scholarly journals Role of Histopathology in Autoimmune Hepatitis

2015 ◽  
Vol 33 (Suppl. 2) ◽  
pp. 53-64 ◽  
Author(s):  
Dina G. Tiniakos ◽  
John G. Brain ◽  
Yvonne A. Bury
Keyword(s):  
Differential Diagnosis ◽  
Liver Disease ◽  
Autoimmune Hepatitis ◽  
Plasma Cells ◽  
Early Stage ◽  
Scoring Systems ◽  
Histological Features ◽  
Pattern Of Injury ◽  
Hepatocellular Necrosis

The diagnosis of autoimmune hepatitis (AIH) is based on a combination of biochemical, immunological and histological features and exclusion of other causes of liver disease. Typical histological features include a chronic hepatitis pattern of injury with portal inflammation and interface activity, predominance of plasma cells in the portal infiltrate, emperipolesis, and hepatocellular rosette formation. Centrilobular injury with prominent hepatocellular necrosis and mononuclear inflammation is now recognised in the histological spectrum of AIH and may represent an early stage of the disease. Liver histology plays a major role in clinical diagnostic scoring systems and is important to confirm or support the clinical diagnosis of AIH. This review focuses on the role of histopathology in AIH and highlights the contribution of histological interpretation to the diagnosis of AIH, differential diagnosis from other entities, recognition of concurrent liver disease, and identification of the so-called overlap or variant syndromes, and addresses the importance of liver biopsy in the management and prognosis of patients with AIH.

scholarly journals The Differential Diagnosis of Hypopigmented Mycosis Fungoides and Vitiligo With Reflectance Confocal Microscopy: A Preliminary Study

2021 ◽  
Vol 7 ◽  
Author(s):  
Huaxu Liu ◽  
Leilei Wang ◽  
Yan Lin ◽  
Xiaofeng Shan ◽  
Min Gao
Keyword(s):  
Differential Diagnosis ◽  
Confocal Microscopy ◽  
Mycosis Fungoides ◽  
Early Stage ◽  
Reflectance Confocal Microscopy ◽  
Inflammatory Conditions ◽  
Dark Space ◽  
Superficial Dermis ◽  
Melanin Contents

Objective: To investigate the role of reflectance confocal microscopy (RCM) in the differential diagnosis of hypopigmented mycosis fungoides (HMF) and vitiligo.Methods: Cases with persistent hypopigmented patches, suspicious of early stage vitiligo, or HMF were imaged with RCM. The melanin contents and inflammatory conditions of the epidermis and superficial dermis of the lesions were compared with the same layers of the adjacent skin, and then, the imaged lesions were biopsied and analyzed by histology.Results: 15 cases were enrolled in this study, and based on the RCM findings, there was just slight or moderate reduction of melanin but no melanin absence in the basal cell layer of HMF lesions. The finding of monomorphous weakly refractile, oval to round cells on the basis of vesicle-like dark space was clearly elucidated in the epidermis of the lesions by RCM, which indicates the Pautrier's microabscesses on histopathology. Among those 15 cases, 13 cases were identified as HMF, and the other two cases were vitiligo, based on RCM findings, which were confirmed by histology analysis.Conclusions: The RCM findings correlated well with histology results in the screening of HMF, which indicates the RCM is an important tool in the early detection and differential diagnosis of HMF.

scholarly journals Impact of Sarcopenia and Myosteatosis in Non-Cirrhotic Stages of Liver Diseases: Similarities and Differences across Aetiologies and Possible Therapeutic Strategies

Biomedicines ◽  
2022 ◽  
Vol 10 (1) ◽  
pp. 182
Author(s):  
Annalisa Cespiati ◽  
Marica Meroni ◽  
Rosa Lombardi ◽  
Giovanna Oberti ◽  
Paola Dongiovanni ◽  
...  
Keyword(s):  
Liver Disease ◽  
Liver Diseases ◽  
Early Stage ◽  
Alcoholic Fatty Liver ◽  
Early Stages ◽  
Advanced Stages ◽  
Related Liver Disease ◽  
And Function ◽  
The Relationship

