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*Only for medical professionals to read for reference.
Is it difficult to treat? That also has to be cured ~ Myositis is a disease often encountered by rheumatologists.
It is often manifested as muscle weakness and weakened muscle endurance.
Pathology can show inflammatory cell infiltration in muscle tissue
.
Myositis is difficult to treat because of its strong heterogeneity, but a more difficult challenge-idiopathic inflammatory myopathy (IIM), which is not ideal, makes the current undesirable treatment situation worse
.
How is this type of refractory myositis diagnosed and treated? Follow Professor Yan Geng from Peking University First Hospital to review the 2021 American Academy of Rheumatology Annual Meeting (ACR 2021), and you may get some new inspiration in front of the screen
.
Recognize that IIM/myositis IIM/myositis has various clinical manifestations.
In addition to symptoms such as muscle weakness, systemic symptoms are often seen, such as fever, rash, Raynaud’s phenomenon, mechanic hands, interstitial lung disease, myocarditis, arthritis or Nailfold capillary changes and other non-specific symptoms
.
Combined with recent research, the current clinical practice uses myositis-specific autoantibodies (MSA) as the classification criteria for IIM/myositis in children and adults.
Not all patients with IIM/myositis can be detected with MSA, and there are still 20% -50% of patients were negative for MSA antibodies
.
Figure 1: According to pathological and clinical data, it is divided into inclusion body myositis, dermatomyositis, immune-mediated necrotizing myopathy, and polymyositis.
What are the therapeutic goals of IIM/myositis? IIM/myositis is the same as many rheumatic immune diseases The overall treatment goal of treatment is mainly to improve body function and improve the quality of life of patients, such as eliminating inflammation, preventing disease damage, restoring muscle strength and muscle endurance, restoring lung function, eliminating skin rashes, and controlling arthritis
.
At present, the high-level clinical evidence on IIM/myositis is relatively lacking, and the treatment options are mostly based on case reports and expert experience: the first-line regimen is mainly glucocorticoid + immunosuppressant (methotrexate, azathioprine); and if The first-line treatment is not effective, the second-line treatment can consider the use of mycophenolate mofetil, tacrolimus, cyclosporine and other immunomodulatory drugs under glucocorticoid therapy, or even combined with the first-line treatment plan; the third-line treatment plan can consider glucocorticoids Hormone + rituximab, other biological agents or cyclophosphamide; it should be emphasized that each line of treatment can be used in combination or intravenous infusion of gamma globulin alone
.
Figure 2: Common IIM/myositis clinical manifestations The prognosis assessment of IIM/myositis is based on disease activity, disease damage [often using International Myositis Clinical Research Working Group (IMACS) standards] and quality of life (often using SF-36) Scale) as an indicator
.
Among them, IMACS' core indicators for assessing IIM/myositis disease activity include: overall visual analogue scores of both doctors and patients, muscle strength assessment (MMT-8 scale), body function (HAQ scale), and muscle enzyme laboratory examinations [muscles] At least two of acid phosphokinase (CK), lactate dehydrogenase (LDH), aldolase, alanine aminotransferase (ALT) or aspartate aminotransferase (AST)] and assessment of disease activity other than skeletal muscle
.
For children or adult patients who meet the following criteria, it is recognized as disease improvement: more than 3 core indicators improved by ≥20%, and less than 2 core indicators deteriorated ≥25% (if the deterioration indicator is muscle strength, it is not recognized as Disease improvement) Practical experience: a case of refractory myositis is finally shallow on paper, then through the case of refractory myositis, the understanding of treatment options for myositis will be more profound
.
This case is a 37-year-old female who was diagnosed in 2006.
There was no family history of myopathy or rheumatism.
Only the progressive weakening of the pelvic girdle muscles was seen, without any other systemic manifestations
.
Figure 3: Part of the clinical data of the patient The attending doctor, after careful consideration, combined with the clinical data, diagnosed polymyositis
.
However, after following the above treatment for half a year, the doctor found that the patient’s muscle strength was weakened and did not improve, so he couldn’t help but mumble: Is this patient refractory myositis? If the patient’s inflammation progresses or the disease is more damaged, how should I evaluate it? ? The answer is obvious.
When a patient with persistent muscle strength weakened myositis after 3-6 months of treatment, the core indicators still do not improve, it can be judged as refractory myositis
.
After clinical screening of such patients, the core evaluation indicators should be used to grasp their condition, and if necessary, muscle biopsy should be repeated to determine the disease state
.
