Background:The neuropsychiatric disorders due to post-streptococcal autoimmune complications such as Sydenham's chorea(SC)are associated with acute rheumatic fever and rheumatic heart disease(ARF/RHD).An animal mo...Background:The neuropsychiatric disorders due to post-streptococcal autoimmune complications such as Sydenham's chorea(SC)are associated with acute rheumatic fever and rheumatic heart disease(ARF/RHD).An animal model that exhibits char-acteristics of both cardiac and neurobehavioral defects in ARF/RHD would be an important adjunct for future studies.Since age,gender,strain differences,and geno-types impact on the development of autoimmunity,we investigated the behavior of male and female Wistar and Lewis rat strains in two age cohorts(6 weeks and 12 weeks)under normal husbandry conditions and following exposure to group A streptococcus(GAS).Methods:Standard behavioral assessments were performed to determine the impair-ments in fine motor control(food manipulation test),gait and balance(beam walk-ing test),and obsessive-compulsive behavior(grooming and marble burying tests).Furthermore,electrocardiography,histology,and behavioral assessments were per-formed on male and female Lewis rats injected with GAS antigens.Results:For control Lewis rats there were no significant age and gender dependent differences in marble burying,food manipulation,beam walking and grooming be-haviors.In contrast significant age-dependent differences were observed in Wistar rats in all the behavioral tests except for food manipulation.Therefore,Lewis rats were selected for further experiments to determine the effect of GAS.After ex-posure to GAS,Lewis rats demonstrated neurobehavioral abnormalities and cardiac pathology akin to SC and ARF/RHD,respectively.Conclusion:We have characterised a new model that provides longitudinal stability of age-dependent behavior,to simultaneously investigate both neurobehavioral and cardiac abnormalities associated with post-streptococcal complications.展开更多
Dear Editor,We present the reported case of rapid onset bull’s eye maculopathy.Chloroquine(CQ)and its safer,more widely used analogue,hydroxychloroquine(HCQ),were originally developed as antimalarial medications.Howe...Dear Editor,We present the reported case of rapid onset bull’s eye maculopathy.Chloroquine(CQ)and its safer,more widely used analogue,hydroxychloroquine(HCQ),were originally developed as antimalarial medications.However,they have since become essential in the treatment of various autoimmune disorders due to their anti-inflammatory and immunomodulatory properties.HCQ is also being investigated for potential applications in diabetes mellitus,coronavirus disease 2019,heart disease,and as an adjunct in cancer therapy^([1-2]).展开更多
BACKGROUND Common autoimmune diseases(AID)tend to occur together in the same individual and families.Type 1 diabetes(T1D)is caused by an autoimmuneinduced inflammatory destruction of the pancreatic tissue and clusters...BACKGROUND Common autoimmune diseases(AID)tend to occur together in the same individual and families.Type 1 diabetes(T1D)is caused by an autoimmuneinduced inflammatory destruction of the pancreatic tissue and clusters with several other AID.AIM To compare the demographic,clinical,and serological features of patients with single T1D vs those with T1D and associated AID.METHODS From October 1999 to February 2020,a total of 665 patients with T1D and their first-degree relatives were evaluated.RESULTS Compared to patients with isolated T1D,those with T1D+AID were older and had a higher female:male ratio.Average patient age and age at disease onset were higher in T1D+AID vs T1D only.The average time interval between T1D onset and the onset of a second glandular AID was markedly shorter than the time interval between T1D and the occurrence of a non-endocrine AID.T1Dspecific autoantibodies were more frequent in patients with T1D+AID and relatives vs those with T1D only.However,the prevalence of AID and autoantibodies against various tissues were found to be higher in relatives of patients with T1D only compared to relatives of patients with T1D+AID.CONCLUSION Annual serological and subsequent functional screening for AID in patients with T1D and their first-degree relatives is recommended.展开更多
BACKGROUND Bullous pemphigoid(BP)is an autoimmune blistering skin disorder.It is associated with other autoimmune disorders and the use of certain drugs.We describe a case of BP in a patient with ulcerative colitis(UC...BACKGROUND Bullous pemphigoid(BP)is an autoimmune blistering skin disorder.It is associated with other autoimmune disorders and the use of certain drugs.We describe a case of BP in a patient with ulcerative colitis(UC)treated with mesalamine.CASE SUMMARY A 38-year-old male patient with UC and a history of multiple flares was maintained on mesalamine with good clinical response.One year after starting mesalamine,he sought medical care following the onset of a severe itchy rash of several weeks’duration with a recent appearance of skin bullae.A biopsy of the skin revealed subepidermal blistering dermatitis with focal eosinophilic spongiosis.Direct immunofluorescence studies revealed linear IgG and C3 immune reactant deposits at the dermoepidermal junction,consistent with the diagnosis of BP.Prednisone therapy alleviated his symptoms.However,tapering prednisone led to re-eruption of the bullae.CONCLUSION BP should be considered when patients with UC develop skin manifestations.Although BP is not one of the extraintestinal manifestations of UC,there may be an association between these two conditions.Whether treatment with mesalamine or other therapeutic agents plays a role in the development of BP remains unclear.展开更多
