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*Only for medical professionals to read for reference.
What research progress will be made in 2020 on the complicated and mysterious IgG4-related diseases? Listen to Professor Xiehe’s words.
.
.
March 26-28, 2021, organized by the National Center for Clinical Research on Skin and Immune Diseases (NCRC-DID), the Chinese Association of Rheumatology and Immunology (CRCA), and the National Rheumatology Association Data Center (CRDC), China Systemic Lupus Erythematosus Research Collaboration Group (CSTAR), Chinese Rehabilitation Medicine Association, Beijing Medical Award Foundation, Zhongguancun Creative Research Institute of Medical Engineering Research Center for Immunological Diseases co-sponsored the "First National Skin and Immunity The Academic Annual Meeting of the Center for Disease Research and Clinical Medicine and the 11th CSTAR/CRDC/CRCA Annual Meeting" was successfully held in Beijing.
In the last session of the conference, the "IgG4-related disease research (PUMCH)" brought by Professor Zhang Wen from Peking Union Medical College Hospital.
Picture: Professor Zhang Wen began to share the content of the theme In view of the previous conference reports, Professor Zhang Wen has shared the research progress of IgG4-related diseases in the world.
Therefore, at this meeting, Professor Zhang Wen, based on the Union Hospital where he is located, introduced the progress made by Union Hospital in the research of IgG4-related diseases in 2020.
Basic research—finding new biomarkers Professor Zhang Wen’s team used single-cell sequencing to find several differentially expressed cell populations and major differential molecules (research has not yet been published); the gene expression of the TCR receptor library was analyzed and it was found that immune clones were in Abnormal expression of IgG4 in patients; using a lectin chip to find serum IgG4 glycosylation biomarkers; using UPLC-MS plasma metabolomics analysis, it is found that the metabolic performance of IgG4 patients is different from that of normal people.
Clinical diagnosis—discuss the pathological characteristics of IgG4-related diseases in the new method of influencing science: immune disorders, inflammation and fibrosis.
At present, CT and magnetic resonance (MRI) are still commonly used clinical imaging methods for IgG4-related diseases, but PET/CT also has special effects in the diagnosis of IgG4-related diseases.
Professor Zhang Wen's team used 68Ga-FAPI tracer (targeted fibroblast activation protein) to show the pathological characteristics of fibrosis of IgG4-related diseases, thereby assisting in the diagnosis of IgG4-related diseases.
Figure: Different tracers show different pathological types of lesions.
Clinical diagnosis and treatment-a comprehensive understanding of IgG4 related diseases Professor Zhang Wen's team analyzed the clinical characteristics of 737 patients with IgG4 related diseases.
It was found that the incidence of IgG4-related diseases between 50-70 years old is the highest among those aged 50-70 years; with age, the proportion of male IgG4-related diseases increases; the older the age, the lower the probability of IgG4-related diseases occurring in superficial organs.
The more it occurs in internal organs; IgG4-related patients have the highest serology in the 60-69-year-old population; the treatment response and recurrence rate of younger IgG4-related patients is higher than that of the older ones.
Figure: Analysis of the clinical characteristics of 737 cases of IgG4-RD in different age groups.
Commonly affected organs of IgG4-related diseases include: lacrimal gland, salivary gland, pancreas, retroperitoneum, lung, biliary tract, kidney, dura mater, aorta, thyroid, etc.
Professor Zhang Wen's team also analyzed and summarized several of them.
01.
IgG4-related aortitis/periarteritis (PAO/PA) In 2020, after reviewing patient data, Professor Zhang Wen’s team found that in patients with IgG4-related vascular disease, PAO alone accounted for 32%, and those with other organs 68% and 62% had hydronephrosis, 49% had elevated creatinine, and 35% required DJ tube drainage.
The types of patients with IgG4-related vascular disease are divided into three types.
Type 1 involves the aorta, including the ascending aorta, aortic arch or descending aorta; Type 2 involves the abdominal aorta, renal artery or internal iliac artery; Type 3 involves the blood vessels, including both type 1 and type 2.
Figure: Types of patients with IgG4-related vascular disease.
After comparing patients with IgG4-related diseases with or without vascular disease, it was found that patients with IgG4-related vascular disease accounted for a higher proportion of fibrosis and showed more obvious inflammation.
However, patients with IgG4-related diseases that are not vascular lesions are more likely to be immune.
Figure: Comparison of IgG4-RD patients with and without PAO/PA The efficacy of IgG4-related PAO/PA is generally good.
Under active treatment, 38%-44% of patients with retroperitoneal soft tissue decreased> 70%, and only 18% of patients decreased <30%; most patients with hydronephrosis and renal insufficiency improved; 2/3 of patients successfully removed DJ tube.
02.
IgG4-related sclerosing mediastinitis Approximately 2.
8% of patients with IgG4-related diseases have IgG4-related sclerosing mediastinitis.
Among them, 35% had simple mediastinitis, and 65% had other organ involvement, such as the aorta and thoracic spine.
