OBJECTIVE: To investigate the clinical and immunological features of cardiac involvement in patients with anti-synthetase syndrome (ASS). METHODS: In the study, 96 patients diagnosed with ASS hospitalized in the Department of Rheumatology and Immunology, Peking University People's Hospital from April 2003 to November 2020 were included. The patients were divided into two groups according to whether they were accompanied with cardiac involvement. Demographic features, clinical characteristics (Gottron's sign/papules, muscle damage, etc.), comorbidities, laboratory indices (creatine kinase, inflammatory indicators, immunoglobulin, complement, lymphocyte subset, autoantibodies, etc.) were collected and the differences between the two groups were analyzed statistically. RESULTS: The prevalence of cardiac involvement in the patients with ASS was 25.0% (24/96). The ASS patients complicated with cardiac involvement presented with elevated cardiac troponin I (cTnI, 75.0%, 18/24), pericardial effusion (33.3%, 8/24), reduction of left ventricular function (33.3%, 8/24) and valves regurgitation (33.3%, 8/24). The age of onset of the patients with cardiac involvement was older than that of the patients without cardiac involvement [(54.58±10.58) years vs. (48.47±13.22) years, P=0.043). Arthritis was observed less frequently in the patients with cardiac involvement than those without cardiac involvement (37.5% vs. 61.1%, P=0.044). In addition, rapidly progressive interstitial lung disease (54.2% vs. 30.6%, P=0.037) was observed more frequently in the patients with cardiac involvement than those without cardiac involvement. As compared with the ASS patients without cardiac involvement, C-reactive protein (CRP) [(13.55 (8.96, 38.35) mg/L vs. 4.60 (1.37, 17.40) mg/L, P=0.001], and lactate dehydrogenase (LDH) [408.0 (255.0, 587.0) U/L vs. 259.5 (189.8, 393.8) U/L, P=0.007] were significantly higher in the patients with cardiac involvement. Anti-Ro-52 antibody was detected more commonly in the ASS patients with cardiac involvement compared with the patients without cardiac involvement (91.7% vs. 69.4%, P=0.029). No significant differences were found in the comorbidities, alanine transaminase (ALT), aspartate transaminase (AST), creatine kinase (CK), erythrocyte sedimentation rate (ESR), ferritin (Fer), immunoglobulin G (IgG), complement 3 (C3), complement 4 (C4), lymphocyte subset between the two groups. CONCLUSION Cardiac involvement is common in ASS, mainly manifested as myocardial damage. It is necessary to be aware of cardiac complications in patients with elevated CRP, elevated LDH and positive anti-Ro-52 antibody.
Adult ; Aged ; Antibodies, Antinuclear ; Autoantibodies ; Blood Sedimentation ; C-Reactive Protein ; Heart Diseases/complications* ; Humans ; Immunoglobulin G ; L-Lactate Dehydrogenase ; Middle Aged ; Myositis/diagnosis*

OBJECTIVE: To understand the characteristics of patients with mimicking specificity autoantibodies through the analysis of the causes of autoantibodies, specificity of antibodies, strategy of blood transfusion, effect of transfusion and distribution of antibodies in China and abroad. METHODS: A total of 23 patients who applied for blood in our hospital from January 2017 to June 2019 were identified as mimicking specificity autoantibodies by antibody identification or absorption-elution test. The causes of mimicking specificity autoantibodies, antibody specificity, blood transfusion strategy and blood transfusion effect were analyzed. The relevant articles on antibodies published in China and abroad were summarized and sorted out, and the distribution of antibodies was analyzed. RESULTS: All the 23 patients with mimicking specificity autoantibodies were Rh blood group system antibodies, of which mimicking anti-Ce autoantibodies were the most common (34.8%), followed by mimicking anti-e autoantibodies (26.1%), mimicking anti-D autoantibodies (21.7%), mimicking anti-C autoantibodies (8.7%) and mimicking anti-E autoantibodies (8.7%). Except for 2 cases with suspected history of blood transfusion, the other 21 cases had a history of blood transfusion / pregnancy. The most common cause of mimicking autoantibodies was drug, followed by infection and autoimmune diseases. The hemoglobin (Hb) of pretransfusion in the blood transfusion group was (48.4±23.9) g/L, which was significantly lower than (86.0±38.9) g/L in the non-transfusion group (P<0.01). Except for 2 cases who could not evaluate the effect of blood transfusion, the effective rate of transfusion was 100%. According to the retrospective statistics of 32 related articles published in China and abroad, the most type of mimicking antibodies were in Rh blood group system, accounting for 79.28%, among which anti-E was the main part of all mimicking autoantibodies, accounting for 21.95%. The following ones were in Kidd system MNSs system, and Kell system. CONCLUSION Combined with the clinical symptoms and the degree of difficulty of blood matching, the best strategy of blood transfusion should be selected to ensure the safety of blood transfusion.
Autoantibodies ; Blood Group Antigens ; Blood Transfusion ; Female ; Humans ; Isoantibodies ; Pregnancy ; Retrospective Studies