Microscopic polyangiitis (MPA) is an autoimmune disorder characterized by pulmonary capillaritis and necrotizing glomerulonephritis triggered by the deposition of oligoimmune complexes.This condition primarily aff ect...Microscopic polyangiitis (MPA) is an autoimmune disorder characterized by pulmonary capillaritis and necrotizing glomerulonephritis triggered by the deposition of oligoimmune complexes.This condition primarily aff ects the lungs and kidneys.^([1])The key pathological features in the lungs associated with MPA include pulmonary interstitial fibrosis and diffuse alveolar hemorrhage (DAH),which clinically present as cough,sputum production,hemoptysis,and dyspnea.[2] In this report,we present a patient with MPA complicated by severe anemia and DAH,notably without the typical symptoms of hemoptysis.展开更多
Antiphospholipid syndrome(APS)is a systemic autoimmune disorder characterized by the presence of antiphospholipid antibodies and is associated with thrombotic events and pregnancy complications.The classification and ...Antiphospholipid syndrome(APS)is a systemic autoimmune disorder characterized by the presence of antiphospholipid antibodies and is associated with thrombotic events and pregnancy complications.The classification and management of APS has evolved over time.The classification criteria for APS include laboratory,macrovascular,microvascular,obstetric,cardiac,and hematologic domains.Management focuses on prevention of thrombotic events and/or anticoagulation as the primary treatment for thrombosis.Postpartum and long-term thromboprophylaxis after delivery are recommended to reduce the risk of thrombotic events.Despite these recommendations,optimal anticoagulation agents and intensity of treatment are still topics of debate.Further research is needed to understand the pathophysiology of APS and improve its management during pregnancy.In this review,we discuss the classification and pathophysiology of APS.Current treatment options and clinical trials are also discussed.展开更多
BACKGROUND Mixed connective tissue disease(MCTD)is a rare autoimmune disorder charac-terized by overlapping features of systemic lupus erythematosus,systemic sc-lerosis,and polymyositis,and presence of anti-U1 ribonuc...BACKGROUND Mixed connective tissue disease(MCTD)is a rare autoimmune disorder charac-terized by overlapping features of systemic lupus erythematosus,systemic sc-lerosis,and polymyositis,and presence of anti-U1 ribonucleoprotein antibodies.Coexistence with tuberculosis(TB),a common infectious disease in endemic areas,poses a significant diagnostic challenge due to overlapping clinical and radio-logical features.CASE SUMMARY We report a 35-year-old Pakistani female presenting with oral ulcers,body rash,worsening dyspnea,and a history of joint pains initially treated as rheumatoid arthritis.She was on antituberculous therapy(ATT)for presumed pulmonary TB.Laboratory findings revealed anemia,leukopenia,raised erythrocyte sedimen-tation rate,positive anti-Sm/RNP,anti-dsDNA,and anti-SSA/Ro antibodies,confirming MCTD with clinical features of systemic lupus erythematosus,Sjogren syndrome,and systemic sclerosis.The patient was also positive for hepatitis C and active TB.Treatment involved corticosteroids alongside continuation of ATT,resulting in significant clinical improvement over 12 days,with resolution of symptoms and improved laboratory parameters.The patient remained stable on follow-up with hydroxychloroquine and prednisolone.CONCLUSION This case highlights the diagnostic complexity when autoimmune diseases coexist with TB,particularly in TB-endemic regions.Early recognition and integrated management of both conditions are crucial to improving outcomes.Clinicians should maintain a broad differential diagnosis and perform compre-hensive immunological workup in patients with overlapping symptoms.展开更多
Clinicians should be cognizant of the close relationship that exists between two of the most common endocrine disorders, primary hypothyroidism and diabetes mellitus. This applies to patients with both type 1 and type...Clinicians should be cognizant of the close relationship that exists between two of the most common endocrine disorders, primary hypothyroidism and diabetes mellitus. This applies to patients with both type 1 and type 2 diabetes mellitus(T1DM and T2 DM respectively). However, the association is greater in T1 DM, probably because of the shared autoimmune predisposition. In patients with T2 DM, the relationship is somewhat weaker and the explanation less clear-cut. Factors such as dietary iodine deficiency, metformin-induced thyroid stimulating hormone suppression and poor glycemic control may all be implicated. Further translational research is required for greater clarification. Biochemical screening for abnormal thyroid function in individuals who have diabetes is warranted, particularly in females with T1 DM, and therapy with L-thyroxine appropriately instituted if hypothyroidism is confirmed.展开更多
