvement 3 Pneumothorax/mediastinal subcutaneous emphysema Pneumothorax and mediastinal subcutaneous emphysema are bad organ involvement that rheumatologists worry about.
The analysis found that patients with elevated CRP and pulmonary interstitial disease appeared The risk of this problem is higher; while ANA positive is a protective factor, but its ratio is not very high.
In addition, the long-term prognosis analysis of pneumothorax and subcutaneous emphysema in the mediastinum showed that the clinical symptoms related to the prognosis were mainly fever and lymphocyte decline.
4 The age of onset of malignant tumors, anti-TIF1y antibody positive, and swallowing muscle involvement are risk factors for malignant tumors in IIM; the appearance of pulmonary interstitial lesions is a relatively protective factor.
Malignant tumors in female patients are mainly breast and ovarian cancer, while in male patients, lung and gastric cancer are the most common.
In addition, the respiratory department of the hospital conducted a single-center tumor-related dermatomyositis prognostic analysis, and found that patients with tumors have the worst prognosis.
Figure 7: Risk factors for malignant tumors in IMM patients 5 Risk factors for ectopic calcification in ectopic calcification include: vasculitis, skin ulceration, Raynaud’s phenomenon, respiratory muscle weakness; suggesting that in the case of repeated ectopic calcification, it is necessary Strengthen the treatment of hormone immunity inhibitors.
The relative protective factors are: the age of onset is older, which is related to the immune response of the juvenile immune strength.
In-depth research: What is the significance of nucleosomes in the pathogenesis of IIM? First, through bioinformatics analysis of IIM-related genes, and network analysis of these genes, it was found that the mitochondrial function of IIM patients was simplified and the interferon pathway obstacles.
In addition to the genetic analysis, the mitochondrial matrix has also been studied.
The nucleosomes are usually inside the cell, but in the case of cell necrosis, the nucleosomes may be released outside the cell and into the blood.
Studies have found that in patients with IIM, the level of nucleosomes in the plasma is increased, suggesting that it has cell necrosis and inflammation activation effects.
New method-functional nuclear magnetic In addition, Dr.
Wu Chanyuan's team cooperated with the radiology department and made some explorations in the functional nuclear magnetic field of IIM. Functional NMR has more displays on the acute and chronic lesions of myositis, as well as muscle inflammation, so that we have more methods to evaluate the patient's condition.
Figure 8: In addition to hormones and immunosuppressive agents, how should IIM be treated for imaging examinations of IIM patients? IIM still uses conventional hormones and immunosuppressive agents as the first-line treatment.
For severe IIM patients, IVIG and even plasma exchange are also tried.
But because of the inhibitory nature of myositis, it is difficult for us to obtain effective randomized controlled trial (RCT) studies or real-world research data, so there are only a few cases reported.
Figure 9: Summary of IIM baseline treatment plan.
Dr.
Wu Chanyuan’s team used CRDC database to introduce the current status of IIM in my country, analysis of organ involvement, pathogenesis and treatment from a large-scale cohort, showing us the progress of myositis in recent years .
Future research focuses are: "real-world evidence" replaces traditional clinical trials for research on expanded indications; multi-center core data accumulation, a good platform, accumulation of data and biological samples; basic pathogenesis research, clinical analysis and research on the basis of the platform Validation, prognostic prediction model, and therapeutic exploration and evaluation.
Wu Chanyuan from Peking Union Medical College Hospital reported on inflammatory diseases based on the National Rheumatism Data Center (CRDC) The progress in 2020 introduces the current status of my country's idiopathic inflammatory myopathy (IIM), analysis of IIM organ involvement based on CRDC, research on the pathogenesis of IIM and progress in treatment.
IIM is a group of systemic autoimmune connective tissue diseases that can cause chronic damage to muscles and other organs.
Based on clinical and immunopathological characteristics, IIM is mainly divided into polymyositis (PM), dermatomyositis (DM) and inclusion body muscles.
Three subtypes of inflammatory disease (IBM).
At present, a total of 193 centers and 4620 patients have entered CRDC.
However, due to the insufficient completeness of the cases, the overall completeness is only 55%.
Figure 1: Current status of IIM in CRDC What are the characteristics of my country's native IIM patients? The team of Dr.
Wu Chanyuan conducted a case analysis of 2334 IIM patients who met the criteria for direct reporting.
The patients were mainly female, the male to female ratio was 1:3, the average age of onset was 46.
8 years, and the course of disease was about half a year at the time of diagnosis.
A low body mass index (BMI) indicates that inflammatory diseases consume more nutrition for the body.
Patients with a history of smoking accounted for 11.
7%.
Figure 2: General situation of IIM IIM is dominated by dermatomyositis, accounting for more than 70%, and the rest are polymyositis and amyopathy dermatomyositis (CADM).
In terms of organ involvement, muscle (78.
4%) and skin (77.
2%) were mainly involved.
The other more common ones have respiratory system involvement.
With the collection of multicenter cases, cardiovascular and gastrointestinal involvement has also increased.
In terms of muscle involvement, the team of Dr.
Wu Chanyuan's manual muscle strength test (MMT8) found that the main muscle groups involved were the biceps and quadriceps.