Sarcopenia is defined as a loss of muscle strength, mass and function and it is a predictor of mortality. Sarcopenia is not only a geriatric disease, but it is related to several chronic conditions, including liver diseases in both its early and advanced stages. Despite the increasing number of studies exploring the role of sarcopenia in the early stages of chronic liver disease (CLD), its prevalence and the relationship between these two clinical entities are still controversial. Myosteatosis is characterized by fat accumulation in the muscles and it is related to advanced liver disease, although its role in the early stages is still under researched. Therefore, in this narrative review, we firstly aimed to evaluate the prevalence and the pathogenetic mechanisms underlying sarcopenia and myosteatosis in the early stage of CLD across different aetiologies (mainly non-alcoholic fatty liver disease, alcohol-related liver disease and viral hepatitis). Secondly, due to the increasing prevalence of sarcopenia worldwide, we aimed to revise the current and the future therapeutic approaches for the management of sarcopenia in CLD.

scholarly journals Autoimmune Hepatitis and Primary Sclerosing Cholangitis: An Overlap Syndrome Resistant to Azathioprine and Intolerant to Tacrolimus with Sepsis Mimicking Flare UP-A Clinical Dilemma

2020 ◽  
Vol 07 (02) ◽  
pp. 11-14
Author(s):  
Sharon T Mathews ◽  
Keyword(s):  
Liver Disease ◽  
Autoimmune Hepatitis ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Kidney Injury ◽  
Scoring Systems ◽  
Overlap Syndrome ◽  
Rare Presentation ◽  
First Line Therapy ◽  
Progressive Liver Disease

Patients with autoimmune liver disease in a frequency of 7% -18% fit into the diagnostic criteria for more than one condition. Up to 12.5% of Autoimmune Hepatitis (AIH) and Primary Sclerosing Cholangitis (PSC) cohorts have a label of AIH/PSC overlap. There can be an unpredictable interval of many years between the diagnosis of the two conditions. There are scoring systems but no established criteria for the diagnosis of AIH/PSC overlap. Therefore, a diagnosis based on combination of biochemistry, autoantibody profile, cholangiogram, and liver histologyis made. Histopathology staging of AIH/PSC overlap influences therapeutic options and prognosis. There is beneficial role of immunosuppression, even though there is a higher relapse rate and evidence of progressive liver disease despite immunosuppression in some cases. Liver related outcomes in this overlap are better than PSC alone but are poorer than AIH. We herein report a rare presentation of an overlap syndrome with AIH and PSC in a patient in whom, treatment with first line therapy showed incomplete response and the salvage therapy was not tolerated, presenting with Acute Kidney Injury (AKI) and a clinical picture of sepsis resembling AIH flare.

The role of interferon regulatory factor 7 in the pathogenicity and immunogenicity of rabies virus in a mouse model

2021 ◽  
Vol 102 (10) ◽  
Author(s):  
Caiqian Wang ◽  
Lei Lv ◽  
Qiong Wu ◽  
Zongmei Wang ◽  
Zhaochen Luo ◽  
...  
Keyword(s):  
Mouse Model ◽  
Rabies Virus ◽  
Plasma Cells ◽  
Early Stage ◽  
Interferon Regulatory Factor ◽  
Type I ◽  
Type I Ifn ◽  
Regulatory Factor ◽  
Interferon Regulatory Factor 7