If the evaluation of the core indicators shows that the patient does not have a worsening of myopathy, then the optimization of the hormone regimen can be considered; otherwise, the dermatology, oncology and other multidisciplinary consultations should be excluded to confirm the diagnosis of IIM
.
When the patient returned 1 year later, the attending doctor performed a muscle biopsy.
It showed that there was no obvious pathological changes related to myositis, and the assessment of weakened muscle strength did not improve.
Only the serum CK was found to be too high, and the muscle system was not seen.
External performance
.
The attending doctor is still hesitant to confirm the diagnosis: Although the patient's disease is active, it is difficult to diagnose refractory myositis with negative MSA
.
A neurologist is requested to evaluate the diagnosis, and the consultation opinion is considered to be limb-girdle muscular dystrophy and late-onset Pompe disease (ie glycogen deposition disease), but the genetics and enzyme related tests involved in this consideration are negative
.
This can make the attending doctor difficult again: Is it refractory myositis or limb-girdle muscular dystrophy? Difficult to diagnose, we had to choose long-term follow-up
.
After 12 years, the patient participated in a clinical study and donated a blood sample, and unexpectedly tested positive for serum anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) autoantibody
.
The investigator of the clinical study immediately contacted the patient's attending doctor many years ago, and the attending doctor took a muscle biopsy and MRI examination showed that: anti-HMGCR positive
.
There is no need to entangle the name of this antibody, just know one thing: this is in line with the diagnosis of immune-mediated necrotizing myopathy, and this type of myositis usually does not respond well to conventional immunosuppressive therapy
.
At this point, the patient finally got a clear diagnosis: refractory myositis
.
So the doctor began to use high-dose intravenous immunoglobulin to treat it.
Finally, after more than ten years of deterioration in muscle strength, the patient saw for the first time that his symptoms improved-muscle strength was recovering! The patient’s refractory myositis is classified as immune-mediated necrotizing myopathy (IMNM).
Clinically, there are obvious proximal muscle weakness and dysphagia, while lung involvement is relatively rare.
Examination shows high serum CK levels and pathology.
Muscle fiber necrosis and inflammation are not obvious.
If the myocardium is affected, anti-signal recognition particles (SRP) antibodies can be seen positive
.
The anti-HMGCR found in this patient can generally also be positive in patients using statins, and can be seen in children or adults without statin treatment
.
If the same patient is encountered in the clinic, but the serological examination is negative, tumor diseases must be excluded
.
This patient not only brought a lot of enlightenment for refractory myositis, but also reminded the doctor: When a patient suspected of muscular dystrophy has not seen a positive gene test, IMNM can be considered to be excluded
.
If anti-HMGCR antibody appears in patients who are not treated with statins, its diagnostic value can be comprehensively evaluated
.
If the condition is limited and the diagnosis cannot be made, the doctor's first concern should be to identify the source of the patient's symptoms from inflammation or injury
.
If conditions permit, muscle biopsy can be repeated if necessary, and the treatment plan can be adjusted in time according to the changes in the diagnosis
.
Conclusion When facing patients with myositis clinically, the cause of inflammation or injury should be considered first, and the diagnosis can be repeated if necessary to avoid misdiagnosis
.
If it is difficult for patients with myositis to confirm the cause, when considering IIM, the intervention should follow the treatment guidelines mentioned above.
If the effect is not good, the immunosuppressive agent needs to be replaced
.
If the effect is acceptable after changing the drug, you can consider continuing the current treatment and gradually reducing the hormone dose
.
On the contrary, it is necessary to consider whether to take MSA testing based on the symptoms and manifestations, while considering the use of biological agents or intravenous gamma globulin treatment.
If the effect is good, hormonal program optimization should be considered, otherwise, other biological agents should be considered
.
Among the treatment principles, the most important thing is to formulate an individualized diagnosis and treatment plan based on the myositis classification, and it is recommended that physical exercise combined with treatment be intervened at the same time
.
Expert profile Geng Yan, Deputy Chief Physician and Associate Professor, Department of Rheumatology and Immunology, Peking University First Hospital
.
Member and Secretary of the Youth Committee of the Chinese Medical Association Rheumatology Branch, Member of the Rheumatology Imaging Group of the Cross-Strait Medical and Health Exchange Association, and Member and Secretary of the Imaging Group of the Rheumatology and Immunology Physicians Branch of the Chinese Medical Doctor Association
.
European Union Against Rheumatism (EULAR) certified international musculoskeletal ultrasound trainer
.
Pay attention to the video number of the rheumatism and immunity channel in the medical field.
Here are the most professional and interesting texts.
If you are tired, just take a look at the video to ensure that you will open the door to a new world.