Celiac disease （CD） is a common autoimmune disorder, induced by the intake of gluten proteins present in wheat, barley and rye. Contrary to common belief, this disorder is a protean systemic disease, rather than mer...Celiac disease （CD） is a common autoimmune disorder, induced by the intake of gluten proteins present in wheat, barley and rye. Contrary to common belief, this disorder is a protean systemic disease, rather than merely a pure digestive alteration. CD is closely associated with genes that code HLA-Ⅱ antigens, mainly of DQ2 and DQ8 classes. Previously, it was considered to be a rare childhood disorder, but is actually considered a frequent condition, present at any age, which may have multiple complications. Tissue transglutaminase-2 （tTG）, appears to be an important component of this disease, both, in its pathogenesis and diagnosis. Active CD is characterized by intestinal and/or extra-intestinal symptoms, villous atrophy and crypt hyperplasia, and strongly positive tTG auto-antibodies. The duodenal biopsy is considered to be the ＂gold standard＂ for diagnosis, but its practice has significant limitations in its interpretation, especially in adults. Occasionally, it results in a false-negative because of patchy mucosal changes and the presence of mucosal villous atrophy is often more severe in the proximal jejunum, usually not reached by endoscopic biopsies. CD is associated with increased rates of several diseases, such as iron deficiency anemia, osteoporosis, dermatitis herpetiformis, several neurologic and endocrine diseases, persistent chronic hypertransami-nasemia of unknown origin, various types of cancer and other autoimmune disorders. Treatment of CD dictates a strict, life-long gluten-free diet, which results in remission for most individuals, although its effect on some associated extraintestinal manifestations remains to be established.展开更多
In this editorial we comment on the article published in the recent issue of the W orld Journal of Gastroenterology.We focus specifically on the problem of occult hepatitis B virus(HBV)infection,that is a result of pr...In this editorial we comment on the article published in the recent issue of the W orld Journal of Gastroenterology.We focus specifically on the problem of occult hepatitis B virus(HBV)infection,that is a result of previous hepatitis B(PHB)and a source for reactivation of HBV.The prevalence of PHB is underestimated due to the lack of population testing programs.However,this condition not only com-plicate anticancer treatment,but may be responsible for the development of other diseases,like cancer or autoimmune disorders.Here we unveil possible mecha-nisms responsible for realization of these processes and suggest practical approa-ches for diagnosis and treatment.展开更多
In this paper</span><span style="font-family:Verdana;">,</span><span style="font-family:Verdana;"> we present a thorough review of one of the most</span><span style...In this paper</span><span style="font-family:Verdana;">,</span><span style="font-family:Verdana;"> we present a thorough review of one of the most</span><span style="font-family:Verdana;"> life-threatening autoimmune diseases, Systemic lupus erythematosus (lupus). Symptoms, risk factors, including genetic and epidemiological factors are discussed. Treatment, life expectancies, and Health Related Quality of Life of patients with SLE will be discussed as well. Special attention will be given to Lupus Nephritis.展开更多
Alternative splicing is the process of producing variably spliced mRNAs by choosing distinct combinations of splice sites within a messenger RNA precursor.This splicing enables mRNA from a single gene to synthesize di...Alternative splicing is the process of producing variably spliced mRNAs by choosing distinct combinations of splice sites within a messenger RNA precursor.This splicing enables mRNA from a single gene to synthesize different proteins,which have different cellular properties and functions and yet arise from the same single gene.A family of splicing factors,Serine-arginine rich proteins,are needed to initiate the assembly and activation of the spliceosome.Serine and arginine rich splicing factor 1,part of the arginine/serine-rich splicing factor protein family,can either activate or inhibit the splicing of mRNAs,depending on the phosphorylation status of the protein and its interaction partners.Considering that serine and arginine rich splicing factor 1 is either an activator or an inhibitor,this protein has been studied widely to identify its various roles in different diseases.Research has found that serine and arginine rich splicing factor 1 is a key target for neuroprotection,showing its promising potential use in therapeutics for neurodegenerative disorders.Furthermore,serine and arginine rich splicing factor 1 might be used to regulate cancer development and autoimmune diseases.In this review,we highlight how serine and arginine rich splicing factor 1 has been studied concerning neuroprotection.In addition,we draw attention to how serine and arginine rich splicing factor 1 is being studied in cancer and immunological disorders,as well as how serine and arginine rich splicing factor 1 acts outside the central or peripheral nervous system.展开更多
To the Editor:Autoimmune bullous diseases(AIBDs)are a diverse group of autoimmune disorders affecting the skin and mucous membranes,characterized by vesicles,blisters,and erosions due to autoantibodies targeting struc...To the Editor:Autoimmune bullous diseases(AIBDs)are a diverse group of autoimmune disorders affecting the skin and mucous membranes,characterized by vesicles,blisters,and erosions due to autoantibodies targeting structural proteins.AIBDs are mainly categorized into pemphigus,[1]with antibodies against epidermal cell surface proteins,and pemphigoid diseases,[2]targeting proteins in the epidermal basement membrane.[3]Pemphigus vulgaris(PV)is the most common intraepidermal AIBD and typically affects individuals aged 40–60 years,while bullous pemphigoid(BP)is the leading subepidermal AIBD,with incidence increasing among older populations.Given the variety of available topical and systemic treatments,it is vital to evaluate their effectiveness and safety.However,there is a lack of knowledge regarding the comparative effectiveness of these therapies.Our objective is to synthesize existing evidence on AIBD management through direct comparisons and generate treatment rankings using network meta-analysis(NMA)to inform clinical decision-making and optimize therapeutic strategies.展开更多
