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*Only for medical professionals to read and refer to the treatment of scleroderma and comorbidities here! Co-sponsored by the National Clinical Research Center for Skin and Immune Diseases (NCRC-DID), China Rheumatism and Immunology Medical Association (CRCA), National Rheumatism Data Center (CRDC), and China Systemic Lupus Erythematosus Research Collaboration Group (CSTAR) The "First National Clinical Research Center for Skin and Immune Diseases Annual Conference and CSTAR/CRDC/CRCA Annual Conference" has successfully concluded.
At the meeting, Professor Hou Yong from the Department of Rheumatology and Immunology of Peking Union Medical College Hospital introduced the progress of diagnosis and treatment of systemic sclerosis (SSc).
Focal scleroderma and SSc coexist Professor Hou Yong introduced that a question often asked by outpatients is, "I have focal scleroderma (LoS), will I still get SSc?" This question can be found in the literature.
Find the answer in.
The first systematic literature review on scleroderma investigated the coexistence of SSc and LoS.
The review pointed out that the coexistence of SSc and LoS is higher than their individual prevalence in the normal population.
The incidence of both coexistence is 2.
4%~7.
4%.
The order of appearance of the two is not fixed.
SSc may appear first, may appear later, or both appear at the same time.
The onset of SSc in the elderly can occur at any age, but most patients are 40-50 years old.
A German study showed that elderly patients are more likely to have localized cutaneous SSc (lcSSc) subgroups at the onset of the disease.
They have more frequent pulmonary hypertension, but fewer finger ulcers.
This type of patients (60+) progresses faster, and the onset of internal organ involvement is significantly accelerated.
Therefore, more frequent follow-up examinations are required to detect organ complications early.
The onset of the disease in old age is a risk factor for the progression of the disease.
In SSc patients, acrogangrene tends to recur.
Patients with diffuse skin SSc and finger ulcers have a higher risk of gangrene.
In SSc, traditional cardiovascular risk factors are not closely related to gangrene.
SSc Renal Crisis Peking Union Medical College Hospital analyzed 25 SSc patients who underwent renal puncture and found that 3 patients had two renal punctures due to lupus nephritis (LN).
Among the remaining 22 patients, 9 patients had SSc renal crisis (SRC); 1 patient had both SRC and type Ⅲ LN; 4 patients had LN; 1 patient had aristolochic acid nephropathy; 2 patients had combined resistance Renal damage caused by neutrophil cytoplasmic antibody (ANCA)-related vasculitis.
It can be seen that kidney damage in patients with SSc can be caused by SRC, other diseases caused by SSc, or other kidney damage caused by overlapping systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA).
From 2009 to 2016, there were 538 confirmed SSc patients in Peking Union Medical College Hospital.
A cohort analysis of these patients showed that SRC is a rare complication in Chinese patients, with a cohort prevalence rate of 5.
4%.
The main causes of death in SRC patients are dialysis discontinuation and cardiac complications.
SRC patients with serum creatinine (SCr) levels> 500 μmol/L or require dialysis, are associated with poor prognosis.
Foreign studies have shown that 40% to 50% of SRC cases can show signs of microvascular disease.
Recent studies have proposed that SSc thrombotic microangiopathies (TMA) is a condition different from narrow-sense SRC.
Disorders of the complement pathway play a key pathogenic role in SRC manifested by TMA.
Therefore, plasma can be considered in severely refractory cases.
Replacement.
SSc combined with hypertension When hypertension occurs in patients with SSc, the treatment process is shown in Figure 1.
Figure 1 SSc combined with hypertension management process overlap syndrome.
The screening of 534 SSc patients in Bordeaux and Strasbourg University Hospitals found that 34 cases of SSc overlapped with RA, Sjogren syndrome (SgS) and/or SLE. Twenty-one patients had RA, 14 had SgS, and 4 had SLE.
Five patients were accompanied by two autoimmune diseases (4 patients with RA+SgS and 1 patient with SLE+SgS).
RA, SgS, and SLE are more than 6% of SSc patients; the prognosis of patients with overlapping SSc in Sjogren’s syndrome may be worse than that of patients with non-overlapping SSc.
