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*Only for medical professionals to read for reference.
In the process of diagnosing and treating this difficult case, the clinician has been alarmed at every step and turned danger into a breeze! Solving a difficult case is like closing a case.
Only by constantly asking more questions and looking for answers in every detail can the truth be revealed.
At the 3rd Peking University Rheumatology and Immunology Hotspot Forum, the attending physician Zhang Haoze from the Department of Rheumatology and Immunology, Peking University First Hospital, brought us a wonderful case report.
This article summarizes the main content of this case and invites everyone to come to the conclusion of the case~ Case introduction patient A 17-year-old woman who developed skin rash and low-grade fever 10 years ago was diagnosed as "systemic lupus erythematosus and lupus nephritis" in an outside hospital, and was treated with prednisone combined with immunosuppressive agents.
Three years ago, the patient had a new rash.
Considering the recurrence of lupus, he continued to receive effective prednisone combined with immunosuppressive therapy.
The patient started to reduce the hormone dose 1 year ago, and the patient stopped taking the drug by himself 9 months ago.
Two months ago, the patient recurred fever and cheek rash, accompanied by cough, suffocation, inability to lie down, abdominal pain, nausea, vomiting, hematuria, proteinuria, and deterioration of renal function.
One month ago, the patient developed headache, blurred vision, nosebleeds, bleeding spots on both lower extremities, continued deterioration of renal function, decreased urine output, increased serum potassium, severe anemia, multiple serous effusions, and increased blood pressure As high as 180/130mmHg, prednisone 50mg/d is ineffective.
Two days ago, the patient's renal function deteriorated, blood transfusion and diuretic treatment were not effective, and severe anemia could not be corrected, so he came to the hospital for treatment (see Figure 1).
Figure 1 Brief introduction of the medical history Case characteristics: Young female, medical history for 10 years, chronic course, acute exacerbation, skin rash, nephritis and acute kidney injury, anemia, multiple serous effusion, laboratory tests have ANA, anti-Sm antibody, anti-nRNP antibody , Anti-dsDNA antibody is positive, complement is reduced, hormone therapy is effective, and relapse after drug withdrawal.
For every patient who considers systemic lupus erythematosus (SLE) for diagnosis, we often need to answer the following 3 questions (see Figure 2): 1.
Is it SLE? 2.
Which organs or systems are affected? 3.
Are there comorbidities? After answering these three questions, we can make the final diagnosis~ Figure 2 Is there any organ damage in the trilogy of SLE diagnosis? So, this patient is initially considering SLE, what organs or systems are involved? We will analyze in detail through the following points: 1.
The patient has cough and suffocation symptoms.
Is the heart involved? Or is it lung disease? Patients have symptoms such as edema, hypertension, and inability to lie on their backs, and further improve BNP and cardiac color Doppler ultrasound.
BNP: 3148pg/ml, color Doppler ultrasound showed enlarged left atrium, severe mitral regurgitation, moderate tricuspid regurgitation, pulmonary artery systolic pressure 69.
2mmHg, and a small amount of pericardial effusion.
Lung imaging and etiological examinations do not support lung infection and lupus lung involvement.
Therefore, consider the patient's cough and suffocation symptoms, the main reason is heart failure.
There may be multiple mechanisms involved in the manifestations of heart failure in patients.
Oligouria causes an increase in preload, hypertension causes an increase in afterload, anemia can also cause an increase in heart rate and hypoxia, and pulmonary hypertension causes an increase in right heart afterload.
2.
The patient also has symptoms of kidney damage.
Is it caused by prerenal, lupus nephritis or malignant hypertension? The patient has fever, heart failure and low intake, and is given symptomatic treatment and continued observation.
The patient has a history of discontinuation of the drug, deterioration of renal function, and appearance of the rash at the same time.
Further diagnosis requires a renal biopsy to confirm lupus nephritis.
The patient's blood pressure is 180/130mmHg, and it is necessary to control blood pressure and perfect kidney biopsy.
3.
The patient's blood system shows anemia and thrombocytopenia.
Consider lupus blood system involvement, bone marrow disease, or other reasons? Lupus causes blood system involvement is more common, the patient’s Coombs test result is negative, and the reticulocyte count is normal.
Patients with acute kidney injury, anemia, thrombocytopenia, fever, and thrombotic microangiopathies (TMA) need to be further improved to determine the detection of broken red blood cells, kidney biopsy, etc.
4.
The patient has abdominal pain, nausea, and vomiting.
Is the digestive tract involved, malignant hypertension, or functional disease? The patient's complete color Doppler ultrasound, CT and other examinations found no evidence of lupus gastrointestinal involvement.
5.
The patient has blurred vision, is it fundus vasculitis, or is it highly manifested? The patient had a complete ophthalmological examination.
