BACKGROUND Autoimmune hepatitis(AIH)and primary biliary cholangitis(PBC)are two common clinical autoimmune liver diseases,and some patients have both diseases;this feature is called AIH-PBC overlap syndrome.Autoimmune...BACKGROUND Autoimmune hepatitis(AIH)and primary biliary cholangitis(PBC)are two common clinical autoimmune liver diseases,and some patients have both diseases;this feature is called AIH-PBC overlap syndrome.Autoimmune thyroid disease(AITD)is the most frequently overlapping extrahepatic autoimmune disease.Immunoglobulin(IgG)4-related disease is an autoimmune disease recognized in recent years,characterized by elevated serum IgG4 levels and infiltration of IgG4-positive plasma cells in tissues.CASE SUMMARY A 68-year-old female patient was admitted with a history of right upper quadrant pain,anorexia,and jaundice on physical examination.Laboratory examination revealed elevated liver enzymes,multiple positive autoantibodies associated with liver and thyroid disease,and imaging and biopsy suggestive of pancreatitis,hepatitis,and PBC.A diagnosis was made of a rare and complex overlap syndrome of AIH,PBC,AITD,and IgG4-related disease.Laboratory features improved on treatment with ursodeoxycholic acid,methylprednisolone,and azathioprine.CONCLUSION This case highlights the importance of screening patients with autoimmune diseases for related conditions.展开更多
BACKGROUND Survival in patients with autoimmune liver disease overlap syndromes(AILDOS)compared to those with single autoimmune liver disease is unclear.AIM To investigate the survival of patients with AILDOS and asse...BACKGROUND Survival in patients with autoimmune liver disease overlap syndromes(AILDOS)compared to those with single autoimmune liver disease is unclear.AIM To investigate the survival of patients with AILDOS and assess the accuracy of non-invasive serum models for predicting liver-related death.METHODS Patients with AILDOS were defined as either autoimmune hepatitis and primary biliary cholangitis overlap(AIH-PBC)or autoimmune hepatitis and primary sclerosing cholangitis overlap(AIH-PSC)and were identified from three tertiary centres for this cohort study.Liver-related death or transplantation(liver-related mortality)was determined using a population-based data linkage system.Prognostic scores for liver-related death were compared for accuracy[including liver outcome score(LOS),Hepascore,Mayo Score,model for end-stage liver disease(MELD)score and MELD incorporated with serum sodium(MELD-Na)score].RESULTS Twenty-two AILDOS patients were followed for a median of 3.1 years(range,0.35-7.7).Fourteen were female,the median age was 46.7 years(range,17.8 to 82.1)and median Hepascore was 1(range,0.07-1).At five years post enrolment,57%of patients remained free from liver-related mortality(74%AIH-PBC,27%AIH-PSC).There was no significant difference in survival between AIH-PBC and AIH-PSC.LOS was a significant predictor of liver-related mortality(P<0.05)in patients with AIH-PBC(n=14)but not AIH-PSC(n=8).A LOS cut-point of 6 discriminated liver-related mortality in AIH-PBC patients(P=0.012,log-rank test,100%sensitivity,77.8%specificity)(Harrell's C-statistic 0.867).The MELD score,MELD-Na score and Mayo Score were not predictive of liver-related mortality in any group.CONCLUSION Survival in the rare,AILDOS is unclear.The current study supports the LOS as a predictor of liver-related mortality in AIH-PBC patients.Further trials investigating predictors of survival in AILDOS are required.展开更多
Irritable bowel syndrome (IBS) is a common chronic gastrointestinal disease with a significant impact on patients’ quality of life and a high socioeconomic burden. And the understanding of IBS has changed since the r...Irritable bowel syndrome (IBS) is a common chronic gastrointestinal disease with a significant impact on patients’ quality of life and a high socioeconomic burden. And the understanding of IBS has changed since the release of the Rome Ⅳ diagnosis in 2016. With the upcoming Rome Ⅴ revision, it is necessary to review the results of IBS research in recent years. In this review of IBS, we can highlight future concerns by reviewing the results of IBS research on epidemiology, overlap disorders, pathophysiology, and treatment over the past decade and summarizing the latest research.展开更多
AIM To investigate the performance of transient elastography(TE) for diagnosis of fibrosis in patients with autoimmune hepatitis-primary biliary cholangitis(AIHPBC) overlap syndrome.METHODS A total of 70 patients with...AIM To investigate the performance of transient elastography(TE) for diagnosis of fibrosis in patients with autoimmune hepatitis-primary biliary cholangitis(AIHPBC) overlap syndrome.METHODS A total of 70 patients with biopsy-proven AIH-PBC overlap syndrome were included. Spearman correlation test was used to analyze the correlation of liver stiffness measurement(LSM) and fibrosis stage. Independent samples Student's t-test or one-way analysis of variance was used to compare quantitative variables. Receiver operating characteristics(ROC) curve was used to calculate the optimal cut-off values of LSM for predicting individual fibrosis stages. A comparison on the diagnostic accuracy for severe fibrosis was made between LSM and other serological scores.RESULTS Patients with AIH-PBC overlap syndrome had higher median LSM than healthy controls(11.3 ± 6.4 k Pa vs 4.3 ± 1.4 k Pa, P < 0.01). LSM was significantly correlated with fibrosis stage(r = 0.756, P < 0.01). LSM values increased gradually with an increased fibrosis stage. The areas under the ROC curves of LSM for stages F ≥ 2, F ≥ 3, and F4 were 0.837(95%CI: 0.729-0.914), 0.910(0.817-0.965), and 0.966(0.893-0.995), respectively. The optimal cut-off values of LSM for fibrosis stages F ≥ 2, F ≥ 3, and F4 were 6.55, 10.50, and 14.45 k Pa, respectively. LSM was significantly superior to fibrosis-4, glutaglumyl-transferase/platelet ratio, and aspartate aminotransferase-to-platelet ratio index scores in detecting severe fibrosis(F ≥ 3)(0.910 vs 0.715, P < 0.01; 0.910 vs 0.649, P < 0.01; 0.910 vs 0.616, P < 0.01, respectively).CONCLUSION TE can accurately detect hepatic fibrosis as a noninvasive method in patients with AIH-PBC overlap syndrome.展开更多