Patients with overlapping SSc are more likely to receive glucocorticoids including doses> 15 mg/d, which is related to SRC.
Patients with RA overlapping SSc will receive more glucocorticoids, methotrexate, and biological agents.
It is recommended to actively screen for concurrent autoimmune diseases in patients with SSc, because this may directly affect the prognosis and treatment options.
Figure 2 shows the diagnosis and treatment process of SSc overlap syndrome in the 2016 British Rheumatology Association and British Association of Rheumatology Health Professionals (BSR and BHPR) guidelines.
Figure 2 SSc overlap syndrome diagnosis and treatment process Youth SSc management recommendations BSR issued consensus-based youth (jSSc) management recommendations as follows: Evaluation 1.
General principles: all children suspected of jSSc should be transferred to a specialized pediatric rheumatology center for multidisciplinary treatment.
2.
Vascular involvement: All patients with RP alone should undergo nailfold capillary assessment and antinuclear antibody (ANA) testing.
If the capillary microscopy is abnormal or the ANA is positive, regular follow-up is recommended.
3.
Skin involvement: Standardized skin scoring tools should be used for clinical injury assessment.
The modified Rodnan skin score is suitable, but needs to be adapted and validated for use by children.
4.
Visceral damage: jSSc severity score (j4s) can be used as a severity assessment tool for jSSc, but it needs to be verified.
5.
Lung function tests, including diffusible carbon monoxide (DLCO) and DRCT, are very sensitive to the presence and severity of interstitial lung diseases.
Pulmonary function test is also an indicator of respiratory function monitoring.
6.
Patients with jSSc should undergo lung function tests including DLCO, echocardiography, renal function and modified Rodnan skin score at least every 6 months.
Treatment 1.
Skin involvement: Systemic corticosteroids combined with disease-improving anti-rheumatic drugs (DMARDs) are useful in the active inflammatory phase of jSSc.
2.
When diagnosing jSSc, systemic immunomodulatory therapy such as methotrexate should be considered.
3.
If there is no response to methotrexate, the immunomodulatory mycophenolate mofetil (MMF) should be considered.
4.
Lung involvement: Cyclophosphamide can be used to treat heart and/or lung involvement.
5.
Vascular involvement: Iloprost can be used to treat ischemic digits and finger ulcers.
6.
For New York Grade II pulmonary hypertension and/or other treatments that are ineffective for finger ulcers, bosentan should be considered.
7.
Experimental treatment: Biological agents, especially tocilizumab or rituximab, should be considered for use in severe or refractory cases.
8.
Autologous stem cell transplantation may be an option for patients with progressively progressing jSSc that are ineffective in immunosuppressive therapy.
See the figure below for the diagnosis and treatment process of jSSc.
Figure 3 JSSc diagnosis and treatment process Tofacitinib research study found that for patients with progressive skin sclerosis and refractory diffuse SSc (dcSSc), tofacitinib is as effective as or even better than conventional immunosuppressive agents, with faster onset.
More efficient.
SSc pulmonary interstitial disease SSc pulmonary interstitial disease (ILD) has become the most important cause of death in SSc, and has received increasing clinical attention.
Studies have shown that 70% to 80% of SSc patients have SSc-ILD, and their disease processes are different and have great heterogeneity.
Some patients are in stable condition, but 25%~30% of patients will experience progressive aggravation.
It is very important to closely monitor all patients.
Risk factors for the progression of SSc-ILD include males, advanced age, African Americans, diffuse skin lesions, early disease (within 5 years after diagnosis), autoantibodies, Scl-70, and lung function (FVC) decline within 1 year (> 10%) )Wait.
The large-scale clinical trial SENSCIS shows that Nintedanib can delay the decline of SSc-ILD lung function, and it has been approved for the treatment of SSc-ILD abroad.
The processing flow of SSc-ILD is shown in Figure 4.
Figure 4 SSc-ILD treatment process Professor Hou Yong concluded that the results of the EUSTAR study have greatly improved the diagnosis and treatment of SSc in the past 15 years.