Exudation and hemorrhage were seen on both sides of the retina, papilledema, and grade IV hypertensive retinopathy.
6.
The patient has epistaxis and bleeding points in the lower extremities.
Is it considered to be caused by lupus vasculitis, abnormal blood coagulation, and thrombocytopenia? Or is there another reason? The patient's blood coagulation function is normal, the platelet is 71×109/L, which cannot be explained well by lupus vasculitis, abnormal blood coagulation function, and thrombocytopenia.
At this time, consider pointing the finger at TMA again.
One wave has not settled, and another wave has been summed up.
Assess this patient with SLE, there is blood, kidney and heart involvement, whether there are comorbidities? The patient's peripheral blood has broken red blood cells, occasionally broken red blood cells, slightly elevated lactate dehydrogenase (LDH), and lack of pathological evidence.
The patient's blood system, kidney, and heart involvement can be explained by SLE, so SLE is still the main treatment, and methylprednisolone 80mg/d is given, and symptomatic treatment for blood pressure, acid suppression and cough suppression is also given.
However, unfortunately, the patient has a new condition-hemoptysis, mainly for the following three reasons (see Figure 3).
Figure 3 The cause of hemoptysis The patient's perfect lung CT showed multiple ground glass shadows in both lungs, pulmonary edema and alveolar hemorrhage cannot be excluded (see Figure 4).
PCT is significantly higher than before.
Therefore, the three-pronged approach for treatment is to give a 1g shock of methylprednisolone, antibiotics to strengthen anti-infection and CRRT replacement therapy.
After hormone shock therapy, platelets increased temporarily, but the later effect was not ideal.
Figure 4 Lung CT showed that the patient's heart and digestive tract symptoms improved, but the blood and kidney performance still did not improve significantly, analyze the reasons again (see Figure 5).
Figure 5 The reticulocyte count in patients with anemia + thrombocytopenia is significantly increased, and bone marrow abnormalities are not considered for the time being.
Repeated peripheral blood smear detection, 0.
9% of the red blood cells in the peripheral blood were broken, which was in the range of suspicious TMA.
Plasma exchange was started for treatment, and the platelet recovery was satisfactory, but the anemia still did not improve (see Figure 6).
Figure 6 Hemoglobin and platelet changes after treatment The culprit of anemia has been found! Next, let's look for the culprit of anemia.
Re-examination of reticulocytes began to decline progressively, perfect bone marrow puncture.
Bone marrow smear: low bone marrow hyperplasia; high proportion of granule lines and no nucleated red blood cells.
Therefore, considering pure red aplastic anemia, what are the reasons for pure red aplastic anemia, mainly including the following aspects (see Figure 7).
Figure 7: Causes of pure red aplastic anemia.
Parvovirus B19 was detected.
It was found that the parvovirus B19-IgM was 123.
14U/ml, which was significantly higher than the normal value.
Therefore, symptomatic treatment was given.
Cyclophosphamide, blood transfusion and observation were suspended.
After 20 days, the reticulocyte count returned to normal, and the parvovirus B19-IgM 77U/ml was rechecked (see Figure 8).
Figure 8 After symptomatic treatment, the reticulocyte count returns to normal.
Does TMA cause kidney damage? Evaluate the curative effect again, so it seems that the blood system problem has been basically solved~Next, we will start to solve the kidney problem.
The patient had a complete kidney biopsy (see Figure 9), suggesting a typical TMA-like lesion.
After a kidney biopsy, the patient developed heavy bleeding.
He was treated with embolization to stop bleeding and blood transfusion support, and hemoglobin gradually recovered.
Figure 9 Renal biopsy results.
However, the patient also experienced 2 convulsions during hospitalization.
Factors such as infection, toxins, and hypertension were ruled out, and lupus encephalopathy or TMA was considered.
Perfect head magnetic nucleus showed abnormal white matter signals in the center of the right half oval and around the bilateral lateral ventricles.
The demyelinating lesions may be large, except for lupus encephalopathy (see Figure 10).
Figure 10 Follow-up treatment of head MRI (see Figure 11): Figure 11 Evaluation of follow-up treatment before discharge (see Figure 12): Figure 12 Evaluation and follow-up before discharge: The patient was discharged from the hospital for 8 months, the blood third system was normal, and the urine output was increased.
Regular hemodialysis 2 times/week. Conclusion: Combined with the analysis of this case, the causes of kidney and blood system involvement in lupus patients are TMA and pure red aplastic anemia.
Although the diagnosis of SLE is mostly similar, each patient may have its own story and misfortune behind it.
In the process of diagnosing and treating this difficult case, the clinician was shocked at every step and turned danger to a breeze.
Grasp the doubts, review the game in time, pull the cocoon, and find out the real culprit.