We report a 12 years old female patient with an overlap syndrome involving autoimmune hepatitis (AIH) and systemic lupus erythematosus (SLE). The patient presented with jaundice, hepatosplenomegaly, malaise, polya...We report a 12 years old female patient with an overlap syndrome involving autoimmune hepatitis (AIH) and systemic lupus erythematosus (SLE). The patient presented with jaundice, hepatosplenomegaly, malaise, polyarthralgia, arthritis and butterfly rash on the face. Laboratory tests revealed severe liver dysfunction, Coombs positive hemolytic anemia and a positive ANA/ anti-dsDNA test. Renal biopsy showed class IIA kidney disease, while liver biopsy showed chronic hepatitis with severe inflammatory activity. The patient satisfied the international criteria for both SLE and AIH. Clinical symptoms and laboratory findings of SLE improved with high dose treatment with corticosteroids and azathioprine, however, remission of the liver disease could not be achieved. Repeat biopsy of the liver after three years of therapy revealed ongoing chronic hepatitis with high level of inflammatory activity. The present case indicates that children with liver dysfunction and SLE should be investigated for AIH. There is much diagnostic and therapeutic dilemma in patients with AIH-SLE overlap syndrome.展开更多
BACKGROUND Systemic lupus erythematosus(SLE)and antineutrophil cytoplasmic antibodyassociated vasculitis(AAV)are classically thought to cause renal impairment and small vessel vasculitis with different pathophysiologi...BACKGROUND Systemic lupus erythematosus(SLE)and antineutrophil cytoplasmic antibodyassociated vasculitis(AAV)are classically thought to cause renal impairment and small vessel vasculitis with different pathophysiologies.Their overlap constitutes a rare rheumatologic disease.To date,only dozens of such cases with biopsyproven glomerulonephritis have been reported worldwide typically in women of childbearing age.Here,we present a unique clinical case due to its rarity and individualized treatment of a Chinese man in his eighth decade of life.CASE SUMMARY A 77-year-old man was admitted to several hospitals for shortness of breath and received nonspecific treatments over the past 3 years.As his symptoms were not completely relieved,he visited our hospital for further treatment.Laboratory examinations revealed kidney dysfunction,severe anaemia,hypocomplementemia,glomerular proteinuria,and microscopic haematuria.Antinuclear antibodies,as well as anti-dsDNA antibodies,were positive.Computed tomography of the chest showed right pleural effusion.Renal biopsy was performed,and histology suggested crescentic glomerulonephritis,pauci-immune type.After treatment with plasmapheresis,glucocorticoid,and cyclophosphamide,the disease was in remission,and the patient remained in a stable condition for over 3 years post-hospital discharge.CONCLUSION Due to its complexity and rarity,SLE and AAV overlap syndrome is easily misdiagnosed.An accurate diagnosis and treatment at the earliest stage may significantly improve the condition and reduce irreversible organ injury.展开更多
Objective:To explore the feasibility of tiotropium bromide therapy and its effect on the lung function of patients with asthma-COPD overlap syndrome(ACOS).Method:The 58 subjects selected in this study were all ACOS pa...Objective:To explore the feasibility of tiotropium bromide therapy and its effect on the lung function of patients with asthma-COPD overlap syndrome(ACOS).Method:The 58 subjects selected in this study were all ACOS patients admitted to our hospital(Hohhot First Hospital)from October 2020 to October 2022.They were grouped according to the random number table method and divided into a control group(29 cases)and an observation group(29 cases).The control group received routine treatment plus salmeterol-fluticasone powder inhalation treatment,and the observation group received tiotropium bromide treatment.The relevant indicators of the two groups were compared.Results:The total clinical effective rate of the observation group was significantly higher than that of the control group.Besides,the forced vital capacity(FVC),forced expiratory volume in 1 second(FEV1)levels,and ACT scores of the two groups increased after treatment,with the observation group having better results than the control group.The residual volume-total lung capacity ratio(RV/TLC),acute exacerbation frequency,and CAT scores all decreased,with the observation group showing smaller values than the control group.The difference between the results of both groups were significant(P<0.05)Conclusion:Tiotropium bromide has a significant clinical effect in the treatment of ACOS patients and can effectively improve the lung function of patients.展开更多
The myasthenia gravis is an autoimmune disease which can be associated frequently with a thymoma and also with autoimmune pathologies. We report the case of a 64-year-old man affected an overlap syndrome corresponding...The myasthenia gravis is an autoimmune disease which can be associated frequently with a thymoma and also with autoimmune pathologies. We report the case of a 64-year-old man affected an overlap syndrome corresponding to the association of autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC). He also presented concomitant myasthenia gravis and thymoma. The physical examination notes only a disturbed voice. The liver tests showed: alkaline phosphatase 600 U/l, alanine aminotransferase 1280 U/l, and aspartate aminotransferase 985 U/l. Viral serologies of hepatitis were all negative, as well as antinuclear, antimitochondrial and antismooth muscle antibodies. The diagnosis of an overlap syndrome was posed according to the biological and hepatic histhological results. An associated myasthenia was confirmed by the results of the EMG and the positivity of the anti-acetylcholine receptor antibody. A chest CT revealed a thymoma. The treatment consisted of ursodeoxycholic acid, prednisone, azathioprine and pyridostigmine. And the patient improved his voice and the liver function. Thymectomy was practiced without incidents.展开更多
