scholarly journals Eosinophilic Cholangitis with Poor Prognosis after Corticosteroid- and Ursodeoxycholic Acid-Related Remission of Peripheral and Peribiliary Eosinophilia

2021 ◽  
pp. 232-243
Author(s):  
Takahito Shimomura ◽  
Tomoki Nakajima ◽  
Toshiaki Nakashima ◽  
Yasutaka Morimoto ◽  
Junko Yamaoka ◽  
...  

A 79-year-old man presented with high fever, marked eosinophilia, altered biochemical liver function tests (LFT) with predominance of biliary enzymes, and severe wall thickening of the gallbladder. Magnetic resonance cholangiopancreatography (MRCP) suggested cholecystitis, without signs of biliary strictures. Laparoscopic cholecystectomy and exploratory liver excision revealed eosinophilic cholangitis and cholecystitis, complicated with hepatitis and portal phlebitis. Prednisolone monotherapy rapidly improved peripheral eosinophilia, but not LFT. Liver biopsy showed that infiltrating eosinophils were replaced by lymphocytes and plasma cells. Treatment with ursodeoxycholic acid improved LFT abnormalities. Nevertheless, after 2 months, transaminase-dominant LFT abnormalities appeared. Transient prednisolone dose increase improved LFT, but biliary enzymes’ levels re-elevated and jaundice progressed. The second and third MRCP within a 7-month interval showed rapid progression of biliary stricture. The repeated liver biopsy showed lymphocytic, not eosinophilic, peribiliary infiltration and hepatocellular reaction to cholestasis. Eighteen months after the first visit, the patient died of hepatic failure. Autopsy specimen of the liver showed lymphocyte-dominant peribiliary infiltration and bridging fibrosis due to cholestasis. Though eosinophil-induced biliary damage was an initial trigger, repeated biopsy suggested that lymphocytes played a key role in progression of the disease. Further studies are needed to elucidate the relationship between eosinophils and lymphocytes in eosinophilic cholangitis.

Open Medicine ◽  
2020 ◽  
Vol 15 (1) ◽  
pp. 396-402
Author(s):  
Qisi Zhang ◽  
Yingli Qiao ◽  
Dongmei Yan ◽  
Yuhui Deng ◽  
Mengyang Zhang ◽  
...  

AbstractMultiple myeloma (MM) is an immunoglobulin-producing tumor of plasma cells, which occurs commonly in the elderly. The incidence of myocardial amyloidosis with MM is extremely low and early clinical manifestations are nonspecific. The diversity of clinical manifestations and first episode symptoms often cause misdiagnosis in young patients with myocardial amyloidosis following MM. In this study, we analyzed the clinical data of a young woman with MM and impaired cardiac function combined with echocardiography, electrocardiography (ECG), laboratory data, cell Congo Red staining, and other manifestations to diagnose amyloidosis. Considering the rapid progression, short survival, and poor prognosis in most patients, a clear, definitive, and timely diagnosis is essential for the treatment of patients with MM complicated with myocardial amyloidosis.


Diagnostics ◽  
2021 ◽  
Vol 11 (7) ◽  
pp. 1286
Author(s):  
Junya Sato ◽  
Kazunari Nakahara ◽  
Yosuke Michikawa ◽  
Ryo Morita ◽  
Keigo Suetani ◽  
...  

Endoscopic transpapillary gallbladder drainage (ETGBD) for acute cholecystitis is challenging. We evaluated the influence of pre-procedural imaging and cystic duct cholangiography on ETGBD. Patients who underwent ETGBD for acute cholecystitis were retrospectively examined. The rate of gallbladder contrast on cholangiography, the accuracy of cystic duct direction and location by computed tomography (CT) and magnetic resonance cholangiopancreatography (MRCP), and the relationship between pre-procedural imaging and the technical success of ETGBD were investigated. A total of 145 patients were enrolled in this study. Gallbladder contrast on cholangiography was observed in 29 patients. The accuracy of cystic duct direction and location (proximal or distal, right or left, and cranial or caudal) by CT were, respectively, 79%, 60%, and 58% by CT and 68%, 55%, and 58% by MRCP. Patients showing gallbladder contrast on cholangiography underwent ETGBD with a significantly shorter procedure time and a lower rate of cystic duct injury. No other factors affecting procedure time, technical success, and cystic duct injury were identified. Pre-procedural evaluation of cystic duct direction and location by CT or MRCP was difficult in patients with acute cholecystitis. Patients who showed gallbladder contrast on cholangiography showed a shorter procedure time and a lower rate of cystic duct injury.


2021 ◽  
Vol 11 (6) ◽  
pp. 495
Author(s):  
Jakub Gazda ◽  
Sylvia Drazilova ◽  
Martin Janicko ◽  
Ivica Grgurevic ◽  
Tajana Filipec Kanizaj ◽  
...  