OBJECTIVE: To explore the clinical application of screening cell combination method in the prediction of red blood cell alloantibody, so as to provide basis for clinical diagnosis. METHODS: From October 2018 to April 2020, 9 680 samples were screened with automatic blood group instrument, 79 patients with positive alloantibodies were identified by 4 sets of screening cells from different manufacturers (referred to as combined method). At the same time, cell panel Panocell-16 was used for comparative analysis. Meanwhile, the combined method was also used to identify the antibodies of 20 samples from National Center for Clinical Laboratories external quality assessment (EQA) in China and 12 samples from WHO EQA. RESULTS: The 79 alloantibodies included anti-Mia antibody (7 cases), anti-M antibody (13 cases), anti-Le CONCLUSION The combined method can identify the alloantibodies of red blood cells in Chinese population. The screening cells can be used for screening of irregular antibodies without wasting reagents at the same time.
Autoantibodies ; Blood Group Antigens ; China ; Erythrocytes ; Humans ; Isoantibodies

OBJECTIVE: To analyze the causes of positive irregular antibody screening test and incompatibility of cross matching in one patient with autoimmune hemolytic anemia complicated with neonatal hemolytic disease, and to accurately identify the type of antibodies in patients, and to select a reasonable strategy for blood transfusion. METHODS: One children was enrolled, blood group positive and reverse typing, Rh typing, direct anti-human globulin test, free test, dispersal test and cross matching test were carried out by test tube method and microcolumn gel card; irregular antibodies were identified by the reaction of DTT treatment and untreated panel cells with patients' plasma. RESULTS: The blood group of the patient was RhD positive B and irregular antibody screening positive, while the blood group of the mother was RhD positive O and irregular anti-screening negative, the result showed that the anti-LW detected in the plasma of the patient was autoantibody and ABO neonatal hemolytic disease (ABO-HDN) was present. Both O type RhD positive washing RBCs and B type RhD negative RBCs were transfused effectively. CONCLUSION Irregular antibodies in patients are anti-LW antibodies, and transfusion of homotype RhD negative suspended erythrocytes after the exclusion of ABO-HDN shows a better effect.
Anemia, Hemolytic, Autoimmune ; Autoantibodies ; Blood Group Incompatibility ; Blood Transfusion ; Erythroblastosis, Fetal ; Humans

OBJECTIVE: To investigate the clinical characteristics and high risk factors of Rheumatoid arthritis (RA) complicated with tuberculosis infection. METHODS: Patients with rheumatoid arthritis diagnosed in the hospital of Sichuan Provincial People's Hospital from January 2007 to January 2017 was retrospectively collected, who were enrolled in the study group. A control group was randomly selected from the RA patients hospitalized in the same period without co-infection at a ratio of 1 :2. The general data, clinical data, laboratory test data, treatment plan, etc. of the two groups were collected in detail for single factor statistical analysis. Then multivariate Logistic regression was used to analyze the independent risk factors of RA complicated with tuberculosis infection with statistical significance in univariate analysis. RESULTS: The clinical manifestations of fever (83.3%) were most common, followed by cough (69%) and body mass loss (45.2%). In the infected group, pulmonary tuberculosis accounted for 73.3%. In the infected group the chest CT showed two or more cases, accounting for 59%. There were 9 cases (33.3%) occurring in the typical tuberculosis occurrence site. Compared with the control group, the erythrocyte sedimentation rate (ESR), C-reaction protein (CRP) levels, and the daily average dose of glucocorticoid in 1 year in the infected group were higher than those in the control group. And those differences were statistically significant(P < 0.05). There were no significant differences in gender, age, disease duration, disease activity score, white blood cell (WBC), platelet (PLT), hemoglobin (Hb), immunoglobulin G (IgG), complement (C), Anti cyclic citrullinated peptide antibody (anti-CCP), CD4⁺T cell count, and immunosuppressant use (P > 0.05). Multivariate Logistic regression analysis showed that CRP levels(OR=1.016, 95%CI:1.002-1.031) and the daily average dose of glucocorticoid in 1 year(OR=1.229, 95%CI:1.066-1.418)were the independent risk factors of RA complica-ted with tuberculosis infection. CONCLUSION RA patients with tuberculosis infection are mainly phthisis. The clinical manifestations of RA combined with tuberculosis infection are lack of specificity, and the chest imaging features of pulmonary tuberculosis are diverse, which are easy to be misdiagnosed. CRP levels and the daily average dose level of glucocorticoid in 1 year were risk factors for RA and tuberculosis infection.
Arthritis, Rheumatoid/complications* ; Autoantibodies ; Blood Sedimentation ; Humans ; Peptides, Cyclic ; Retrospective Studies ; Rheumatoid Factor ; Tuberculosis/epidemiology*