Although asthma and chronic obstructive pulmonary disease(COPD) are distinct airway diseases characterized by chronic inflammation, in some cases distinguishing between them is puzzling. For example, chronic smoking l...Although asthma and chronic obstructive pulmonary disease(COPD) are distinct airway diseases characterized by chronic inflammation, in some cases distinguishing between them is puzzling. For example, chronic smoking leads asthmatic inflammation to a differentiated pattern resembling the COPD inflammation, and in some cases to fixed obstruction as in COPD, and on the other hand, few COPD patients may present with airway reversibility. ACOS is the condition sharing features encountered both in asthma and COPD. Asthma-COPD overlap syndrome(ACOS) represents a diagnostic challenge in the clinical practice, since there is lack of specific indicators to distinguish it from asthma or COPD, and moreover, genetic risk factors, underlying pathology and molecular pathways, clinical characteristics, therapeutic interventions, response to treatment and prognosis are poorly described. The management of ACOS is recommended to be individualized and should target on the maximum effectiveness with the least side effects. Combination therapy with ICS/LABA or LAMA, or newly developed specific anti-eosinophil therapies and treatments specifically targeting neutrophils might be of relevance in the management of ACOS, but studies are needed in order to assess the response and prognosis. Based on the current knowledge about ACOS thus far, it would be recommended that we approached chronic obstructive airway disease rather by describing than by classifying the disease; this would allow us to have a picture that better describes the disease and to implement an individualized therapeutic approach, according to the custom phenotype. Nevertheless, more studies are needed in order to clarify several important issues with regard to ACOS, such as the genetic risk factors for developing ACOS, the links between genotype and phenotype, the molecular pathways and underlying mechanisms of ACOS, the identification of possible specific biomarkers for diagnosis and targeted treatment, the optimal therapeutic interventions, and finally, the prognosis of ACOS.展开更多
Posterior ankle impingement syndrome(PAIS) is a common injury in athletes engaging in repetitive plantarflexion, particularly ballet dancers and soccer players. Despite the increase in popularity of the posterior twop...Posterior ankle impingement syndrome(PAIS) is a common injury in athletes engaging in repetitive plantarflexion, particularly ballet dancers and soccer players. Despite the increase in popularity of the posterior twoportal hindfoot approach, concerns with the technique remain, including; the technical difficulty, relatively steep learning curve, and difficulty performing simultaneous anterior ankle arthroscopy. The purpose of the current literature review is to provide comprehensive knowledge about PAIS, and to describe a systematic four-stage approach of the posterior two-portal arthroscopy. The etiology, clinical presentation, diagnostic strategies are first introduced followed by options in conservative and surgical management. A detailed systematic approach to posterior hindfoot arthroscopy is then described. This technique allows for systematic review of the anatomic structures and treatment of the bony and/or soft tissue lesions in four regions of interest in the hindfoot(superolateral, superomedial, inferomedial, and inferolateral). The review then discusses biological adjuncts and postoperative rehabilitation and ends with a discussion on the most recent clinical outcomes after posterior hindfoot arthroscopy for PAIS. Although clinical evidence suggests high success rates following posterior hindfoot arthroscopy in the short- and mid-term it may be limited in the pathology that can be addressed due to the technical skills required, but the systematic four-stage approach of the posterior two-portal arthroscopy may improve upon this problem.展开更多
Autoimmune phenomena are common in patients with chronic hepatitis C. Management of chronic hepatitis C/autoimmune hepatitis syndrome has until recently been problematic due to the adverse effects of interferon on aut...Autoimmune phenomena are common in patients with chronic hepatitis C. Management of chronic hepatitis C/autoimmune hepatitis syndrome has until recently been problematic due to the adverse effects of interferon on autoimmune processes and immunosuppression on viral replication. In this report we describe 3 patients with chronic hepatitis C/autoimmune hepatitis overlap syndrome who responded rapidly to direct acting antiviral therapy. The resolution of the autoimmune process supports a direct viral role in its pathophysiology.展开更多
The association of primary sclerosing cholangitis(PSC)and autoimmune hepatitis(AIH)is known as an overlap syndrome(OS).OS can also be described in the setting of concomitant presence of AIH and PSC.These diseases can ...The association of primary sclerosing cholangitis(PSC)and autoimmune hepatitis(AIH)is known as an overlap syndrome(OS).OS can also be described in the setting of concomitant presence of AIH and PSC.These diseases can in some cases be associated with ulcerative colitis.In this case report we describe,to our knowledge,the first case in the literature of a young Caucasian male suffering from ulcerative colitis and an overlap syndrome consisting of an association betweenPSC-AIH,with the concomitant presence of a membranous glomerulonephritis.展开更多