In the future, further efforts are needed to determine the early prognostic markers of the disease and stratify the patients who may benefit the most from vasoactivity, immunosuppression, and/or anti-fibrosis therapy.
For SSc, the principles of early diagnosis, individualization, stratification and combined treatment should be adopted.
At the meeting, Professor Hou Yong from the Department of Rheumatology and Immunology of Peking Union Medical College Hospital introduced the progress of diagnosis and treatment of systemic sclerosis (SSc).
Focal scleroderma and SSc coexist Professor Hou Yong introduced that a question often asked by outpatients is, "I have focal scleroderma (LoS), will I still get SSc?" This question can be found in the literature.
Find the answer in.
The first systematic literature review on scleroderma investigated the coexistence of SSc and LoS.
The review pointed out that the coexistence of SSc and LoS is higher than their individual prevalence in the normal population.
The incidence of both coexistence is 2.
4%~7.
4%.
The order of appearance of the two is not fixed.
SSc may appear first, may appear later, or both appear at the same time.
The onset of SSc in the elderly can occur at any age, but most patients are 40-50 years old.
A German study showed that elderly patients are more likely to have localized cutaneous SSc (lcSSc) subgroups at the onset of the disease.
They have more frequent pulmonary hypertension, but fewer finger ulcers.
This type of patients (60+) progresses faster, and the onset of internal organ involvement is significantly accelerated.
Therefore, more frequent follow-up examinations are required to detect organ complications early.
The onset of the disease in old age is a risk factor for the progression of the disease.
In SSc patients, acrogangrene tends to recur.
Patients with diffuse skin SSc and finger ulcers have a higher risk of gangrene.
In SSc, traditional cardiovascular risk factors are not closely related to gangrene.
SSc Renal Crisis Peking Union Medical College Hospital analyzed 25 SSc patients who underwent renal puncture and found that 3 patients had two renal punctures due to lupus nephritis (LN).
Among the remaining 22 patients, 9 patients had SSc renal crisis (SRC); 1 patient had both SRC and type Ⅲ LN; 4 patients had LN; 1 patient had aristolochic acid nephropathy; 2 patients had combined resistance Renal damage caused by neutrophil cytoplasmic antibody (ANCA)-related vasculitis.
It can be seen that kidney damage in patients with SSc can be caused by SRC, other diseases caused by SSc, or other kidney damage caused by overlapping systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA).
From 2009 to 2016, there were 538 confirmed SSc patients in Peking Union Medical College Hospital.
A cohort analysis of these patients showed that SRC is a rare complication in Chinese patients, with a cohort prevalence rate of 5.
4%.
The main causes of death in SRC patients are dialysis discontinuation and cardiac complications.
SRC patients with serum creatinine (SCr) levels> 500 μmol/L or require dialysis, are associated with poor prognosis.
Foreign studies have shown that 40% to 50% of SRC cases can show signs of microvascular disease.
Recent studies have proposed that SSc thrombotic microangiopathies (TMA) is a condition different from narrow-sense SRC.
Disorders of the complement pathway play a key pathogenic role in SRC manifested by TMA.
Therefore, plasma can be considered in severely refractory cases.
Replacement.
SSc combined with hypertension When hypertension occurs in patients with SSc, the treatment process is shown in Figure 1.
Figure 1 SSc combined with hypertension management process overlap syndrome.
The screening of 534 SSc patients in Bordeaux and Strasbourg University Hospitals found that 34 cases of SSc overlapped with RA, Sjogren syndrome (SgS) and/or SLE. Twenty-one patients had RA, 14 had SgS, and 4 had SLE.
Five patients were accompanied by two autoimmune diseases (4 patients with RA+SgS and 1 patient with SLE+SgS).
RA, SgS, and SLE are more than 6% of SSc patients; the prognosis of patients with overlapping SSc in Sjogren’s syndrome may be worse than that of patients with non-overlapping SSc.
Patients with overlapping SSc are more likely to receive glucocorticoids including doses> 15 mg/d, which is related to SRC.
Patients with RA overlapping SSc will receive more glucocorticoids, methotrexate, and biological agents.