03.
IgG4-related diseases Kidney involvement IgG4 kidney disease is divided into primary and secondary.
Primary include interstitial nephropathy, renal pelvis and ureteral disease, renal parenchymal low-density lesions or nodules, and glomerulonephritis; secondary abdominal aortic inflammation/retroperitoneal fibrosis.
When the kidney is involved, the imaging manifestations of enhanced CT can be: low-density foci, parenchymal nodules, and thickening of the perinephric capsule.
In addition, Professor Zhang Wen emphasized that sometimes IgG4-related disease kidney involvement can only manifest as renal pelvis and ureter disease, without involving the renal parenchyma.
04.
IgG4-related diseases nervous system involvement IgG4-related diseases nervous system involvement includes: hypertrophic dura mater/spinalitis, hypophysitis, brain parenchymal disease, inflammatory pseudotumor, peripheral neuropathy, and others (peripheral disease).
Professor Zhang Wen's team found that about 2.
5% of IgG4-related diseases are nervous system involvement, and they are all central neuropathy.
Among them, the most common symptom is diabetes insipidus.
In the laboratory examination of IgG4-related diseases, Professor Zhang Wen’s team found that the higher the serum IgG4 concentration, the more organs involved; the higher the serum IgG4 baseline, the worse the treatment effect.
Although in patients with IgG4-related diseases, the level of serum IgE is similar to that of IgG4.
However, it will have an upward trend during follow-up, and it cannot predict recurrence like IgG4.
Finally, Professor Zhang Wen said that due to the uneven diagnosis and treatment of IgG4-related diseases in various hospitals, the "Chinese Experts Consensus on the Diagnosis and Treatment of IgG4-related Diseases" was recently published to unify Chinese clinicians’ understanding of the diagnosis and treatment of the disease.
, Reduce missed diagnosis and misdiagnosis, and improve patient prognosis.
Picture: Screenshot of the “Consensus of Chinese Experts on the Diagnosis and Treatment of IgG4 Related Diseases” The consensus includes a total of 12 recommendations.
Colleagues in need can read the detailed content of the consensus.
Picture: Screenshot of the 12 opinions of the "Consensus of Chinese Experts on the Diagnosis and Treatment of IgG4 Related Diseases" After Professor Zhang Wen, the host, Professor Tan Wenfeng from Jiangsu Provincial People's Hospital commented that the sharing was the perfect ending to the conference.
Professor Tan Wenfeng said that Professor Zhang Wen is the voice of IgG4 Chinese in China.
What research progress will be made in 2020 on the complicated and mysterious IgG4-related diseases? Listen to Professor Xiehe’s words.
.
.
March 26-28, 2021, organized by the National Center for Clinical Research on Skin and Immune Diseases (NCRC-DID), the Chinese Association of Rheumatology and Immunology (CRCA), and the National Rheumatology Association Data Center (CRDC), China Systemic Lupus Erythematosus Research Collaboration Group (CSTAR), Chinese Rehabilitation Medicine Association, Beijing Medical Award Foundation, Zhongguancun Creative Research Institute of Medical Engineering Research Center for Immunological Diseases co-sponsored the "First National Skin and Immunity The Academic Annual Meeting of the Center for Disease Research and Clinical Medicine and the 11th CSTAR/CRDC/CRCA Annual Meeting" was successfully held in Beijing.
In the last session of the conference, the "IgG4-related disease research (PUMCH)" brought by Professor Zhang Wen from Peking Union Medical College Hospital.
Picture: Professor Zhang Wen began to share the content of the theme In view of the previous conference reports, Professor Zhang Wen has shared the research progress of IgG4-related diseases in the world.
Therefore, at this meeting, Professor Zhang Wen, based on the Union Hospital where he is located, introduced the progress made by Union Hospital in the research of IgG4-related diseases in 2020.
Basic research—finding new biomarkers Professor Zhang Wen’s team used single-cell sequencing to find several differentially expressed cell populations and major differential molecules (research has not yet been published); the gene expression of the TCR receptor library was analyzed and it was found that immune clones were in Abnormal expression of IgG4 in patients; using a lectin chip to find serum IgG4 glycosylation biomarkers; using UPLC-MS plasma metabolomics analysis, it is found that the metabolic performance of IgG4 patients is different from that of normal people.
Clinical diagnosis—discuss the pathological characteristics of IgG4-related diseases in the new method of influencing science: immune disorders, inflammation and fibrosis.
At present, CT and magnetic resonance (MRI) are still commonly used clinical imaging methods for IgG4-related diseases, but PET/CT also has special effects in the diagnosis of IgG4-related diseases.
Professor Zhang Wen's team used 68Ga-FAPI tracer (targeted fibroblast activation protein) to show the pathological characteristics of fibrosis of IgG4-related diseases, thereby assisting in the diagnosis of IgG4-related diseases.
Figure: Different tracers show different pathological types of lesions.