BACKGROUND: Dubin-Johnson syndrome (DJS) is a rare clinical entity. We describe a case of DJS complicated by systemic lupus erythematosus (SLE). METHODS: A case of congenital hyperbilirubinemia with SLE was evaluated ...BACKGROUND: Dubin-Johnson syndrome (DJS) is a rare clinical entity. We describe a case of DJS complicated by systemic lupus erythematosus (SLE). METHODS: A case of congenital hyperbilirubinemia with SLE was evaluated systematically including review of history, physical examination for the stigmata of chronic liver disease, and other investigations. RESULT: Liver biopsy revealed a black liver with preserved architecture suggestive of DJS. CONCLUSIONS: SLE may develop in DJS. The relationship between DJS and SLE in this case is most likely a chance occurrence.展开更多
BACKGROUND Celiac disease(CeD)is an autoimmune disorder triggered by the immune response to gluten in genetically predisposed individuals.Recent research has unveiled a heightened risk of developing specific malignant...BACKGROUND Celiac disease(CeD)is an autoimmune disorder triggered by the immune response to gluten in genetically predisposed individuals.Recent research has unveiled a heightened risk of developing specific malignant neoplasms(MN)and various malignancies,including gastrointestinal,lymphomas,skin,and others,in individuals with CeD.AIM To investigate the prevalence of MN in hospitalized CeD patients in the United States.METHODS Using data from the National Inpatient Sample spanning two decades,from January 2000 to December 2019,we identified 529842 CeD patients,of which 78128(14.75%)had MN.Propensity score matching,based on age,sex,race,and calendar year,was employed to compare CeD patients with the general non-CeD population at a 1:1 ratio.RESULTS Positive associations were observed for several malignancies,including small intestine,lymphoma,nonmelanoma skin,liver,melanoma skin,pancreas myelodysplastic syndrome,biliary,stomach,and other neuroendocrine tumors(excluding small and large intestine malignant carcinoid),leukemia,uterus,and testis.Conversely,CeD patients exhibited a reduced risk of respiratory and secondary malignancies.Moreover,certain malignancies showed null associations with CeD,including head and neck,nervous system,esophagus,colorectal,anus,breast,malignant carcinoids,bone and connective tissues,myeloma,cervix,and ovary cancers.CONCLUSION Our study is unique in highlighting the detailed results of positive,negative,or null associations between different hematologic and solid malignancies and CeD.Furthermore,it offers insights into evolving trends in CeD hospital outcomes,shedding light on advancements in its management over the past two decades.These findings contribute valuable information to the understanding of CeD’s impact on health and healthcare utilization.展开更多
Anti-acetylcholine receptor antibodies （AAR） are considered pathognomonic and pathogenetic for myasthenia gravis （MG）. AAR detection confirms clinical diagnosis of MG. However, AAR is rarely detected in patients w...Anti-acetylcholine receptor antibodies （AAR） are considered pathognomonic and pathogenetic for myasthenia gravis （MG）. AAR detection confirms clinical diagnosis of MG. However, AAR is rarely detected in patients without MG. The underlying pathophysiological mechanisms in a normal subject without MG have not been adequately addressed in previous studies. The present study reports on a case study of a healthy, elderly man with high AAR titers for 14 years. Pathophysiological mechanisms could be due to AAR heterogeneity in specificity, affinity, and multiform, and to muscle variability in response to AAR.展开更多
It is well known that the treatment of Dampness-heat falling into the lowerburner(DHFLB) is very difficult to deal with by both traditional Chinese medicine(TCM) and western medicine.After TCM system of Epidemic Febri...It is well known that the treatment of Dampness-heat falling into the lowerburner(DHFLB) is very difficult to deal with by both traditional Chinese medicine(TCM) and western medicine.After TCM system of Epidemic Febril Disease(composed of three parts,Shanghan,exogenous febril disease;)展开更多
Myasthenia gravis(MG)is an autoimmune antibody-mediated disorder which causes fluctuating weakness in ocular,bulbar and limb skeletal muscles.There are two major clinical types of MG.Ocular MG(OMG)affects extra ocular...Myasthenia gravis(MG)is an autoimmune antibody-mediated disorder which causes fluctuating weakness in ocular,bulbar and limb skeletal muscles.There are two major clinical types of MG.Ocular MG(OMG)affects extra ocular muscles associated with eye movement and eyelid function and generalized MG results in muscle weakness throughout the body.Patients with OMG have painless fluctuating extra ocular muscles weakness,diplopia and ptosis accompanied by normal visual acuity and pupillary function.Frequently,patients with OMG develop generalized MG over 24 months.Pure OMG is more often earlier in onset(<45 years)than generalized MG.It can also occur as part of an immune-genetic disorder or paraneoplastic syndrome related to thymus tumors.Diagnosis is based on clinical manifestations,laboratory findings,electrophysiological evaluation and pharmacologic tests.Therapeutic strategies for MG consist of symptom relieving medications(e.g.,acetylcholine esterase inhibitors),immunosuppressive agents,and surgical intervention(e.g.,thymectomy).展开更多