Is it difficult to treat? That also has to be cured ~ Myositis is a disease often encountered by rheumatologists.
It is often manifested as muscle weakness and weakened muscle endurance.
Pathology can show inflammatory cell infiltration in muscle tissue
.
Myositis is difficult to treat because of its strong heterogeneity, but a more difficult challenge-idiopathic inflammatory myopathy (IIM), which is not ideal, makes the current undesirable treatment situation worse
.
How is this type of refractory myositis diagnosed and treated? Follow Professor Yan Geng from Peking University First Hospital to review the 2021 American Academy of Rheumatology Annual Meeting (ACR 2021), and you may get some new inspiration in front of the screen
.
Recognize that IIM/myositis IIM/myositis has various clinical manifestations.
In addition to symptoms such as muscle weakness, systemic symptoms are often seen, such as fever, rash, Raynaud’s phenomenon, mechanic hands, interstitial lung disease, myocarditis, arthritis or Nailfold capillary changes and other non-specific symptoms
.
Combined with recent research, the current clinical practice uses myositis-specific autoantibodies (MSA) as the classification criteria for IIM/myositis in children and adults.
Not all patients with IIM/myositis can be detected with MSA, and there are still 20% -50% of patients were negative for MSA antibodies
.
Figure 1: According to pathological and clinical data, it is divided into inclusion body myositis, dermatomyositis, immune-mediated necrotizing myopathy, and polymyositis.
What are the therapeutic goals of IIM/myositis? IIM/myositis is the same as many rheumatic immune diseases The overall treatment goal of treatment is mainly to improve body function and improve the quality of life of patients, such as eliminating inflammation, preventing disease damage, restoring muscle strength and muscle endurance, restoring lung function, eliminating skin rashes, and controlling arthritis
.
At present, the high-level clinical evidence on IIM/myositis is relatively lacking, and the treatment options are mostly based on case reports and expert experience: the first-line regimen is mainly glucocorticoid + immunosuppressant (methotrexate, azathioprine); and if The first-line treatment is not effective, the second-line treatment can consider the use of mycophenolate mofetil, tacrolimus, cyclosporine and other immunomodulatory drugs under glucocorticoid therapy, or even combined with the first-line treatment plan; the third-line treatment plan can consider glucocorticoids Hormone + rituximab, other biological agents or cyclophosphamide; it should be emphasized that each line of treatment can be used in combination or intravenous infusion of gamma globulin alone
.
Figure 2: Common IIM/myositis clinical manifestations The prognosis assessment of IIM/myositis is based on disease activity, disease damage [often using International Myositis Clinical Research Working Group (IMACS) standards] and quality of life (often using SF-36) Scale) as an indicator
.
Among them, IMACS' core indicators for assessing IIM/myositis disease activity include: overall visual analogue scores of both doctors and patients, muscle strength assessment (MMT-8 scale), body function (HAQ scale), and muscle enzyme laboratory examinations [muscles] At least two of acid phosphokinase (CK), lactate dehydrogenase (LDH), aldolase, alanine aminotransferase (ALT) or aspartate aminotransferase (AST)] and assessment of disease activity other than skeletal muscle
.
For children or adult patients who meet the following criteria, it is recognized as disease improvement: more than 3 core indicators improved by ≥20%, and less than 2 core indicators deteriorated ≥25% (if the deterioration indicator is muscle strength, it is not recognized as Disease improvement) Practical experience: a case of refractory myositis is finally shallow on paper, then through the case of refractory myositis, the understanding of treatment options for myositis will be more profound
.
This case is a 37-year-old female who was diagnosed in 2006.
There was no family history of myopathy or rheumatism.
Only the progressive weakening of the pelvic girdle muscles was seen, without any other systemic manifestations
.
Figure 3: Part of the clinical data of the patient The attending doctor, after careful consideration, combined with the clinical data, diagnosed polymyositis
.
However, after following the above treatment for half a year, the doctor found that the patient’s muscle strength was weakened and did not improve, so he couldn’t help but mumble: Is this patient refractory myositis? If the patient’s inflammation progresses or the disease is more damaged, how should I evaluate it? ? The answer is obvious.
When a patient with persistent muscle strength weakened myositis after 3-6 months of treatment, the core indicators still do not improve, it can be judged as refractory myositis
.
After clinical screening of such patients, the core evaluation indicators should be used to grasp their condition, and if necessary, muscle biopsy should be repeated to determine the disease state
.