BACKGROUND Extramedullary plasmacytoma(EMP)is a rare kind of soft tissue plasma cell neoplasm without bone marrow involvement;this type of plasma cell neoplasm involves a lack of other systemic characteristics of mult...BACKGROUND Extramedullary plasmacytoma(EMP)is a rare kind of soft tissue plasma cell neoplasm without bone marrow involvement;this type of plasma cell neoplasm involves a lack of other systemic characteristics of multiple myeloma.Primary pulmonary plasmacytoma(PPP),with no specific clinical manifestations,is an exceedingly rare type of EMP.Because of its complexity,PPP is often difficult to diagnose,and there is no report in the literature on cases accompanied by overlap syndrome(OS).CASE SUMMARY A 61-year-old woman without a familial lung cancer history was admitted to our hospital in 2018,for intermittent cough,expectoration,and a stuffy feeling in the chest for 50 years;these symptoms appeared intermittently,especially occurred after being cold,and had been aggravated for the last 10 d.She was diagnosed with pulmonary fibrosis and emphysema,bronchiectasis,OS,and autoimmune hepatic cirrhosis in 2017.A pulmonary examination revealed rough breath sounds in both lungs;other physical examinations found no obvious abnormalities.A routine laboratory work-up showed decreased haemoglobin,increased ESR,and abnormal GGT,ALT,Ig G,γ-globulin,κ-light chain,λ-light chain,rheumatoid factor,and autoimmune antibodies.Emission computed tomography demonstrated abnormally concentrated 99 m Tc-MDP.Chest computed tomography revealed a soft tissue mass in the middle and lower lobes of the right lung.After right middle and inferior lobe resection with complete mediastinal lymph node dissection,immunohistochemical analysis revealed an isolated pulmonary plasmacytoma.The patient received chemotherapy for more than 1.5 years and remains in good general condition.CONCLUSION PPP is a type of EMP,and we report an exceedingly rare presentation of PPP accompanied by OS.展开更多
Objective:To study the M receptor blocker on inhalation in patients with overlap syndrome(chronic obstructive pulmonary disease and Obstructive sleep apnea syndrome)curative effect analysis.Methods:25 patients with ov...Objective:To study the M receptor blocker on inhalation in patients with overlap syndrome(chronic obstructive pulmonary disease and Obstructive sleep apnea syndrome)curative effect analysis.Methods:25 patients with overlap syndrome as the experimental group,chronic obstructive pulmonary disease patients(30)as control group,patients with overlap syndrome use inhaled tiotropium powder treat 30 days,to observe the changes of pulmonary function,polysomnography,and other indicators after treatment.Results:Overlap syndrome were treated by tiotropium bromide inhalation powder,has improved the pulmonary function,the sleep apnea index and lowest nocturnal oxygen saturation after treatment.Conclusion:tiotropium bromide has a preferable effective in treatment of overlap syndrome,COPD and OSAHS are interacting with each other.展开更多
Acquired immunodeficiency syndrome and lupus erythematosus are multisystem diseases that can affect several organs and systems at different stages of disease evolution. Both diseases share common clinical manifestatio...Acquired immunodeficiency syndrome and lupus erythematosus are multisystem diseases that can affect several organs and systems at different stages of disease evolution. Both diseases share common clinical manifestations, which may lead to diagnostic difficulties, especially at the onset of the disease. Another additional challenge is when there is an association of the two pathologies. The objective of this brief review is to describe the clinical manifestations of the diseases and to make considerations regarding the moment of onset of symptoms. Knowledge of these common manifestations and their peculiarities may alert clinicians to possible diagnoses and avoid errors in the evaluation and conduction of these patients.展开更多
The overlap syndrome of functional dyspepsia and irritable bowel syndrome (FD-IBS) is very common and difficult to treat. There are many risk factors of FD-IBS. Mental illness of FD-IBS patients is more serious. Funct...The overlap syndrome of functional dyspepsia and irritable bowel syndrome (FD-IBS) is very common and difficult to treat. There are many risk factors of FD-IBS. Mental illness of FD-IBS patients is more serious. Functional dyspepsia and irritable bowel syndrome have some similarities in the aspects of pathophysiology, pathogenesis, and treatment. We should pay attention to two aspects of the treatment of overlap syndrome, one is simplifying medications, the other is using gastrointestinal motility drug with bidirectional regulative function when necessary. Traditional Chinese medicine in this respect shows some advantages. This review addresses the epidemiology, risk factors, clinical features, pathogenesis and management of FD-IBS.展开更多
In the last two decades two new paradigms changed our way of perceiving primary immunodeficiencies:An increasing number of immune defects are more associated with inflammatory or autoimmune features rather than with i...In the last two decades two new paradigms changed our way of perceiving primary immunodeficiencies:An increasing number of immune defects are more associated with inflammatory or autoimmune features rather than with infections.Some primary immune defects are due to hyperactive pathways that can be targeted by specific inhibitors,providing innovative precision treatments that can change the natural history of diseases.In this article we review some of these“druggable”inborn errors of immunity and describe how they can be suspected and diagnosed in diverse pediatric and adult medicine specialties.Since the availability of precision treatments can dramatically impact the course of these diseases,preventing the development of organ damage,it is crucial to widen the awareness of these conditions and to provide practical hints for a prompt detection and cure.展开更多
基金Supported by National Natural Science Foundation of China,No.82060123National Health Commission of Guizhou Province,No.gzwjk2019-1-082.