Figure 3: The MMT8 score included imaging data of more than 1,000 patients for lung involvement.
The results showed that non-specific interstitial pneumonia (NSIP) was the main one, followed by ordinary interstitial pneumonia (UIP).
Because UIP and f-NSIP have poor response to treatment, imaging analysis is meaningful for later treatment guidance.
Figure 4: CRDC imaging classification of lung involvement shows that the most important myositis-specific antibodies are anti-Jo-1 and anti-MDA5 antibodies.
Special attention should be paid to the fact that the anti-Ro-52 antibody in myositis-related antibodies is related to poor treatment response and has certain clinical value.
In terms of disease activity, the disease evaluation-activity score (DAS) of skin and muscle involvement is around 2~2.
5, which is mainly mild activity.
In terms of organ involvement, muscle involvement and lung involvement are mainly involved, and some other chronic injury lesions also mainly involve these two systems.
What is the involvement of organs in IIM patients? Dr.
Wu Chanyuan’s team performed a multivariate logistic regression analysis on different organ involvements, and summarized the independent prevalence factors related to IIM.
1 The independent risk factors for myocardial involvement are increased creatine kinase (CK), increased γ-glutamyl transpeptidase (GGT), increased C-reactive protein (CRP), and positive anti-mitochondrial antibodies (AMA).
Figure 5: Independent risk factors for myocardial involvement 2 Gastrointestinal tract involvement Although it is not a common manifestation of organ involvement, it is closely related to clinical diagnosis and treatment and clinical prognosis.
Dr.
Wu Chanyuan’s team performed a multivariate logistic regression analysis on the involvement of the digestive tract and found that independent risk factors include male, skin ulceration, Raynaud’s phenomenon, swallowing muscle involvement, and myocardial involvement; independent protective factors for disease include: IgG rise high. Figure 6: Multivariate logistic regression analysis of digestive tract involvement 3 Pneumothorax/mediastinal subcutaneous emphysema Pneumothorax and mediastinal subcutaneous emphysema are bad organ involvement that rheumatologists worry about.
The analysis found that patients with elevated CRP and pulmonary interstitial disease appeared The risk of this problem is higher; while ANA positive is a protective factor, but its ratio is not very high.
In addition, the long-term prognosis analysis of pneumothorax and subcutaneous emphysema in the mediastinum showed that the clinical symptoms related to the prognosis were mainly fever and lymphocyte decline.
4 The age of onset of malignant tumors, anti-TIF1y antibody positive, and swallowing muscle involvement are risk factors for malignant tumors in IIM; the appearance of pulmonary interstitial lesions is a relatively protective factor.
Malignant tumors in female patients are mainly breast and ovarian cancer, while in male patients, lung and gastric cancer are the most common.
In addition, the respiratory department of the hospital conducted a single-center tumor-related dermatomyositis prognostic analysis, and found that patients with tumors have the worst prognosis.
Figure 7: Risk factors for malignant tumors in IMM patients 5 Risk factors for ectopic calcification in ectopic calcification include: vasculitis, skin ulceration, Raynaud’s phenomenon, respiratory muscle weakness; suggesting that in the case of repeated ectopic calcification, it is necessary Strengthen the treatment of hormone immunity inhibitors.
The relative protective factors are: the age of onset is older, which is related to the immune response of the juvenile immune strength.
In-depth research: What is the significance of nucleosomes in the pathogenesis of IIM? First, through bioinformatics analysis of IIM-related genes, and network analysis of these genes, it was found that the mitochondrial function of IIM patients was simplified and the interferon pathway obstacles.
In addition to the genetic analysis, the mitochondrial matrix has also been studied.
The nucleosomes are usually inside the cell, but in the case of cell necrosis, the nucleosomes may be released outside the cell and into the blood.
Studies have found that in patients with IIM, the level of nucleosomes in the plasma is increased, suggesting that it has cell necrosis and inflammation activation effects.
New method-functional nuclear magnetic In addition, Dr.
Wu Chanyuan's team cooperated with the radiology department and made some explorations in the functional nuclear magnetic field of IIM. Functional NMR has more displays on the acute and chronic lesions of myositis, as well as muscle inflammation, so that we have more methods to evaluate the patient's condition.
Figure 8: In addition to hormones and immunosuppressive agents, how should IIM be treated for imaging examinations of IIM patients? IIM still uses conventional hormones and immunosuppressive agents as the first-line treatment.
For severe IIM patients, IVIG and even plasma exchange are also tried.
But because of the inhibitory nature of myositis, it is difficult for us to obtain effective randomized controlled trial (RCT) studies or real-world research data, so there are only a few cases reported.
Figure 9: Summary of IIM baseline treatment plan.
Dr.
Wu Chanyuan’s team used CRDC database to introduce the current status of IIM in my country, analysis of organ involvement, pathogenesis and treatment from a large-scale cohort, showing us the progress of myositis in recent years .
Future research focuses are: "real-world evidence" replaces traditional clinical trials for research on expanded indications; multi-center core data accumulation, a good platform, accumulation of data and biological samples; basic pathogenesis research, clinical analysis and research on the basis of the platform Validation, prognostic prediction model, and therapeutic exploration and evaluation.