It is recommended to actively screen for concurrent autoimmune diseases in patients with SSc, because this may directly affect the prognosis and treatment options.
Figure 2 shows the diagnosis and treatment process of SSc overlap syndrome in the 2016 British Rheumatology Association and British Association of Rheumatology Health Professionals (BSR and BHPR) guidelines.
Figure 2 SSc overlap syndrome diagnosis and treatment process Youth SSc management recommendations BSR issued consensus-based youth (jSSc) management recommendations as follows: Evaluation 1.
General principles: all children suspected of jSSc should be transferred to a specialized pediatric rheumatology center for multidisciplinary treatment.
2.
Vascular involvement: All patients with RP alone should undergo nailfold capillary assessment and antinuclear antibody (ANA) testing.
If the capillary microscopy is abnormal or the ANA is positive, regular follow-up is recommended.
3.
Skin involvement: Standardized skin scoring tools should be used for clinical injury assessment.
The modified Rodnan skin score is suitable, but needs to be adapted and validated for use by children.
4.
Visceral damage: jSSc severity score (j4s) can be used as a severity assessment tool for jSSc, but it needs to be verified.
5.
Lung function tests, including diffusible carbon monoxide (DLCO) and DRCT, are very sensitive to the presence and severity of interstitial lung diseases.
Pulmonary function test is also an indicator of respiratory function monitoring.
6.
Patients with jSSc should undergo lung function tests including DLCO, echocardiography, renal function and modified Rodnan skin score at least every 6 months.
Treatment 1.
Skin involvement: Systemic corticosteroids combined with disease-improving anti-rheumatic drugs (DMARDs) are useful in the active inflammatory phase of jSSc.
2.
When diagnosing jSSc, systemic immunomodulatory therapy such as methotrexate should be considered.
3.
If there is no response to methotrexate, the immunomodulatory mycophenolate mofetil (MMF) should be considered.
4.
Lung involvement: Cyclophosphamide can be used to treat heart and/or lung involvement.
5.
Vascular involvement: Iloprost can be used to treat ischemic digits and finger ulcers.
6.
For New York Grade II pulmonary hypertension and/or other treatments that are ineffective for finger ulcers, bosentan should be considered.
7.
Experimental treatment: Biological agents, especially tocilizumab or rituximab, should be considered for use in severe or refractory cases.
8.
Autologous stem cell transplantation may be an option for patients with progressively progressing jSSc that are ineffective in immunosuppressive therapy.
See the figure below for the diagnosis and treatment process of jSSc.
Figure 3 JSSc diagnosis and treatment process Tofacitinib research study found that for patients with progressive skin sclerosis and refractory diffuse SSc (dcSSc), tofacitinib is as effective as or even better than conventional immunosuppressive agents, with faster onset.
More efficient.
SSc pulmonary interstitial disease SSc pulmonary interstitial disease (ILD) has become the most important cause of death in SSc, and has received increasing clinical attention.
Studies have shown that 70% to 80% of SSc patients have SSc-ILD, and their disease processes are different and have great heterogeneity.
Some patients are in stable condition, but 25%~30% of patients will experience progressive aggravation.
It is very important to closely monitor all patients.
Risk factors for the progression of SSc-ILD include males, advanced age, African Americans, diffuse skin lesions, early disease (within 5 years after diagnosis), autoantibodies, Scl-70, and lung function (FVC) decline within 1 year (> 10%) )Wait.
The large-scale clinical trial SENSCIS shows that Nintedanib can delay the decline of SSc-ILD lung function, and it has been approved for the treatment of SSc-ILD abroad.
The processing flow of SSc-ILD is shown in Figure 4.
Figure 4 SSc-ILD treatment process Professor Hou Yong concluded that the results of the EUSTAR study have greatly improved the diagnosis and treatment of SSc in the past 15 years.
In the future, further efforts are needed to determine the early prognostic markers of the disease and stratify the patients who may benefit the most from vasoactivity, immunosuppression, and/or anti-fibrosis therapy.
For SSc, the principles of early diagnosis, individualization, stratification and combined treatment should be adopted.