Rabies is a zoonotic disease caused by the rabies virus (RABV). RABV can lead to fatal encephalitis and is still a serious threat in most parts of the world. Interferon regulatory factor 7 (IRF7) is the main transcriptional regulator of type I IFN, and it is crucial for the induction of IFNα/β and the type I IFN-dependent immune response. In this study, we focused on the role of IRF7 in the pathogenicity and immunogenicity of RABV using an IRF7-/- mouse model. The results showed that the absence of IRF7 made mice more susceptible to RABV, because IRF7 restricted the replication of RABV in the early stage of infection. IRF7 deficiency affected the recruitment of plasmacytoid dendritic cells to the draining lymph nodes (dLNs), reduced the production of type I IFN and expression of IFN-stimulated genes. Furthermore, we found that the ability to produce specific RABV-neutralizing antibody was impaired in IRF7-/- mice. Consistently, IRF7 deficiency affected the recruitment of germinal-centre B cells to dLNs, and the generation of plasma cells and RABV-specific antibody secreting cells. Moreover, the absence of IRF7 downregulated the induction of IFN-γ and reduced type 1 T helper cell (Th1)-dependent antibody production. Collectively, our findings demonstrate that IRF7 promotes humoral immune responses and compromises the pathogenicity of RABV in a mouse model.

scholarly journals Multiple myeloma: Looking beyond standards

2016 ◽  
Vol 02 (01) ◽  
pp. 023-028
Author(s):  
Esha Kaul ◽  
Sanjeev Sharma
Keyword(s):  
Multiple Myeloma ◽  
Diagnostic Criteria ◽  
Plasma Cells ◽  
Residual Disease ◽  
Early Stage ◽  
Treatment Options ◽  
Autologous Stem Cell Transplant ◽  
Cell Transplant ◽  
Frequent Relapses

AbstractMultiple myeloma has been regarded as an incurable disease with frequent relapses. The diagnostic criteria have been revised multiple times to include early stage of the disease where treatment can be effective and can prolong the survival. Newer diagnostic criteria for myeloma have incorporated ≥60% plasma cells in the bone marrow and serum free light chain ratio (involved to uninvolved free light chains) of ≥100. The role of positron emission tomography-computed tomography scans has been recognized, and it has been increasingly utilized upfront in the management of multiple myeloma. Role of minimal residual disease monitoring has been studied in multiple trials and will in near future guide the treatment. Autologous stem cell transplant is still the preferred consolidation therapy after initial three or four drug induction. With the use of novel drugs combinations and with emerging treatment options the standard of care of myeloma patients will change.

scholarly journals From Biomarkers to Models in the Changing Landscape of Chronic Lymphocytic Leukemia: Evolve or Become Extinct

Cancers ◽  
2021 ◽  
Vol 13 (8) ◽  
pp. 1782
Author(s):  
Isabel González-Gascón-y-Marín ◽  
Carolina Muñoz-Novas ◽  
Ana-Eugenia Rodríguez-Vicente ◽  
Miguel Quijada-Álamo ◽  
María Hernández-Sánchez ◽  
...  
Keyword(s):  
Chronic Lymphocytic Leukemia ◽  
Early Stage ◽  
Predictive Biomarkers ◽  
Scoring Systems ◽  
Point Of View ◽  
Lymphocytic Leukemia ◽  
Prognostic Models ◽  
Resistance Mutations ◽  
Mutational Status

Chronic lymphocytic leukemia (CLL) is an extremely heterogeneous disease. With the advent of oral targeted agents (Tas) the treatment of CLL has undergone a revolution, which has been accompanied by an improvement in patient’s survival and quality of life. This paradigm shift also affects the value of prognostic and predictive biomarkers and prognostic models, most of them inherited from the chemoimmunotherapy era but with a different behavior with Tas. This review discusses: (i) the role of the most relevant prognostic and predictive biomarkers in the setting of Tas; and (ii) the validity of classic and new scoring systems in the context of Tas. In addition, a critical point of view about predictive biomarkers with special emphasis on 11q deletion, novel resistance mutations, TP53 abnormalities, IGHV mutational status, complex karyotype and NOTCH1 mutations is stated. We also go over prognostic models in early stage CLL such as IPS-E. Finally, we provide an overview of the applicability of the CLL-IPI for patients treated with Tas, as well as the emergence of new models, generated with data from patients treated with Tas.