In the process of diagnosing and treating this difficult case, the clinician has been alarmed at every step and turned danger into a breeze! Solving a difficult case is like closing a case.
Only by constantly asking more questions and looking for answers in every detail can the truth be revealed.
At the 3rd Peking University Rheumatology and Immunology Hotspot Forum, the attending physician Zhang Haoze from the Department of Rheumatology and Immunology, Peking University First Hospital, brought us a wonderful case report.
This article summarizes the main content of this case and invites everyone to come to the conclusion of the case~ Case introduction patient A 17-year-old woman who developed skin rash and low-grade fever 10 years ago was diagnosed as "systemic lupus erythematosus and lupus nephritis" in an outside hospital, and was treated with prednisone combined with immunosuppressive agents.
Three years ago, the patient had a new rash.
Considering the recurrence of lupus, he continued to receive effective prednisone combined with immunosuppressive therapy.
The patient started to reduce the hormone dose 1 year ago, and the patient stopped taking the drug by himself 9 months ago.
Two months ago, the patient recurred fever and cheek rash, accompanied by cough, suffocation, inability to lie down, abdominal pain, nausea, vomiting, hematuria, proteinuria, and deterioration of renal function.
One month ago, the patient developed headache, blurred vision, nosebleeds, bleeding spots on both lower extremities, continued deterioration of renal function, decreased urine output, increased serum potassium, severe anemia, multiple serous effusions, and increased blood pressure As high as 180/130mmHg, prednisone 50mg/d is ineffective.
Two days ago, the patient's renal function deteriorated, blood transfusion and diuretic treatment were not effective, and severe anemia could not be corrected, so he came to the hospital for treatment (see Figure 1).
Figure 1 Brief introduction of the medical history Case characteristics: Young female, medical history for 10 years, chronic course, acute exacerbation, skin rash, nephritis and acute kidney injury, anemia, multiple serous effusion, laboratory tests have ANA, anti-Sm antibody, anti-nRNP antibody , Anti-dsDNA antibody is positive, complement is reduced, hormone therapy is effective, and relapse after drug withdrawal.
For every patient who considers systemic lupus erythematosus (SLE) for diagnosis, we often need to answer the following 3 questions (see Figure 2): 1.
Is it SLE? 2.
Which organs or systems are affected? 3.
Are there comorbidities? After answering these three questions, we can make the final diagnosis~ Figure 2 Is there any organ damage in the trilogy of SLE diagnosis? So, this patient is initially considering SLE, what organs or systems are involved? We will analyze in detail through the following points: 1.
The patient has cough and suffocation symptoms.
Is the heart involved? Or is it lung disease? Patients have symptoms such as edema, hypertension, and inability to lie on their backs, and further improve BNP and cardiac color Doppler ultrasound.
BNP: 3148pg/ml, color Doppler ultrasound showed enlarged left atrium, severe mitral regurgitation, moderate tricuspid regurgitation, pulmonary artery systolic pressure 69.
2mmHg, and a small amount of pericardial effusion.
Lung imaging and etiological examinations do not support lung infection and lupus lung involvement.
Therefore, consider the patient's cough and suffocation symptoms, the main reason is heart failure.
There may be multiple mechanisms involved in the manifestations of heart failure in patients.
Oligouria causes an increase in preload, hypertension causes an increase in afterload, anemia can also cause an increase in heart rate and hypoxia, and pulmonary hypertension causes an increase in right heart afterload.
2.
The patient also has symptoms of kidney damage.
Is it caused by prerenal, lupus nephritis or malignant hypertension? The patient has fever, heart failure and low intake, and is given symptomatic treatment and continued observation.
The patient has a history of discontinuation of the drug, deterioration of renal function, and appearance of the rash at the same time.
Further diagnosis requires a renal biopsy to confirm lupus nephritis.
The patient's blood pressure is 180/130mmHg, and it is necessary to control blood pressure and perfect kidney biopsy.
3.
The patient's blood system shows anemia and thrombocytopenia.
Consider lupus blood system involvement, bone marrow disease, or other reasons? Lupus causes blood system involvement is more common, the patient’s Coombs test result is negative, and the reticulocyte count is normal.
Patients with acute kidney injury, anemia, thrombocytopenia, fever, and thrombotic microangiopathies (TMA) need to be further improved to determine the detection of broken red blood cells, kidney biopsy, etc.
4.
The patient has abdominal pain, nausea, and vomiting.
Is the digestive tract involved, malignant hypertension, or functional disease? The patient's complete color Doppler ultrasound, CT and other examinations found no evidence of lupus gastrointestinal involvement.
5.
The patient has blurred vision, is it fundus vasculitis, or is it highly manifested? The patient had a complete ophthalmological examination.