To investigate the clinical manifestations and laboratory characteristics of 6 cases of pemphigoid nodularis (PN). The clinical and laboratory data of 6 patients with PN admitted to the Department of Dermatology,Peking Union Medical College Hospital from January 2016 to August 2019 were retrospectively analyzed. PN mainly occurred in middle-aged and elderly people,with an average age of (58±16) years. Eosinophils were elevated in 4 patients. Immunoglobulin E (IgE) level was (530±672) kU/L in five patients. Direct immunofluorescence showed IgG and/or C3 deposition on basal membrane zone. Indirect immunofluorescence showed positive IgG anti-basement membrane zone,with a titer of 1:40-1:320. Enzyme-linked immunosorbent assay showed the anti-BP180 antibodies were positive [24-85 U/ml,average(43±26) U/ml] in 5 patients. None of the patients had neurological disorders. One patient was lost to follow-up. The disease recurred in 3 of 5 patients during the follow-up,and two patients still received maintenance corticosteroids. PN mainly occurs in middle-aged and elderly individuals. It is featured by elevated eosinophils and total IgE and relatively low anti-BP180 antibody titers. Recurrence is common but PN is less likely to be associated with neurological diseases.
Adult ; Aged ; Autoantibodies ; blood ; Enzyme-Linked Immunosorbent Assay ; Eosinophils ; Humans ; Immunoglobulin E ; blood ; Middle Aged ; Pemphigoid, Bullous ; diagnosis ; pathology ; Retrospective Studies

OBJECTIVE: To evaluate the efficacy and safety of different types of red blood cell (RBC) transfusion and hormone therapy in patients with autoimmune hemolytic anemia (AIHA). METHODS: The clinical data and serological characteristics of 40 patients with AIHA treated in our hospital from 2014 to 2018 were collected and analyzed retrospectively. The efficacy and safety of different type of RBC transfusion and hormone therapy were evaluated according to the principle of minimally incompatible RBC transfusion after cross-matching. RESULTS: Among 40 patients with AIHA, the female cases were more than the male cases, the cases of secondary AIHA was more than cases of primary AIHA, and the warm autoantibodies were in the majority. 11 cases of AIHA underwent 26 times minimally incompatible red blood cell transfusions. The total effective rate was 46.2%, the partial efficiency was 23.1%, and total inefficiency was 30.8%. Among them, the same type of non-washing red blood cell group showed efficiency of 42.1%, partial effective rate of 21.1%, and inefficiency of 36.8%; the same type of washed red blood cell group showed efficiency of 57.1%, partial effective rate of 28.6%, and inefficiency of 14.3%. the infusion effects was not significanly different between the two groups, and no hemolytic transfusion reaction occurred. In the hormone-treated group, the complete remission rate was 15.2%, the partial remission rate was 63.6%, and the ineffective rate was 21.2%. Among them, the side effects appeared in 2 patients after using hormones. CONCLUSION When AIHA patients need blood transfusion, use the same type of non-washed red blood cells or homologous washed cells is relatively safe, and the difference in efficacy is not significant. The partial remission of patients received hormone therapy is much higher than that of red blood cell transfusion, but the side effects easily happen.
Anemia, Hemolytic, Autoimmune ; Autoantibodies ; Blood Transfusion ; Erythrocyte Transfusion ; Erythrocytes ; Female ; Humans ; Male ; Retrospective Studies