CD4^(+)T cells are critical to the development of autoimmune disorders.Glucose,fatty acids,and glutamine metabolisms are the primary metabolic pathways in immune cells,including CD4^(+)T cells.The distinct metabolic p...CD4^(+)T cells are critical to the development of autoimmune disorders.Glucose,fatty acids,and glutamine metabolisms are the primary metabolic pathways in immune cells,including CD4^(+)T cells.The distinct metabolic programs in CD4^(+)T cell subsets are recognized to reflect the bioenergetic requirements,which are compatible with their functional demands.Gut microbiota affects T cell responses by providing a series of antigens and metabolites.Accumulating data indicate that CD4^(+)T cell metabolic pathways underlie aberrant T cell functions,thereby regulating the pathogenesis of autoimmune disorders,including inflammatory bowel diseases,systemic lupus erythematosus,and rheumatoid arthritis.Here,we summarize the current progress of CD4^(+)T cell metabolic programs,gut microbiota regulation of T cell metabolism,and T cell metabolic adaptions to autoimmune disorders to shed light on potential metabolic therapeutics for autoimmune diseases.展开更多
基金RAM Rafeek is recipient of International Postgraduate Research Award(IPRA)from University of New England.CM Lobbe and E.Wilkinson are recipients of student scholarship from the Royal College of Pathologists of Australasia(RCPA).
文摘Background:The neuropsychiatric disorders due to post-streptococcal autoimmune complications such as Sydenham's chorea(SC)are associated with acute rheumatic fever and rheumatic heart disease(ARF/RHD).An animal model that exhibits char-acteristics of both cardiac and neurobehavioral defects in ARF/RHD would be an important adjunct for future studies.Since age,gender,strain differences,and geno-types impact on the development of autoimmunity,we investigated the behavior of male and female Wistar and Lewis rat strains in two age cohorts(6 weeks and 12 weeks)under normal husbandry conditions and following exposure to group A streptococcus(GAS).Methods:Standard behavioral assessments were performed to determine the impair-ments in fine motor control(food manipulation test),gait and balance(beam walk-ing test),and obsessive-compulsive behavior(grooming and marble burying tests).Furthermore,electrocardiography,histology,and behavioral assessments were per-formed on male and female Lewis rats injected with GAS antigens.Results:For control Lewis rats there were no significant age and gender dependent differences in marble burying,food manipulation,beam walking and grooming be-haviors.In contrast significant age-dependent differences were observed in Wistar rats in all the behavioral tests except for food manipulation.Therefore,Lewis rats were selected for further experiments to determine the effect of GAS.After ex-posure to GAS,Lewis rats demonstrated neurobehavioral abnormalities and cardiac pathology akin to SC and ARF/RHD,respectively.Conclusion:We have characterised a new model that provides longitudinal stability of age-dependent behavior,to simultaneously investigate both neurobehavioral and cardiac abnormalities associated with post-streptococcal complications.
文摘Dear Editor,We present the reported case of rapid onset bull’s eye maculopathy.Chloroquine(CQ)and its safer,more widely used analogue,hydroxychloroquine(HCQ),were originally developed as antimalarial medications.However,they have since become essential in the treatment of various autoimmune disorders due to their anti-inflammatory and immunomodulatory properties.HCQ is also being investigated for potential applications in diabetes mellitus,coronavirus disease 2019,heart disease,and as an adjunct in cancer therapy^([1-2]).
文摘BACKGROUND Common autoimmune diseases(AID)tend to occur together in the same individual and families.Type 1 diabetes(T1D)is caused by an autoimmuneinduced inflammatory destruction of the pancreatic tissue and clusters with several other AID.AIM To compare the demographic,clinical,and serological features of patients with single T1D vs those with T1D and associated AID.METHODS From October 1999 to February 2020,a total of 665 patients with T1D and their first-degree relatives were evaluated.RESULTS Compared to patients with isolated T1D,those with T1D+AID were older and had a higher female:male ratio.Average patient age and age at disease onset were higher in T1D+AID vs T1D only.The average time interval between T1D onset and the onset of a second glandular AID was markedly shorter than the time interval between T1D and the occurrence of a non-endocrine AID.T1Dspecific autoantibodies were more frequent in patients with T1D+AID and relatives vs those with T1D only.However,the prevalence of AID and autoantibodies against various tissues were found to be higher in relatives of patients with T1D only compared to relatives of patients with T1D+AID.CONCLUSION Annual serological and subsequent functional screening for AID in patients with T1D and their first-degree relatives is recommended.