Objective: To identify pretreatment laboratory parameters associated with treatment response and to describe the relationship between treatment response and liver decompensation in patients with primary biliary cholangitis treated with ursodeoxycholic acid. Methods: We defined treatment response as both ALP ≤ 1.67 × ULN and total bilirubin ≤ 2 × ULN. Multiple logistic regression analyses were performed to adjust for confounding effects of sociodemographic variables. Results: Pretreatment total bilirubin ((TB); OR = 0.3388, 95%CI = 0.1671–0.6077), ALT (OR = 0.5306, 95%CI = 0.3830–0.7080), AST (OR = 0.4065, 95%CI = 0.2690–0.5834), ALP (OR = 0.3440, 95%CI = 0.2356–0.4723), total cholesterol ((TC); OR = 0.7730, 95%CI = 0.6242–0.9271), APRI (OR = 0.3375, 95%CI = 0.1833–0.5774), as well as pretreatment albumin (OR = 1.1612, 95%CI = 1.0706–1.2688) and ALT/ALP (OR = 2.4596, 95%CI = 1.2095–5.5472) were associated with treatment response after six months of treatment. Pretreatment TB (OR = 0.2777, 95%CI = 0.1288–0.5228), ALT (OR = 0.5968, 95%CI = 0.4354–0.7963), AST (OR = 0.4161, 95%CI = 0.2736–0.6076), ALP (OR = 0.4676, 95%CI = 0.3487–0.6048), APRI (OR = 0.2838, 95%CI = 0.1433–0.5141), as well as pretreatment albumin (OR = 1.2359, 95%CI = 1.1257–1.3714) and platelet count (OR = 1.0056, 95%CI = 1.0011–1.0103) were associated with treatment response after 12 months of treatment. Treatment response after 6 months of UDCA therapy is significantly associated with treatment response after 12 months of UDCA therapy (OR = 25.2976, 95% CI = 10.5881–68.4917). Treatment responses after 6 and 12 months of UDCA therapy decrease the risk of an episode of liver decompensation in PBC patients (OR = 12.1156, 95%CI = 3.7192–54.4826 and OR = 21.6000, 95%CI = 6.6319–97.3840, respectively). Conclusions: There are several pretreatment laboratory parameters associated with treatment response in patients with primary biliary cholangitis. Treatment response after six months is significantly associated with treatment response after 12 months of ursodeoxycholic acid (UDCA) therapy. Treatment responses after 6 and 12 months of UDCA decrease the risk of an episode of liver decompensation.


1963 ◽  
Vol 112 (1) ◽  
pp. 67-76 ◽  
Author(s):  
R. L. Young ◽  
H. Ward ◽  
D. Hartshorn ◽  
M. Block

2017 ◽  
Vol 71 (5) ◽  
pp. 412-419 ◽  
Author(s):  
Kaushik Majumdar ◽  
Puja Sakhuja ◽  
Amarender Singh Puri ◽  
Kavita Gaur ◽  
Aiman Haider ◽  
...  

BackgroundCoeliac disease (CD) is a gluten-sensitive enteropathy diagnosed on the basis of ESPGHAN criteria and clinical response to gluten-free diet (GFD). Histological abnormalities on liver biopsy have been noted in CD but have seldom been described.AimsTo assess the histological spectrum of ‘coeliac hepatitis’ and possibility of reversal of such features after a GFD.MethodsTwenty-five patients with concomitant CD and hepatic derangement were analysed for clinical profile, laboratory investigations and duodenal and liver biopsy. A histological comparison of pre- and post-GFD duodenal and liver biopsies was carried out, wherever possible.ResultsFifteen patients presenting with CD subsequently developed abnormal liver function tests; 10 patients presenting with liver disease were found to have tissue positive transglutaminase in 70% and antigliadin antibodies in 60%. Serological markers for autoimmune liver disease (AILD) were positive in eight patients. Liver histology ranged from mild reactive hepatitis, chronic hepatitis, steatosis to cirrhosis. Liver biopsies after a GFD were available in six cases, of which five showed a decrease in steatosis, portal and lobular inflammation and fibrosis score.ConclusionCoeliac hepatitis could be a distinct entity and the patients may present with either CD or secondary hepatic derangement. Evaluation for the presence of CD is recommended for patients presenting with AILD, unexplained transaminasaemia or anaemia. This is one of the very few studies demonstrating the continuum of liver histological changes in ‘coeliac hepatitis’. Trial of a GFD may result in clinicopathological improvement of ‘coeliac hepatitis’.


2012 ◽  
Vol 2012 ◽  
pp. 1-12 ◽  
Author(s):  
Mikako Obika ◽  
Hirofumi Noguchi

Nonalcoholic fatty liver disease (NAFLD) is the most common cause of elevated liver function tests results, after the commonly investigated causes have been excluded, and frequently coexists with type 2 diabetes mellitus (T2DM) because the conditions have common risk factors. As both T2DM and NAFLD are related to adverse outcomes of the other, diagnosis and valuation of fatty liver is an important part of the management of diabetes. Although noninvasive methods, such as biomarkers, panel markers, and imaging, may support a diagnostic evaluation of NAFLD patients, accurate histopathological findings cannot be achieved without a liver biopsy. As it is important to know whether steatohepatitis and liver fibrosis are present for the management of NAFLD, liver biopsy remains the gold standard for NAFLD diagnosis and evaluation. Therefore, new investigations of the pathogenesis of NAFLD are necessary to develop useful biomarkers that could provide a reliable noninvasive alternative to liver biopsy.


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