Clinical diagnosis and treatment-a comprehensive understanding of IgG4 related diseases Professor Zhang Wen's team analyzed the clinical characteristics of 737 patients with IgG4 related diseases.
It was found that the incidence of IgG4-related diseases between 50-70 years old is the highest among those aged 50-70 years; with age, the proportion of male IgG4-related diseases increases; the older the age, the lower the probability of IgG4-related diseases occurring in superficial organs.
The more it occurs in internal organs; IgG4-related patients have the highest serology in the 60-69-year-old population; the treatment response and recurrence rate of younger IgG4-related patients is higher than that of the older ones.
Figure: Analysis of the clinical characteristics of 737 cases of IgG4-RD in different age groups.
Commonly affected organs of IgG4-related diseases include: lacrimal gland, salivary gland, pancreas, retroperitoneum, lung, biliary tract, kidney, dura mater, aorta, thyroid, etc.
Professor Zhang Wen's team also analyzed and summarized several of them.
01.
IgG4-related aortitis/periarteritis (PAO/PA) In 2020, after reviewing patient data, Professor Zhang Wen’s team found that in patients with IgG4-related vascular disease, PAO alone accounted for 32%, and those with other organs 68% and 62% had hydronephrosis, 49% had elevated creatinine, and 35% required DJ tube drainage.
The types of patients with IgG4-related vascular disease are divided into three types.
Type 1 involves the aorta, including the ascending aorta, aortic arch or descending aorta; Type 2 involves the abdominal aorta, renal artery or internal iliac artery; Type 3 involves the blood vessels, including both type 1 and type 2.
Figure: Types of patients with IgG4-related vascular disease.
After comparing patients with IgG4-related diseases with or without vascular disease, it was found that patients with IgG4-related vascular disease accounted for a higher proportion of fibrosis and showed more obvious inflammation.
However, patients with IgG4-related diseases that are not vascular lesions are more likely to be immune.
Figure: Comparison of IgG4-RD patients with and without PAO/PA The efficacy of IgG4-related PAO/PA is generally good.
Under active treatment, 38%-44% of patients with retroperitoneal soft tissue decreased> 70%, and only 18% of patients decreased <30%; most patients with hydronephrosis and renal insufficiency improved; 2/3 of patients successfully removed DJ tube.
02.
IgG4-related sclerosing mediastinitis Approximately 2.
8% of patients with IgG4-related diseases have IgG4-related sclerosing mediastinitis.
Among them, 35% had simple mediastinitis, and 65% had other organ involvement, such as the aorta and thoracic spine.
03.
IgG4-related diseases Kidney involvement IgG4 kidney disease is divided into primary and secondary.
Primary include interstitial nephropathy, renal pelvis and ureteral disease, renal parenchymal low-density lesions or nodules, and glomerulonephritis; secondary abdominal aortic inflammation/retroperitoneal fibrosis.
When the kidney is involved, the imaging manifestations of enhanced CT can be: low-density foci, parenchymal nodules, and thickening of the perinephric capsule.
In addition, Professor Zhang Wen emphasized that sometimes IgG4-related disease kidney involvement can only manifest as renal pelvis and ureter disease, without involving the renal parenchyma.
04.
IgG4-related diseases nervous system involvement IgG4-related diseases nervous system involvement includes: hypertrophic dura mater/spinalitis, hypophysitis, brain parenchymal disease, inflammatory pseudotumor, peripheral neuropathy, and others (peripheral disease).
Professor Zhang Wen's team found that about 2.
5% of IgG4-related diseases are nervous system involvement, and they are all central neuropathy.
Among them, the most common symptom is diabetes insipidus.
In the laboratory examination of IgG4-related diseases, Professor Zhang Wen’s team found that the higher the serum IgG4 concentration, the more organs involved; the higher the serum IgG4 baseline, the worse the treatment effect.
Although in patients with IgG4-related diseases, the level of serum IgE is similar to that of IgG4.
However, it will have an upward trend during follow-up, and it cannot predict recurrence like IgG4.
Finally, Professor Zhang Wen said that due to the uneven diagnosis and treatment of IgG4-related diseases in various hospitals, the "Chinese Experts Consensus on the Diagnosis and Treatment of IgG4-related Diseases" was recently published to unify Chinese clinicians’ understanding of the diagnosis and treatment of the disease.
, Reduce missed diagnosis and misdiagnosis, and improve patient prognosis.
Picture: Screenshot of the “Consensus of Chinese Experts on the Diagnosis and Treatment of IgG4 Related Diseases” The consensus includes a total of 12 recommendations.
Colleagues in need can read the detailed content of the consensus.
Picture: Screenshot of the 12 opinions of the "Consensus of Chinese Experts on the Diagnosis and Treatment of IgG4 Related Diseases" After Professor Zhang Wen, the host, Professor Tan Wenfeng from Jiangsu Provincial People's Hospital commented that the sharing was the perfect ending to the conference.
Professor Tan Wenfeng said that Professor Zhang Wen is the voice of IgG4 Chinese in China.
