Five hematological manifestations of lupus, all in one article!

*Only for medical professionals to read for reference.
Spread the strongest rheumatism sound, create a new academic fashion, and watch the latest content of ACR 2021 in advance! The 2021 American Academy of Rheumatology (ACR) Annual Meeting (ACR 2021), as an academic feast gathering the latest research and the most influential experts in the field of rheumatism, has always attracted the attention of clinicians at home and abroad
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On the occasion of the annual meeting, the Medical Rheumatism Channel selected 19 hottest and latest reports of ACR 2021, which were explained by 19 young members from the Rheumatology Branch of the Chinese Medical Association, in order to "spread the strongest rheumatism, Create a new academic fashion"
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Director Cao Heng from the Department of Rheumatology and Immunology, the First Affiliated Hospital of Zhejiang University School of Medicine, will lead us to study the content of the special lecture "The Hematological Manifestations of Systemic Lupus Erythematosus (SLE)".
Professor Nancy Berliner from the affiliated Brigham and Women's Hospital and Professor Michael B.
Streiff from Johns Hopkins College reported together
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Topic 1 SLE-related anemia, neutropenia, and macrophage activation syndrome anemia are the most common hematological abnormalities in lupus, and more than half of the patients are affected.
Nancy from Brigham, Harvard Medical School, and Women’s Hospital In the first topic, Professor Berliner explained in detail the contents of SLE-related anemia, neutropenia, and macrophage activation syndrome (MAS)
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1.
SLE-related anemia SLE patients have various causes of anemia.
The common ones are iron deficiency, inflammation, autoimmune hemolysis, hypersplenism, renal insufficiency, and aplastic anemia
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How to determine which type of anemia a patient is in clinically? Professor Nancy Berliner provided two differentiated cases
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Case 1: Figure 1 Case 1 Table 1 Differentiation of two types of anemia The patient has a significant and comprehensive increase in inflammation indicators, and ferritin is higher than 100, so consider chronic inflammatory anemia
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Professor Nancy Berliner pointed out: Chronic inflammatory anemia may also lead to iron deficiency, so it may be confused with iron deficiency anemia and should be differentiated based on medical history
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Case 2: Figure 2 Case 2 This case is a typical autoimmune hemolytic anemia
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Autoimmune hemolytic anemia is a group of diseases in which the patient’s autoantibodies bind to the patient’s red blood cell antigens to cause extravascular hemolysis.
It is often accompanied by the stimulation of spleen macrophages (RES) or the activation of the complement system.
It is necessary to pay attention to anti-human globulin in the diagnosis.
The positive condition of the test (usually when the IgG type antibody is positive)
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2.
SLE-related neutropenia: Neutropenia is also very common in SLE.
Combined with Case 3, Professor Nancy Berliner analyzed the characteristics of SLE-related neutropenia
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Figure 3 Case 3 About 50% of SLE patients can have neutropenia.
Neutrophils can be regarded as a marker of SLE disease activity, but have little effect on the progression of SLE disease; infection-related complications and immunosuppressive agents The use of neutrophils has nothing to do with the neutrophil count
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Although the reduction of neutrophil antibodies is common, Professor Nancy Berliner believes that the significance of neutrophil antibody detection is not clear and there may be a trap of false positives.
Therefore, it is not necessary to use neutrophil antibody test results for diagnosis in clinical practice
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Table 2 Detectable neutrophil antibodies 3.
SLE-related MAS: In conjunction with case 4, Professor Nancy Berliner interpreted the characteristics of SLE-related MAS
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Director Cao Heng pointed out: MAS is not unique to SLE patients in the Department of Rheumatology, and the interpretation of SLE-related MAS can also help clinicians understand MAS secondary to other rheumatic diseases
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Figure 4 Case 4 The occurrence of MAS may usually be related to infection (EB virus, cytomegalovirus).
Most patients will be combined with the activities of primary rheumatic diseases before the occurrence of MAS.
MAS may also be combined with encephalopathy and natural killer cells (NK).
Decrease in function, decrease in the number of NK cells (with or without), and MAS is not typical in adult patients
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MAS is considered to be a subgroup of hemophagocytosis (HLH).
Juvenile idiopathic arthritis (sJIA) is most likely to be associated with MAS (10%), and MAS secondary to immune diseases is more effective for immunosuppressive therapy than other types of HLH.
Sensitive, and etoposide-containing regimens are usually not recommended
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Professor Nancy Berliner believes that the treatment of MAS secondary to rheumatic immune diseases should focus on the treatment of primary autoimmune diseases.