Exudation and hemorrhage were seen on both sides of the retina, papilledema, and grade IV hypertensive retinopathy.
6.
The patient has epistaxis and bleeding points in the lower extremities.
Is it considered to be caused by lupus vasculitis, abnormal blood coagulation, and thrombocytopenia? Or is there another reason? The patient's blood coagulation function is normal, the platelet is 71×109/L, which cannot be explained well by lupus vasculitis, abnormal blood coagulation function, and thrombocytopenia.
At this time, consider pointing the finger at TMA again.
One wave has not settled, and another wave has been summed up.
Assess this patient with SLE, there is blood, kidney and heart involvement, whether there are comorbidities? The patient's peripheral blood has broken red blood cells, occasionally broken red blood cells, slightly elevated lactate dehydrogenase (LDH), and lack of pathological evidence.
The patient's blood system, kidney, and heart involvement can be explained by SLE, so SLE is still the main treatment, and methylprednisolone 80mg/d is given, and symptomatic treatment for blood pressure, acid suppression and cough suppression is also given.
However, unfortunately, the patient has a new condition-hemoptysis, mainly for the following three reasons (see Figure 3).
Figure 3 The cause of hemoptysis The patient's perfect lung CT showed multiple ground glass shadows in both lungs, pulmonary edema and alveolar hemorrhage cannot be excluded (see Figure 4).
PCT is significantly higher than before.
Therefore, the three-pronged approach for treatment is to give a 1g shock of methylprednisolone, antibiotics to strengthen anti-infection and CRRT replacement therapy.
After hormone shock therapy, platelets increased temporarily, but the later effect was not ideal.
Figure 4 Lung CT showed that the patient's heart and digestive tract symptoms improved, but the blood and kidney performance still did not improve significantly, analyze the reasons again (see Figure 5).
Figure 5 The reticulocyte count in patients with anemia + thrombocytopenia is significantly increased, and bone marrow abnormalities are not considered for the time being.
Repeated peripheral blood smear detection, 0.
9% of the red blood cells in the peripheral blood were broken, which was in the range of suspicious TMA.
Plasma exchange was started for treatment, and the platelet recovery was satisfactory, but the anemia still did not improve (see Figure 6).
Figure 6 Hemoglobin and platelet changes after treatment The culprit of anemia has been found! Next, let's look for the culprit of anemia.
Re-examination of reticulocytes began to decline progressively, perfect bone marrow puncture.
Bone marrow smear: low bone marrow hyperplasia; high proportion of granule lines and no nucleated red blood cells.
Therefore, considering pure red aplastic anemia, what are the reasons for pure red aplastic anemia, mainly including the following aspects (see Figure 7).
Figure 7: Causes of pure red aplastic anemia.
Parvovirus B19 was detected.
It was found that the parvovirus B19-IgM was 123.
14U/ml, which was significantly higher than the normal value.
Therefore, symptomatic treatment was given.
Cyclophosphamide, blood transfusion and observation were suspended.
After 20 days, the reticulocyte count returned to normal, and the parvovirus B19-IgM 77U/ml was rechecked (see Figure 8).
Figure 8 After symptomatic treatment, the reticulocyte count returns to normal.
Does TMA cause kidney damage? Evaluate the curative effect again, so it seems that the blood system problem has been basically solved~Next, we will start to solve the kidney problem.
The patient had a complete kidney biopsy (see Figure 9), suggesting a typical TMA-like lesion.
After a kidney biopsy, the patient developed heavy bleeding.
He was treated with embolization to stop bleeding and blood transfusion support, and hemoglobin gradually recovered.
Figure 9 Renal biopsy results.
However, the patient also experienced 2 convulsions during hospitalization.
Factors such as infection, toxins, and hypertension were ruled out, and lupus encephalopathy or TMA was considered.
Perfect head magnetic nucleus showed abnormal white matter signals in the center of the right half oval and around the bilateral lateral ventricles.
The demyelinating lesions may be large, except for lupus encephalopathy (see Figure 10).
Figure 10 Follow-up treatment of head MRI (see Figure 11): Figure 11 Evaluation of follow-up treatment before discharge (see Figure 12): Figure 12 Evaluation and follow-up before discharge: The patient was discharged from the hospital for 8 months, the blood third system was normal, and the urine output was increased.
Regular hemodialysis 2 times/week. Conclusion: Combined with the analysis of this case, the causes of kidney and blood system involvement in lupus patients are TMA and pure red aplastic anemia.
Although the diagnosis of SLE is mostly similar, each patient may have its own story and misfortune behind it.
In the process of diagnosing and treating this difficult case, the clinician was shocked at every step and turned danger to a breeze.
Grasp the doubts, review the game in time, pull the cocoon, and find out the real culprit.