文摘BACKGROUND Bullous pemphigoid(BP)is an autoimmune blistering skin disorder.It is associated with other autoimmune disorders and the use of certain drugs.We describe a case of BP in a patient with ulcerative colitis(UC)treated with mesalamine.CASE SUMMARY A 38-year-old male patient with UC and a history of multiple flares was maintained on mesalamine with good clinical response.One year after starting mesalamine,he sought medical care following the onset of a severe itchy rash of several weeks’duration with a recent appearance of skin bullae.A biopsy of the skin revealed subepidermal blistering dermatitis with focal eosinophilic spongiosis.Direct immunofluorescence studies revealed linear IgG and C3 immune reactant deposits at the dermoepidermal junction,consistent with the diagnosis of BP.Prednisone therapy alleviated his symptoms.However,tapering prednisone led to re-eruption of the bullae.CONCLUSION BP should be considered when patients with UC develop skin manifestations.Although BP is not one of the extraintestinal manifestations of UC,there may be an association between these two conditions.Whether treatment with mesalamine or other therapeutic agents plays a role in the development of BP remains unclear.
基金supported by the Provincial Natural Science Foundation of Hunan Province (2024JJ5603)。
文摘Microscopic polyangiitis (MPA) is an autoimmune disorder characterized by pulmonary capillaritis and necrotizing glomerulonephritis triggered by the deposition of oligoimmune complexes.This condition primarily aff ects the lungs and kidneys.^([1])The key pathological features in the lungs associated with MPA include pulmonary interstitial fibrosis and diffuse alveolar hemorrhage (DAH),which clinically present as cough,sputum production,hemoptysis,and dyspnea.[2] In this report,we present a patient with MPA complicated by severe anemia and DAH,notably without the typical symptoms of hemoptysis.
文摘Antiphospholipid syndrome(APS)is a systemic autoimmune disorder characterized by the presence of antiphospholipid antibodies and is associated with thrombotic events and pregnancy complications.The classification and management of APS has evolved over time.The classification criteria for APS include laboratory,macrovascular,microvascular,obstetric,cardiac,and hematologic domains.Management focuses on prevention of thrombotic events and/or anticoagulation as the primary treatment for thrombosis.Postpartum and long-term thromboprophylaxis after delivery are recommended to reduce the risk of thrombotic events.Despite these recommendations,optimal anticoagulation agents and intensity of treatment are still topics of debate.Further research is needed to understand the pathophysiology of APS and improve its management during pregnancy.In this review,we discuss the classification and pathophysiology of APS.Current treatment options and clinical trials are also discussed.
文摘BACKGROUND Mixed connective tissue disease(MCTD)is a rare autoimmune disorder charac-terized by overlapping features of systemic lupus erythematosus,systemic sc-lerosis,and polymyositis,and presence of anti-U1 ribonucleoprotein antibodies.Coexistence with tuberculosis(TB),a common infectious disease in endemic areas,poses a significant diagnostic challenge due to overlapping clinical and radio-logical features.CASE SUMMARY We report a 35-year-old Pakistani female presenting with oral ulcers,body rash,worsening dyspnea,and a history of joint pains initially treated as rheumatoid arthritis.She was on antituberculous therapy(ATT)for presumed pulmonary TB.Laboratory findings revealed anemia,leukopenia,raised erythrocyte sedimen-tation rate,positive anti-Sm/RNP,anti-dsDNA,and anti-SSA/Ro antibodies,confirming MCTD with clinical features of systemic lupus erythematosus,Sjogren syndrome,and systemic sclerosis.The patient was also positive for hepatitis C and active TB.Treatment involved corticosteroids alongside continuation of ATT,resulting in significant clinical improvement over 12 days,with resolution of symptoms and improved laboratory parameters.The patient remained stable on follow-up with hydroxychloroquine and prednisolone.CONCLUSION This case highlights the diagnostic complexity when autoimmune diseases coexist with TB,particularly in TB-endemic regions.Early recognition and integrated management of both conditions are crucial to improving outcomes.Clinicians should maintain a broad differential diagnosis and perform compre-hensive immunological workup in patients with overlapping symptoms.
文摘Clinicians should be cognizant of the close relationship that exists between two of the most common endocrine disorders, primary hypothyroidism and diabetes mellitus. This applies to patients with both type 1 and type 2 diabetes mellitus(T1DM and T2 DM respectively). However, the association is greater in T1 DM, probably because of the shared autoimmune predisposition. In patients with T2 DM, the relationship is somewhat weaker and the explanation less clear-cut. Factors such as dietary iodine deficiency, metformin-induced thyroid stimulating hormone suppression and poor glycemic control may all be implicated. Further translational research is required for greater clarification. Biochemical screening for abnormal thyroid function in individuals who have diabetes is warranted, particularly in females with T1 DM, and therapy with L-thyroxine appropriately instituted if hypothyroidism is confirmed.
