BACKGROUND Autoimmune autonomic ganglionopathy(AAG),formerly known as acute pandysautonomia,is a rare,acquired,antibody-mediated,potentially curable autonomic disorder that presents with diffuse autonomic failure.High...BACKGROUND Autoimmune autonomic ganglionopathy(AAG),formerly known as acute pandysautonomia,is a rare,acquired,antibody-mediated,potentially curable autonomic disorder that presents with diffuse autonomic failure.High levels of anti-ganglionic nicotinic acetylcholine receptor(gAChR)serum antibodies are detected in approximately 50%of AAG cases,confirming the diagnosis.CASE SUMMARY We present the case of a 68-year-old man who developed autonomic failure gradually over a 2-year period.Recently,the patient was unable to stand upright for more than a few seconds before fainting.Additionally,he presented with decreased sweating,dry mouth,urinary retention,early satiety,weight loss,bloating,constipation,and erectile dysfunction.Neurological examination revealed dilated pupils that were unresponsive to light.Deep tendon reflexes were absent or diminished.Serologic evaluation revealed the presence of gAChR autoantibodies.An orthostatic hypotension test yielded a positive result.The patient did not respond to symptomatic therapy,including midodrine,fludrocortisone and atomoxetine.Second-line therapy with immunoadsorption produced a noticeable clinical improvement;however,orthostatic hypotension persisted.Sequential rituximab infusion therapy successfully led to a significant improvement in symptoms.CONCLUSION Our case report supports the benefit of combined immunomodulatory therapy for refractory AAG cases that are unresponsive to single-agent treatment.展开更多
Approximately 12-72 million people worldwide are co-infected with hepatitis B virus(HBV)and hepatitis delta virus(HDV).This concurrent infection can lead to several severe outcomes with hepatic disease,such as cirrhos...Approximately 12-72 million people worldwide are co-infected with hepatitis B virus(HBV)and hepatitis delta virus(HDV).This concurrent infection can lead to several severe outcomes with hepatic disease,such as cirrhosis,fulminant hepatitis,and hepatocellular carcinoma,being the most common.Over the past few decades,a correlation between viral hepatitis and autoimmune diseases has been reported.Furthermore,autoantibodies have been detected in the serum of patients co-infected with HBV/HDV,and autoimmune features have been reported.However,to date,very few cases of clinically significant autoimmune hepatitis(AIH)have been reported in patients with HDV infection,mainly in those who have received treatment with pegylated interferon.Interestingly,there are some patients with HBV infection and AIH in whom HDV infection is unearthed after receiving treatment with immunosuppressants.Consequently,several questions remain unanswered with the challenge to distinguish whether it is autoimmune or“autoimmune-like”hepatitis being the most crucial.Second,it remains uncertain whether autoimmunity is induced by HBV or delta virus.Finally,we investigated whether the cause of AIH lies in the previous treatment of HDV with pegylated interferon.These pressing issues should be elucidated to clarify whether new antiviral treatments for HDV,such as Bulevirtide or immu-nosuppressive drugs,are more appropriate for the management of patients with HDV and AIH.展开更多
文摘BACKGROUND Autoimmune autonomic ganglionopathy(AAG),formerly known as acute pandysautonomia,is a rare,acquired,antibody-mediated,potentially curable autonomic disorder that presents with diffuse autonomic failure.High levels of anti-ganglionic nicotinic acetylcholine receptor(gAChR)serum antibodies are detected in approximately 50%of AAG cases,confirming the diagnosis.CASE SUMMARY We present the case of a 68-year-old man who developed autonomic failure gradually over a 2-year period.Recently,the patient was unable to stand upright for more than a few seconds before fainting.Additionally,he presented with decreased sweating,dry mouth,urinary retention,early satiety,weight loss,bloating,constipation,and erectile dysfunction.Neurological examination revealed dilated pupils that were unresponsive to light.Deep tendon reflexes were absent or diminished.Serologic evaluation revealed the presence of gAChR autoantibodies.An orthostatic hypotension test yielded a positive result.The patient did not respond to symptomatic therapy,including midodrine,fludrocortisone and atomoxetine.Second-line therapy with immunoadsorption produced a noticeable clinical improvement;however,orthostatic hypotension persisted.Sequential rituximab infusion therapy successfully led to a significant improvement in symptoms.CONCLUSION Our case report supports the benefit of combined immunomodulatory therapy for refractory AAG cases that are unresponsive to single-agent treatment.
文摘Approximately 12-72 million people worldwide are co-infected with hepatitis B virus(HBV)and hepatitis delta virus(HDV).This concurrent infection can lead to several severe outcomes with hepatic disease,such as cirrhosis,fulminant hepatitis,and hepatocellular carcinoma,being the most common.Over the past few decades,a correlation between viral hepatitis and autoimmune diseases has been reported.Furthermore,autoantibodies have been detected in the serum of patients co-infected with HBV/HDV,and autoimmune features have been reported.However,to date,very few cases of clinically significant autoimmune hepatitis(AIH)have been reported in patients with HDV infection,mainly in those who have received treatment with pegylated interferon.Interestingly,there are some patients with HBV infection and AIH in whom HDV infection is unearthed after receiving treatment with immunosuppressants.Consequently,several questions remain unanswered with the challenge to distinguish whether it is autoimmune or“autoimmune-like”hepatitis being the most crucial.Second,it remains uncertain whether autoimmunity is induced by HBV or delta virus.Finally,we investigated whether the cause of AIH lies in the previous treatment of HDV with pegylated interferon.These pressing issues should be elucidated to clarify whether new antiviral treatments for HDV,such as Bulevirtide or immu-nosuppressive drugs,are more appropriate for the management of patients with HDV and AIH.