The main treatment options include: high-dose steroids, cyclosporine, intravenous gamma globulin, and interleukin receptor antagonism Agent; Etoposide is rarely used
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In response to this diagnosis and treatment recommendation, Director Cao Heng added a point of view: There are also many cases of using etoposide to treat MAS secondary to rheumatism.
Clinicians can make individualized judgments based on the patient's condition and current evidence-based evidence
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Figure 5 Topic 1 Summary Topic 2 SLE-related thrombocytopenia and coagulation dysfunction Professor Michael B.
Streiff from Johns Hopkins College systematically explained SLE-related thrombocytopenia and coagulation dysfunction
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1.
Classification of platelet diseases: Professor Michael B.
Streiff believes that platelet diseases can be divided into quantitative changes (decreased production, increased destruction) and quality changes (receptor defects, dysfunction)
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The former mainly includes aplastic anemia, myeloid dysplasia, and immune thrombocytopenia.
Rare diseases such as hepatolenticular degeneration and Gaucher disease may also lead to a decrease in the number of platelets; the latter mainly include platelet insufficiency, giant platelet syndrome, and platelet storage.
Chi disease and so on
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2.
SLE-related platelet diseases: Thrombocytopenia is more common in SLE.
10%-25% of SLE patients may have platelets <100000/µL.
Thrombocytopenia is associated with bleeding, hemolytic diseases, nephropathy, and antiphospholipid syndrome (APS).
) Antibody correlation is an independent risk factor for patient death
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Its pathogenesis includes the formation of specific antibodies and platelet activation induced by APS antibodies
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Mainly related to immune thrombocytopenia, APS, drug-induced autoantibody production, sepsis, diffuse intravascular coagulation (DIC), MAS, bone marrow suppression drug use, and vitamin B12 deficiency
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Professor Michael B.
Streiff said that when receiving patients with SLE and thrombocytopenia, it can be divided into two steps: Figure 6 Diagnosis and treatment steps In terms of treatment, Professor Michael B.
Streiff recommends individualized treatment based on symptoms and platelet counts.
Treatment goals Platelet 500,000-100,000/μL, symptom relief; commonly used treatments include glucocorticoid, dexamethasone, intravenous gamma globulin (400mg/kg-1g/kg, total amount not exceeding 2g/kg), ritual infliximab, thrombopoietin
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3.
SLE-related APS: The incidence of APS is low, but severe APS may induce catastrophic consequences-miscarriage, preeclampsia, arteriovenous thrombosis, renal dysfunction, thrombocytopenia.
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Professor Michael B.
Streiff emphasized that in During the diagnosis of APS, anticoagulant drugs should be stopped as much as possible to avoid the interference of false positive results
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3 When APS occurs, how to choose anticoagulant drugs? Professor Michael B.
Streiff believes that low-molecular-weight heparin (LMWH) and vitamin K antagonist (VKA) are still the first choices when the first venous/arterial thrombosis occurs; at the same time, patients with venous thrombosis need to pay attention to monitoring INR2-3 and long-term treatment For patients with arterial thrombosis, low-dose aspirin may be considered
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The recommendations for the treatment of recurrent arterial and venous thrombosis are as follows: Recurrent venous thrombosis: pay attention to the evaluation of INR and other risk factors (surgery, vascular catheter, cancer, anatomical risk factors) before embolization; if INR<2, a higher INR target should be considered (2.
5-3.
5); If INR2-3, consider INR3-4 or adjust to the CFX range (20%-35%); if you have used LMWH, consider 25% high-dose LMWH or Fondaparinux
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Recurrent arterial thrombosis: same assessment as above; consider adding aspirin or clopidogrel (P2Y12 antagonist)
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Figure 7 Topic 2 Summary Expert profile Cao Heng, deputy chief physician of the Department of Rheumatology and Immunology, the First Hospital of Zhejiang University, doctor of medicine, master supervisor
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Vice President of Rheumatology and Immunology Branch of Zhejiang Medical Doctor Association, Standing Committee of Rheumatology Branch of Zhejiang Medical Association, Youth Member of Rheumatology Branch of Chinese Medical Association, Youth Member of Rheumatology and Immunology Branch of Chinese Medical Doctor Association, Rheumatology and Immunology of Chinese Medical Doctor Association Member of the Imaging Group of the Physician Branch, Member of the Chinese Scleroderma and Myositis Research Committee, Youth Member of the Internal Medicine Branch of the Zhejiang Medical Association, and Standing Member of the Rheumatology Branch of the Association of Mathematical Medicine
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Visiting scholar at Grenoble University Hospital, France
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Presided over 1 National Natural Youth Science Fund, 3 provincial and departmental scientific research projects, and published 10 SCI papers as the first author or corresponding author
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