文摘Celiac disease （CD） is a common autoimmune disorder, induced by the intake of gluten proteins present in wheat, barley and rye. Contrary to common belief, this disorder is a protean systemic disease, rather than merely a pure digestive alteration. CD is closely associated with genes that code HLA-Ⅱ antigens, mainly of DQ2 and DQ8 classes. Previously, it was considered to be a rare childhood disorder, but is actually considered a frequent condition, present at any age, which may have multiple complications. Tissue transglutaminase-2 （tTG）, appears to be an important component of this disease, both, in its pathogenesis and diagnosis. Active CD is characterized by intestinal and/or extra-intestinal symptoms, villous atrophy and crypt hyperplasia, and strongly positive tTG auto-antibodies. The duodenal biopsy is considered to be the ＂gold standard＂ for diagnosis, but its practice has significant limitations in its interpretation, especially in adults. Occasionally, it results in a false-negative because of patchy mucosal changes and the presence of mucosal villous atrophy is often more severe in the proximal jejunum, usually not reached by endoscopic biopsies. CD is associated with increased rates of several diseases, such as iron deficiency anemia, osteoporosis, dermatitis herpetiformis, several neurologic and endocrine diseases, persistent chronic hypertransami-nasemia of unknown origin, various types of cancer and other autoimmune disorders. Treatment of CD dictates a strict, life-long gluten-free diet, which results in remission for most individuals, although its effect on some associated extraintestinal manifestations remains to be established.
基金Supported by Ministry of Science and Higher education of Russia,No.FGMF-2022-0005Moscow Healthcare Department,No.123040700014-4.
文摘In this editorial we comment on the article published in the recent issue of the W orld Journal of Gastroenterology.We focus specifically on the problem of occult hepatitis B virus(HBV)infection,that is a result of previous hepatitis B(PHB)and a source for reactivation of HBV.The prevalence of PHB is underestimated due to the lack of population testing programs.However,this condition not only com-plicate anticancer treatment,but may be responsible for the development of other diseases,like cancer or autoimmune disorders.Here we unveil possible mecha-nisms responsible for realization of these processes and suggest practical approa-ches for diagnosis and treatment.
文摘In this paper</span><span style="font-family:Verdana;">,</span><span style="font-family:Verdana;"> we present a thorough review of one of the most</span><span style="font-family:Verdana;"> life-threatening autoimmune diseases, Systemic lupus erythematosus (lupus). Symptoms, risk factors, including genetic and epidemiological factors are discussed. Treatment, life expectancies, and Health Related Quality of Life of patients with SLE will be discussed as well. Special attention will be given to Lupus Nephritis.
文摘Alternative splicing is the process of producing variably spliced mRNAs by choosing distinct combinations of splice sites within a messenger RNA precursor.This splicing enables mRNA from a single gene to synthesize different proteins,which have different cellular properties and functions and yet arise from the same single gene.A family of splicing factors,Serine-arginine rich proteins,are needed to initiate the assembly and activation of the spliceosome.Serine and arginine rich splicing factor 1,part of the arginine/serine-rich splicing factor protein family,can either activate or inhibit the splicing of mRNAs,depending on the phosphorylation status of the protein and its interaction partners.Considering that serine and arginine rich splicing factor 1 is either an activator or an inhibitor,this protein has been studied widely to identify its various roles in different diseases.Research has found that serine and arginine rich splicing factor 1 is a key target for neuroprotection,showing its promising potential use in therapeutics for neurodegenerative disorders.Furthermore,serine and arginine rich splicing factor 1 might be used to regulate cancer development and autoimmune diseases.In this review,we highlight how serine and arginine rich splicing factor 1 has been studied concerning neuroprotection.In addition,we draw attention to how serine and arginine rich splicing factor 1 is being studied in cancer and immunological disorders,as well as how serine and arginine rich splicing factor 1 acts outside the central or peripheral nervous system.
基金supported by a grant from the National Natural Science Foundation of China(No.82003347).
文摘To the Editor:Autoimmune bullous diseases(AIBDs)are a diverse group of autoimmune disorders affecting the skin and mucous membranes,characterized by vesicles,blisters,and erosions due to autoantibodies targeting structural proteins.AIBDs are mainly categorized into pemphigus,[1]with antibodies against epidermal cell surface proteins,and pemphigoid diseases,[2]targeting proteins in the epidermal basement membrane.[3]Pemphigus vulgaris(PV)is the most common intraepidermal AIBD and typically affects individuals aged 40–60 years,while bullous pemphigoid(BP)is the leading subepidermal AIBD,with incidence increasing among older populations.Given the variety of available topical and systemic treatments,it is vital to evaluate their effectiveness and safety.However,there is a lack of knowledge regarding the comparative effectiveness of these therapies.Our objective is to synthesize existing evidence on AIBD management through direct comparisons and generate treatment rankings using network meta-analysis(NMA)to inform clinical decision-making and optimize therapeutic strategies.