OBJECTIVE: To explore the clinical significance of platelet membrane glycoprotein (GPIIb/IIIa) detection by flow cytometry (FCM) combined with enzyme-linked immunosorbent assay (ELISA) in diagnosis and treatment of immune thrombocytopenia(ITP). METHODS: Fifty-two patients with immunological thrombocytopenia admitted to the Department of Hematology in our hospital during October 2014-October 2018 were enrolled in ITP group. Thirty healthy people from the physical examination center were enrolled in control group. The positive expression rate of platelet membrane glycoprotein was measured by FCM, and the peripheral platelet count was detected by automatic analyzer, and the plasma membrane glycoprotein level was measured by ELISA. The difference between the two groups was compared. The platelet membrane glycoprotein levels in ITP patients before and after treatment were compared. RESULTS: The positive expression rate of GPIIb/IIIa (i,e CD41/CD61) and plasma GPIIb/IIIa levels in ITP patients were significantly lower than those in the healthy controls. The increased expression of platelet GPIIb/IIIa was associated with the response to therapy. The positive expression rate and level of GPIIb/IIIa postively correlated with its platelet count. The sensitivity of platelet GPIIb/IIIa combined with platelet count for diagnosis of ITP was 90.38%, the specificity was 93.33%, the positive likelihood ratio was 13.57, and the positive predictive value was 95.92%. CONCLUSION The platelet membrane glycoprotein detection as a preliminary screening method used for diagnosis of immune thrombocytopenia is simple, convenient, sensitive and rapid, thus may be considered as a new method for clinical diagnosis.
Autoantibodies ; Blood Platelets ; Humans ; Platelet Glycoprotein GPIIb-IIIa Complex ; Purpura, Thrombocytopenic, Idiopathic

Bullous pemphigoid(BP)is an autoimmune blister disease caused by antibodies to the basement membrane zone.It usually exhibits tense bullae in skin and mucous membrane.BP patients can suffer from thrombosis due to hypercoagulation and/or hemorrhage due to the presence of anti-coagulation factors,which may be explained by the eosinophils,antibodies to the coagulation factors,and fibrinolytic system hyperactivity or inhibition.
Autoantibodies ; Blister ; Blood Coagulation Disorders ; complications ; Blood Coagulation Factors ; Eosinophils ; Hemorrhage ; etiology ; Humans ; Mucous Membrane ; pathology ; Pemphigoid, Bullous ; complications ; Skin ; pathology ; Thrombosis ; etiology

Objective To explore the clinical characteristics of autoimmune disease with dual seropositive antibodies of leucine-rich glioma inactivated 1(LGI1)and contactin-associated protein 2(Caspr2).Methods The clinical data of seven patients with dual seropositive LGI1 and Caspr2 antibodies who were admitted to the Neurology Department of Peking Union Medical College Hospital from July 2014 to December 2017 were retrospectively analyzed.Results Central,peripheral and autonomic nervous systems were all involved in the seven cases;100%(7/7)presented with insomnia,myokymia,neuropahic pain and hyperhydrosis;71%(5/7)showed memory decline or psychiatric and behavioral symptoms;57%(4/7)had urinary hesitation or constipation;and 43%(3/7)had seizure.Electromyography showed 100%(6/6) of the patients had prolonged afterdischarges following normal M waves and/or abnormal spontaneous firing.Electroencephalography revealed slow waves or basic rhythm slowing in 71%(5/7)of patients.Electrocardiography showed sinus tachycardia,axis deviation,and prolonged QT intervals in 71%(5/7)of patients.One patient died from arrhythmia before immunotherapy.One died from pulmonary infection after immunotherapy.Improvement with immunotherapy was documented in the other five cases.No relapse was noted during the 1-2-year follow-up.Conclusions Autoimmune disease with dual seropositive antibodies of LGI1 and Caspr2 can diffusely affect the central,peripheral,and autonomic nervous systems.The possibility of this disease should be considered in patients with acute and subacute onset of neuropsychiatric symptoms,especially in patients with accompanying insomnia,myokymia,and hyperhydrosis.
Autoantibodies ; blood ; Autoimmune Diseases ; immunology ; Humans ; Membrane Proteins ; immunology ; Nerve Tissue Proteins ; immunology ; Proteins ; immunology ; Retrospective Studies