文摘BACKGROUND Autoimmune hepatitis(AIH)and primary biliary cholangitis(PBC)are two common clinical autoimmune liver diseases,and some patients have both diseases;this feature is called AIH-PBC overlap syndrome.Autoimmune thyroid disease(AITD)is the most frequently overlapping extrahepatic autoimmune disease.Immunoglobulin(IgG)4-related disease is an autoimmune disease recognized in recent years,characterized by elevated serum IgG4 levels and infiltration of IgG4-positive plasma cells in tissues.CASE SUMMARY A 68-year-old female patient was admitted with a history of right upper quadrant pain,anorexia,and jaundice on physical examination.Laboratory examination revealed elevated liver enzymes,multiple positive autoantibodies associated with liver and thyroid disease,and imaging and biopsy suggestive of pancreatitis,hepatitis,and PBC.A diagnosis was made of a rare and complex overlap syndrome of AIH,PBC,AITD,and IgG4-related disease.Laboratory features improved on treatment with ursodeoxycholic acid,methylprednisolone,and azathioprine.CONCLUSION This case highlights the importance of screening patients with autoimmune diseases for related conditions.
文摘BACKGROUND Survival in patients with autoimmune liver disease overlap syndromes(AILDOS)compared to those with single autoimmune liver disease is unclear.AIM To investigate the survival of patients with AILDOS and assess the accuracy of non-invasive serum models for predicting liver-related death.METHODS Patients with AILDOS were defined as either autoimmune hepatitis and primary biliary cholangitis overlap(AIH-PBC)or autoimmune hepatitis and primary sclerosing cholangitis overlap(AIH-PSC)and were identified from three tertiary centres for this cohort study.Liver-related death or transplantation(liver-related mortality)was determined using a population-based data linkage system.Prognostic scores for liver-related death were compared for accuracy[including liver outcome score(LOS),Hepascore,Mayo Score,model for end-stage liver disease(MELD)score and MELD incorporated with serum sodium(MELD-Na)score].RESULTS Twenty-two AILDOS patients were followed for a median of 3.1 years(range,0.35-7.7).Fourteen were female,the median age was 46.7 years(range,17.8 to 82.1)and median Hepascore was 1(range,0.07-1).At five years post enrolment,57%of patients remained free from liver-related mortality(74%AIH-PBC,27%AIH-PSC).There was no significant difference in survival between AIH-PBC and AIH-PSC.LOS was a significant predictor of liver-related mortality(P<0.05)in patients with AIH-PBC(n=14)but not AIH-PSC(n=8).A LOS cut-point of 6 discriminated liver-related mortality in AIH-PBC patients(P=0.012,log-rank test,100%sensitivity,77.8%specificity)(Harrell's C-statistic 0.867).The MELD score,MELD-Na score and Mayo Score were not predictive of liver-related mortality in any group.CONCLUSION Survival in the rare,AILDOS is unclear.The current study supports the LOS as a predictor of liver-related mortality in AIH-PBC patients.Further trials investigating predictors of survival in AILDOS are required.
基金National Natural Science Foundation of China,No.81873297the Fundamental Research Funds for the Central Public Welfare Research Institutes,China,No.ZZ13-YQ-006Innovation Fund of Chinese Academy of Chinese Medical Sciences,China,No.CI2021A01003.
文摘Irritable bowel syndrome (IBS) is a common chronic gastrointestinal disease with a significant impact on patients’ quality of life and a high socioeconomic burden. And the understanding of IBS has changed since the release of the Rome Ⅳ diagnosis in 2016. With the upcoming Rome Ⅴ revision, it is necessary to review the results of IBS research in recent years. In this review of IBS, we can highlight future concerns by reviewing the results of IBS research on epidemiology, overlap disorders, pathophysiology, and treatment over the past decade and summarizing the latest research.