文摘BACKGROUND: Dubin-Johnson syndrome (DJS) is a rare clinical entity. We describe a case of DJS complicated by systemic lupus erythematosus (SLE). METHODS: A case of congenital hyperbilirubinemia with SLE was evaluated systematically including review of history, physical examination for the stigmata of chronic liver disease, and other investigations. RESULT: Liver biopsy revealed a black liver with preserved architecture suggestive of DJS. CONCLUSIONS: SLE may develop in DJS. The relationship between DJS and SLE in this case is most likely a chance occurrence.
文摘BACKGROUND Celiac disease(CeD)is an autoimmune disorder triggered by the immune response to gluten in genetically predisposed individuals.Recent research has unveiled a heightened risk of developing specific malignant neoplasms(MN)and various malignancies,including gastrointestinal,lymphomas,skin,and others,in individuals with CeD.AIM To investigate the prevalence of MN in hospitalized CeD patients in the United States.METHODS Using data from the National Inpatient Sample spanning two decades,from January 2000 to December 2019,we identified 529842 CeD patients,of which 78128(14.75%)had MN.Propensity score matching,based on age,sex,race,and calendar year,was employed to compare CeD patients with the general non-CeD population at a 1:1 ratio.RESULTS Positive associations were observed for several malignancies,including small intestine,lymphoma,nonmelanoma skin,liver,melanoma skin,pancreas myelodysplastic syndrome,biliary,stomach,and other neuroendocrine tumors(excluding small and large intestine malignant carcinoid),leukemia,uterus,and testis.Conversely,CeD patients exhibited a reduced risk of respiratory and secondary malignancies.Moreover,certain malignancies showed null associations with CeD,including head and neck,nervous system,esophagus,colorectal,anus,breast,malignant carcinoids,bone and connective tissues,myeloma,cervix,and ovary cancers.CONCLUSION Our study is unique in highlighting the detailed results of positive,negative,or null associations between different hematologic and solid malignancies and CeD.Furthermore,it offers insights into evolving trends in CeD hospital outcomes,shedding light on advancements in its management over the past two decades.These findings contribute valuable information to the understanding of CeD’s impact on health and healthcare utilization.
文摘Anti-acetylcholine receptor antibodies （AAR） are considered pathognomonic and pathogenetic for myasthenia gravis （MG）. AAR detection confirms clinical diagnosis of MG. However, AAR is rarely detected in patients without MG. The underlying pathophysiological mechanisms in a normal subject without MG have not been adequately addressed in previous studies. The present study reports on a case study of a healthy, elderly man with high AAR titers for 14 years. Pathophysiological mechanisms could be due to AAR heterogeneity in specificity, affinity, and multiform, and to muscle variability in response to AAR.
文摘It is well known that the treatment of Dampness-heat falling into the lowerburner(DHFLB) is very difficult to deal with by both traditional Chinese medicine(TCM) and western medicine.After TCM system of Epidemic Febril Disease(composed of three parts,Shanghan,exogenous febril disease;)
文摘Myasthenia gravis(MG)is an autoimmune antibody-mediated disorder which causes fluctuating weakness in ocular,bulbar and limb skeletal muscles.There are two major clinical types of MG.Ocular MG(OMG)affects extra ocular muscles associated with eye movement and eyelid function and generalized MG results in muscle weakness throughout the body.Patients with OMG have painless fluctuating extra ocular muscles weakness,diplopia and ptosis accompanied by normal visual acuity and pupillary function.Frequently,patients with OMG develop generalized MG over 24 months.Pure OMG is more often earlier in onset(<45 years)than generalized MG.It can also occur as part of an immune-genetic disorder or paraneoplastic syndrome related to thymus tumors.Diagnosis is based on clinical manifestations,laboratory findings,electrophysiological evaluation and pharmacologic tests.Therapeutic strategies for MG consist of symptom relieving medications(e.g.,acetylcholine esterase inhibitors),immunosuppressive agents,and surgical intervention(e.g.,thymectomy).
基金supported by National Institutes of Health(Grants No.DK105585,DK112436,DK125011,AI150210,and DK124132)。
文摘CD4^(+)T cells are critical to the development of autoimmune disorders.Glucose,fatty acids,and glutamine metabolisms are the primary metabolic pathways in immune cells,including CD4^(+)T cells.The distinct metabolic programs in CD4^(+)T cell subsets are recognized to reflect the bioenergetic requirements,which are compatible with their functional demands.Gut microbiota affects T cell responses by providing a series of antigens and metabolites.Accumulating data indicate that CD4^(+)T cell metabolic pathways underlie aberrant T cell functions,thereby regulating the pathogenesis of autoimmune disorders,including inflammatory bowel diseases,systemic lupus erythematosus,and rheumatoid arthritis.Here,we summarize the current progress of CD4^(+)T cell metabolic programs,gut microbiota regulation of T cell metabolism,and T cell metabolic adaptions to autoimmune disorders to shed light on potential metabolic therapeutics for autoimmune diseases.