Autoimmune Hepatitis and Stellate Cells: An Insight into the Role of Autophagy

2020 ◽  
Vol 27 (35) ◽  
pp. 6073-6095 ◽  
Author(s):  
Shahram Golbabapour ◽  
Kamran Bagheri-Lankarani ◽  
Saeid Ghavami ◽  
Bita Geramizadeh
Keyword(s):  
Autoimmune Hepatitis ◽  
Metabolic Diseases ◽  
Plasma Cells ◽  
Stellate Cells ◽  
Quiescent State ◽  
Comprehensive Overview ◽  
Basic Model ◽  
Insight Into ◽  
Complementary Mechanism

Autoimmune hepatitis is a necroinflammatory process of liver, featuring interface hepatitis by T cells, macrophages and plasma cells that invade to periportal parenchyma. In this process, a variety of cytokines are secreted and liver tissues undergo fibrogenesis, resulting in the apoptosis of hepatocytes. Autophagy is a complementary mechanism for restraining intracellular pathogens to which the innate immune system does not provide efficient endocytosis. Hepatocytes with their particular regenerative features are normally in a quiescent state, and, autophagy controls the accumulation of excess products, therefore the liver serves as a basic model for the study of autophagy. Impairment of autophagy in the liver causes the accumulation of damaged organelles, misfolded proteins and exceeded lipids in hepatocytes as seen in metabolic diseases. In this review, we introduce autoimmune hepatitis in association with autophagy signaling. We also discuss some genes and proteins of autophagy, their regulatory roles in the activation of hepatic stellate cells and the importance of lipophagy and tyrosine kinase in hepatic fibrogenesis. In order to provide a comprehensive overview of the regulatory role of autophagy in autoimmune hepatitis, the pathway analysis of autophagy in autoimmune hepatitis is also included in this article.

Community acquired pneumonia. Differential diagnosis. COVID-19

2020 ◽  
Vol 15 (16) ◽  
pp. 15-27
Author(s):  
V. N. Sokolov ◽  
◽  
L. V. Anischenko ◽  
A. S. Byanov ◽  
O. V. Nikitina ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Dynamic Models ◽  
Early Stage ◽  
Community Acquired Pneumonia ◽  
Ct Imaging ◽  
Successful Outcome ◽  
Differential Diagnostics ◽  
Radiological Society ◽  
Large Patient

Introduction. The paper presents the results of X-ray examination of patients with lung lesions caused by diseases and computed tomography (CT) of patients with COVID-19 of varying degrees of infiltration according to the categorical CT scheme from CO-RADS 1 to CO-RADS 6, developed by the Dutch Radiological Society (NVvR). Good results can be achieved by detecting the early stage of the disease in COVID-19. The work considers indications, contraindications, methodology and interpretation, at different phases of infil-tration in COVID-19. Objective. To review the dynamic models of CT imaging of lungs affected by COVID-19, to define CT character of primary and post-primary infection and discuss the role of imaging in differential diagnostics and monitoring of the pathology. Materials and research methods. 85 patients (the median of 48 y.o. to the 17 to 84 y.o. range) were examined. All patients un-derwent CT procedure («Toshiba Corp.» 64-sl. CT). Results. At an early stage bilateral basal peripheral changes of the «ground glass» type opacity, generally of minuscule area, pre-dominate which precurse development of viral pneumonia in COVID-19. As the disease progresses areas of change in the tissue appear patterned according to the type of «patchwork», consolidation areas form resembling OP «reverse halo». Complete resorption of changes in lungs is likely. Resolution of pathological changes could be observed after a month and longer with a successful outcome. Conclusion. At the stage of the outbreak progression the role of CT imaging methods in assessing the condition of lungs could not be overestimated. They provide means to identify changes at the subclinical stage, to assess the severity of the course and conduct differential diagnostics, but also to inform about the likelihood of COVID-19 when PCR testing results are lacking. The role of CT is in-deed prevailing in clinical conditions of limited resources and while coping with a large patient cohort with acute respiratory diseases of undefined aetiology. Key words: Differential diagnosis; CT examination; COVID-19; CO-RADS scheme.

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