基金Supported by the National Natural Science Foundation of China,No.81470842 and No.81770572 to Hua J
文摘AIM To investigate the performance of transient elastography(TE) for diagnosis of fibrosis in patients with autoimmune hepatitis-primary biliary cholangitis(AIHPBC) overlap syndrome.METHODS A total of 70 patients with biopsy-proven AIH-PBC overlap syndrome were included. Spearman correlation test was used to analyze the correlation of liver stiffness measurement(LSM) and fibrosis stage. Independent samples Student's t-test or one-way analysis of variance was used to compare quantitative variables. Receiver operating characteristics(ROC) curve was used to calculate the optimal cut-off values of LSM for predicting individual fibrosis stages. A comparison on the diagnostic accuracy for severe fibrosis was made between LSM and other serological scores.RESULTS Patients with AIH-PBC overlap syndrome had higher median LSM than healthy controls(11.3 ± 6.4 k Pa vs 4.3 ± 1.4 k Pa, P < 0.01). LSM was significantly correlated with fibrosis stage(r = 0.756, P < 0.01). LSM values increased gradually with an increased fibrosis stage. The areas under the ROC curves of LSM for stages F ≥ 2, F ≥ 3, and F4 were 0.837(95%CI: 0.729-0.914), 0.910(0.817-0.965), and 0.966(0.893-0.995), respectively. The optimal cut-off values of LSM for fibrosis stages F ≥ 2, F ≥ 3, and F4 were 6.55, 10.50, and 14.45 k Pa, respectively. LSM was significantly superior to fibrosis-4, glutaglumyl-transferase/platelet ratio, and aspartate aminotransferase-to-platelet ratio index scores in detecting severe fibrosis(F ≥ 3)(0.910 vs 0.715, P < 0.01; 0.910 vs 0.649, P < 0.01; 0.910 vs 0.616, P < 0.01, respectively).CONCLUSION TE can accurately detect hepatic fibrosis as a noninvasive method in patients with AIH-PBC overlap syndrome.
文摘We report a 12 years old female patient with an overlap syndrome involving autoimmune hepatitis (AIH) and systemic lupus erythematosus (SLE). The patient presented with jaundice, hepatosplenomegaly, malaise, polyarthralgia, arthritis and butterfly rash on the face. Laboratory tests revealed severe liver dysfunction, Coombs positive hemolytic anemia and a positive ANA/ anti-dsDNA test. Renal biopsy showed class IIA kidney disease, while liver biopsy showed chronic hepatitis with severe inflammatory activity. The patient satisfied the international criteria for both SLE and AIH. Clinical symptoms and laboratory findings of SLE improved with high dose treatment with corticosteroids and azathioprine, however, remission of the liver disease could not be achieved. Repeat biopsy of the liver after three years of therapy revealed ongoing chronic hepatitis with high level of inflammatory activity. The present case indicates that children with liver dysfunction and SLE should be investigated for AIH. There is much diagnostic and therapeutic dilemma in patients with AIH-SLE overlap syndrome.
文摘BACKGROUND Systemic lupus erythematosus(SLE)and antineutrophil cytoplasmic antibodyassociated vasculitis(AAV)are classically thought to cause renal impairment and small vessel vasculitis with different pathophysiologies.Their overlap constitutes a rare rheumatologic disease.To date,only dozens of such cases with biopsyproven glomerulonephritis have been reported worldwide typically in women of childbearing age.Here,we present a unique clinical case due to its rarity and individualized treatment of a Chinese man in his eighth decade of life.CASE SUMMARY A 77-year-old man was admitted to several hospitals for shortness of breath and received nonspecific treatments over the past 3 years.As his symptoms were not completely relieved,he visited our hospital for further treatment.Laboratory examinations revealed kidney dysfunction,severe anaemia,hypocomplementemia,glomerular proteinuria,and microscopic haematuria.Antinuclear antibodies,as well as anti-dsDNA antibodies,were positive.Computed tomography of the chest showed right pleural effusion.Renal biopsy was performed,and histology suggested crescentic glomerulonephritis,pauci-immune type.After treatment with plasmapheresis,glucocorticoid,and cyclophosphamide,the disease was in remission,and the patient remained in a stable condition for over 3 years post-hospital discharge.CONCLUSION Due to its complexity and rarity,SLE and AAV overlap syndrome is easily misdiagnosed.An accurate diagnosis and treatment at the earliest stage may significantly improve the condition and reduce irreversible organ injury.