If the evaluation of the core indicators shows that the patient does not have a worsening of myopathy, then the optimization of the hormone regimen can be considered; otherwise, the dermatology, oncology and other multidisciplinary consultations should be excluded to confirm the diagnosis of IIM
.
When the patient returned 1 year later, the attending doctor performed a muscle biopsy.
It showed that there was no obvious pathological changes related to myositis, and the assessment of weakened muscle strength did not improve.
Only the serum CK was found to be too high, and the muscle system was not seen.
External performance
.
The attending doctor is still hesitant to confirm the diagnosis: Although the patient's disease is active, it is difficult to diagnose refractory myositis with negative MSA
.
A neurologist is requested to evaluate the diagnosis, and the consultation opinion is considered to be limb-girdle muscular dystrophy and late-onset Pompe disease (ie glycogen deposition disease), but the genetics and enzyme related tests involved in this consideration are negative
.
This can make the attending doctor difficult again: Is it refractory myositis or limb-girdle muscular dystrophy? Difficult to diagnose, we had to choose long-term follow-up
.
After 12 years, the patient participated in a clinical study and donated a blood sample, and unexpectedly tested positive for serum anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) autoantibody
.
The investigator of the clinical study immediately contacted the patient's attending doctor many years ago, and the attending doctor took a muscle biopsy and MRI examination showed that: anti-HMGCR positive
.
There is no need to entangle the name of this antibody, just know one thing: this is in line with the diagnosis of immune-mediated necrotizing myopathy, and this type of myositis usually does not respond well to conventional immunosuppressive therapy
.
At this point, the patient finally got a clear diagnosis: refractory myositis
.
So the doctor began to use high-dose intravenous immunoglobulin to treat it.
Finally, after more than ten years of deterioration in muscle strength, the patient saw for the first time that his symptoms improved-muscle strength was recovering! The patient’s refractory myositis is classified as immune-mediated necrotizing myopathy (IMNM).
Clinically, there are obvious proximal muscle weakness and dysphagia, while lung involvement is relatively rare.
Examination shows high serum CK levels and pathology.
Muscle fiber necrosis and inflammation are not obvious.
If the myocardium is affected, anti-signal recognition particles (SRP) antibodies can be seen positive
.
The anti-HMGCR found in this patient can generally also be positive in patients using statins, and can be seen in children or adults without statin treatment
.
If the same patient is encountered in the clinic, but the serological examination is negative, tumor diseases must be excluded
.
This patient not only brought a lot of enlightenment for refractory myositis, but also reminded the doctor: When a patient suspected of muscular dystrophy has not seen a positive gene test, IMNM can be considered to be excluded
.
If anti-HMGCR antibody appears in patients who are not treated with statins, its diagnostic value can be comprehensively evaluated
.
If the condition is limited and the diagnosis cannot be made, the doctor's first concern should be to identify the source of the patient's symptoms from inflammation or injury
.
If conditions permit, muscle biopsy can be repeated if necessary, and the treatment plan can be adjusted in time according to the changes in the diagnosis
.
Conclusion When facing patients with myositis clinically, the cause of inflammation or injury should be considered first, and the diagnosis can be repeated if necessary to avoid misdiagnosis
.
If it is difficult for patients with myositis to confirm the cause, when considering IIM, the intervention should follow the treatment guidelines mentioned above.
If the effect is not good, the immunosuppressive agent needs to be replaced
.
If the effect is acceptable after changing the drug, you can consider continuing the current treatment and gradually reducing the hormone dose
.
On the contrary, it is necessary to consider whether to take MSA testing based on the symptoms and manifestations, while considering the use of biological agents or intravenous gamma globulin treatment.
If the effect is good, hormonal program optimization should be considered, otherwise, other biological agents should be considered
.
Among the treatment principles, the most important thing is to formulate an individualized diagnosis and treatment plan based on the myositis classification, and it is recommended that physical exercise combined with treatment be intervened at the same time
.
Expert profile Geng Yan, Deputy Chief Physician and Associate Professor, Department of Rheumatology and Immunology, Peking University First Hospital
.
Member and Secretary of the Youth Committee of the Chinese Medical Association Rheumatology Branch, Member of the Rheumatology Imaging Group of the Cross-Strait Medical and Health Exchange Association, and Member and Secretary of the Imaging Group of the Rheumatology and Immunology Physicians Branch of the Chinese Medical Doctor Association
.
European Union Against Rheumatism (EULAR) certified international musculoskeletal ultrasound trainer
.
Pay attention to the video number of the rheumatism and immunity channel in the medical field.
Here are the most professional and interesting texts.
If you are tired, just take a look at the video to ensure that you will open the door to a new world.