文摘Objective:To explore the feasibility of tiotropium bromide therapy and its effect on the lung function of patients with asthma-COPD overlap syndrome(ACOS).Method:The 58 subjects selected in this study were all ACOS patients admitted to our hospital(Hohhot First Hospital)from October 2020 to October 2022.They were grouped according to the random number table method and divided into a control group(29 cases)and an observation group(29 cases).The control group received routine treatment plus salmeterol-fluticasone powder inhalation treatment,and the observation group received tiotropium bromide treatment.The relevant indicators of the two groups were compared.Results:The total clinical effective rate of the observation group was significantly higher than that of the control group.Besides,the forced vital capacity(FVC),forced expiratory volume in 1 second(FEV1)levels,and ACT scores of the two groups increased after treatment,with the observation group having better results than the control group.The residual volume-total lung capacity ratio(RV/TLC),acute exacerbation frequency,and CAT scores all decreased,with the observation group showing smaller values than the control group.The difference between the results of both groups were significant(P<0.05)Conclusion:Tiotropium bromide has a significant clinical effect in the treatment of ACOS patients and can effectively improve the lung function of patients.
文摘The myasthenia gravis is an autoimmune disease which can be associated frequently with a thymoma and also with autoimmune pathologies. We report the case of a 64-year-old man affected an overlap syndrome corresponding to the association of autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC). He also presented concomitant myasthenia gravis and thymoma. The physical examination notes only a disturbed voice. The liver tests showed: alkaline phosphatase 600 U/l, alanine aminotransferase 1280 U/l, and aspartate aminotransferase 985 U/l. Viral serologies of hepatitis were all negative, as well as antinuclear, antimitochondrial and antismooth muscle antibodies. The diagnosis of an overlap syndrome was posed according to the biological and hepatic histhological results. An associated myasthenia was confirmed by the results of the EMG and the positivity of the anti-acetylcholine receptor antibody. A chest CT revealed a thymoma. The treatment consisted of ursodeoxycholic acid, prednisone, azathioprine and pyridostigmine. And the patient improved his voice and the liver function. Thymectomy was practiced without incidents.
文摘Although asthma and chronic obstructive pulmonary disease(COPD) are distinct airway diseases characterized by chronic inflammation, in some cases distinguishing between them is puzzling. For example, chronic smoking leads asthmatic inflammation to a differentiated pattern resembling the COPD inflammation, and in some cases to fixed obstruction as in COPD, and on the other hand, few COPD patients may present with airway reversibility. ACOS is the condition sharing features encountered both in asthma and COPD. Asthma-COPD overlap syndrome(ACOS) represents a diagnostic challenge in the clinical practice, since there is lack of specific indicators to distinguish it from asthma or COPD, and moreover, genetic risk factors, underlying pathology and molecular pathways, clinical characteristics, therapeutic interventions, response to treatment and prognosis are poorly described. The management of ACOS is recommended to be individualized and should target on the maximum effectiveness with the least side effects. Combination therapy with ICS/LABA or LAMA, or newly developed specific anti-eosinophil therapies and treatments specifically targeting neutrophils might be of relevance in the management of ACOS, but studies are needed in order to assess the response and prognosis. Based on the current knowledge about ACOS thus far, it would be recommended that we approached chronic obstructive airway disease rather by describing than by classifying the disease; this would allow us to have a picture that better describes the disease and to implement an individualized therapeutic approach, according to the custom phenotype. Nevertheless, more studies are needed in order to clarify several important issues with regard to ACOS, such as the genetic risk factors for developing ACOS, the links between genotype and phenotype, the molecular pathways and underlying mechanisms of ACOS, the identification of possible specific biomarkers for diagnosis and targeted treatment, the optimal therapeutic interventions, and finally, the prognosis of ACOS.
文摘Posterior ankle impingement syndrome(PAIS) is a common injury in athletes engaging in repetitive plantarflexion, particularly ballet dancers and soccer players. Despite the increase in popularity of the posterior twoportal hindfoot approach, concerns with the technique remain, including; the technical difficulty, relatively steep learning curve, and difficulty performing simultaneous anterior ankle arthroscopy. The purpose of the current literature review is to provide comprehensive knowledge about PAIS, and to describe a systematic four-stage approach of the posterior two-portal arthroscopy. The etiology, clinical presentation, diagnostic strategies are first introduced followed by options in conservative and surgical management. A detailed systematic approach to posterior hindfoot arthroscopy is then described. This technique allows for systematic review of the anatomic structures and treatment of the bony and/or soft tissue lesions in four regions of interest in the hindfoot(superolateral, superomedial, inferomedial, and inferolateral). The review then discusses biological adjuncts and postoperative rehabilitation and ends with a discussion on the most recent clinical outcomes after posterior hindfoot arthroscopy for PAIS. Although clinical evidence suggests high success rates following posterior hindfoot arthroscopy in the short- and mid-term it may be limited in the pathology that can be addressed due to the technical skills required, but the systematic four-stage approach of the posterior two-portal arthroscopy may improve upon this problem.
文摘Autoimmune phenomena are common in patients with chronic hepatitis C. Management of chronic hepatitis C/autoimmune hepatitis syndrome has until recently been problematic due to the adverse effects of interferon on autoimmune processes and immunosuppression on viral replication. In this report we describe 3 patients with chronic hepatitis C/autoimmune hepatitis overlap syndrome who responded rapidly to direct acting antiviral therapy. The resolution of the autoimmune process supports a direct viral role in its pathophysiology.
文摘The association of primary sclerosing cholangitis(PSC)and autoimmune hepatitis(AIH)is known as an overlap syndrome(OS).OS can also be described in the setting of concomitant presence of AIH and PSC.These diseases can in some cases be associated with ulcerative colitis.In this case report we describe,to our knowledge,the first case in the literature of a young Caucasian male suffering from ulcerative colitis and an overlap syndrome consisting of an association betweenPSC-AIH,with the concomitant presence of a membranous glomerulonephritis.
基金the National Nature Science Foundation of China,No.81500430 and No.U1304802the Science and Technology Planning Project of Henan Province,No.192102310045,No.182102310544,No.182102310566,and No.182102310573the Henan Medical Science and Technology Tackling Project,No.2018020337,No.2018020332,No.2018020334,and No.2018020320。
文摘BACKGROUND Extramedullary plasmacytoma(EMP)is a rare kind of soft tissue plasma cell neoplasm without bone marrow involvement;this type of plasma cell neoplasm involves a lack of other systemic characteristics of multiple myeloma.Primary pulmonary plasmacytoma(PPP),with no specific clinical manifestations,is an exceedingly rare type of EMP.Because of its complexity,PPP is often difficult to diagnose,and there is no report in the literature on cases accompanied by overlap syndrome(OS).CASE SUMMARY A 61-year-old woman without a familial lung cancer history was admitted to our hospital in 2018,for intermittent cough,expectoration,and a stuffy feeling in the chest for 50 years;these symptoms appeared intermittently,especially occurred after being cold,and had been aggravated for the last 10 d.She was diagnosed with pulmonary fibrosis and emphysema,bronchiectasis,OS,and autoimmune hepatic cirrhosis in 2017.A pulmonary examination revealed rough breath sounds in both lungs;other physical examinations found no obvious abnormalities.A routine laboratory work-up showed decreased haemoglobin,increased ESR,and abnormal GGT,ALT,Ig G,γ-globulin,κ-light chain,λ-light chain,rheumatoid factor,and autoimmune antibodies.Emission computed tomography demonstrated abnormally concentrated 99 m Tc-MDP.Chest computed tomography revealed a soft tissue mass in the middle and lower lobes of the right lung.After right middle and inferior lobe resection with complete mediastinal lymph node dissection,immunohistochemical analysis revealed an isolated pulmonary plasmacytoma.The patient received chemotherapy for more than 1.5 years and remains in good general condition.CONCLUSION PPP is a type of EMP,and we report an exceedingly rare presentation of PPP accompanied by OS.
文摘Objective:To study the M receptor blocker on inhalation in patients with overlap syndrome(chronic obstructive pulmonary disease and Obstructive sleep apnea syndrome)curative effect analysis.Methods:25 patients with overlap syndrome as the experimental group,chronic obstructive pulmonary disease patients(30)as control group,patients with overlap syndrome use inhaled tiotropium powder treat 30 days,to observe the changes of pulmonary function,polysomnography,and other indicators after treatment.Results:Overlap syndrome were treated by tiotropium bromide inhalation powder,has improved the pulmonary function,the sleep apnea index and lowest nocturnal oxygen saturation after treatment.Conclusion:tiotropium bromide has a preferable effective in treatment of overlap syndrome,COPD and OSAHS are interacting with each other.
文摘Acquired immunodeficiency syndrome and lupus erythematosus are multisystem diseases that can affect several organs and systems at different stages of disease evolution. Both diseases share common clinical manifestations, which may lead to diagnostic difficulties, especially at the onset of the disease. Another additional challenge is when there is an association of the two pathologies. The objective of this brief review is to describe the clinical manifestations of the diseases and to make considerations regarding the moment of onset of symptoms. Knowledge of these common manifestations and their peculiarities may alert clinicians to possible diagnoses and avoid errors in the evaluation and conduction of these patients.
文摘The overlap syndrome of functional dyspepsia and irritable bowel syndrome (FD-IBS) is very common and difficult to treat. There are many risk factors of FD-IBS. Mental illness of FD-IBS patients is more serious. Functional dyspepsia and irritable bowel syndrome have some similarities in the aspects of pathophysiology, pathogenesis, and treatment. We should pay attention to two aspects of the treatment of overlap syndrome, one is simplifying medications, the other is using gastrointestinal motility drug with bidirectional regulative function when necessary. Traditional Chinese medicine in this respect shows some advantages. This review addresses the epidemiology, risk factors, clinical features, pathogenesis and management of FD-IBS.
基金Supported by the Italian Ministry of Health RF-2016-02362384the IRCCS Burlo GarofoloNo. RC 24/17
文摘In the last two decades two new paradigms changed our way of perceiving primary immunodeficiencies:An increasing number of immune defects are more associated with inflammatory or autoimmune features rather than with infections.Some primary immune defects are due to hyperactive pathways that can be targeted by specific inhibitors,providing innovative precision treatments that can change the natural history of diseases.In this article we review some of these“druggable”inborn errors of immunity and describe how they can be suspected and diagnosed in diverse pediatric and adult medicine specialties.Since the availability of precision treatments can dramatically impact the course of these diseases,preventing the development of organ damage,it is crucial to widen the awareness of these conditions and to provide practical hints for a prompt detection and cure.