Mitochondrial antigens as targets of cellular and humoral auto-immunity in primary biliary cirrhosis

Several factors point toward an auto-immune pathogenesis for primary biliary cirrhosis (PBC), mostly based on the presence of serum auto-antibodies to mitochondrial antigens (AMAs) and autoreactive T cells (both helper and cytotoxic). Interestingly, epitopes recognized by AMA and T-cell clones are located within overlapping areas of the antigens. Moreover, a role for an imbalance in cytokine pattern and for natural-killer lymphocytes has also been proposed. Despite several experimental reports, no clear evidence is available regarding the interaction of these factors leading to bile duct destruction. This article reviews the current reports regarding the auto-immune reaction against mitochondrial auto-antigens in PBC.     

The enigma of primary biliary cirrhosis

Primary biliary cirrhosis (PBC) is a chronic cholestatic disease characterized by a striking predominance in female patients (with most cases diagnosed between ages 40 and 60 yr) as well as serum auto-antibodies to mitochondrial antigens, elevated serum immunoglobulin M, progressive destruction of intrahepatic bile ducts, and, ultimately, liver cirrhosis and failure (1). The precise mechanisms leading to selective destruction of biliary epithelial cells lining intrahepatic bile ducts are still unknown, although numerous immunomediated pathways have been proposed. Genetic background appears to be important in determining susceptibility to the disease (2), but no clear association with alleles in the major histocompatibility complex has been identified. Molecular mimicry either by infections (3) or xenobiotics (4) has been proposed to be capable of breaking tolerance in genetically predisposed individuals, thus leading to onset of PBC. This article describes and discusses the available data regarding the immunomediated pathogenesis of PBC (with particular attention to auto-antibodies and autoreactive T-cells) and presents the recent evidence indicating a role for either xenobiotic chemicals or novel infectious agents in the induction of the disease.     

Erratum to: Engineering cotton (Gossypium hirsutum L.) for resistance to cotton leaf curl disease using viral truncated AC1 DNA sequences

Virus Genes - An error notified in the labelling of Fig.&;nbsp;1b, 3′tAC1 has been erroneously published as 5′tAC1.     

The biology,pathogenetic role,clinical implications,and open issues of serum anti-neutrophil cytoplasmic antibodies

Anti-neutrophil cytoplasmic antibodies (ANCA) are a group of autoantibodies, predominantly IgG, involved in the pathogenesis of several autoimmune disorders, detected either through indirect immunofluorescence or enzyme-linked immunosorbent assay. By means of indirect immunofluorescence, the main patterns are C-ANCA (cytoplasmic) and P-ANCA (perinuclear), while proteinase 3 (PR3) and myeloperoxidase (MPO) represent the main autoantigens in granulomatosis with polyangiitis and microscopic polyangiitis, both belonging to the family of ANCA-associated vasculitis (AAV). While several experiments established the pathogenicity of MPO-ANCA, evidence remains elusive for PR3-ANCA and an additional target antigen, i.e. LAMP2, has been postulated with specific clinical relevance. The presence of a subset of AAV without ANCA may be explained by the presence of further target antigens or the presence of molecules in blood which make ANCA undetectable. A rise in ANCA titers is not necessarily predictive of a flare of disease in AAV if not accompanied by clinical manifestations. ANCA may develop through variable mechanisms, such as autoantigen complementarity, apoptosis impairment, neutrophil extracellular traps dysfunction and molecular mimicry. We will provide herein a comprehensive review of the available evidence on the biological mechanisms, pathogenetic role, and clinical implications of ANCA testing and disease management. Further, we will address the remaining open challenges in the field, including the role of ANCA in inflammatory bowel disease and in cocaine-induced vasculitis.     

Body image in patients with rheumatoid arthritis

We investigated body image in patients with rheumatoid arthritis (RA), correlating it with self-esteem, function, and quality of life. Forty-three RA patients and 39 control individuals from the community between 18 and 70 years of age and paired for gender, age, and body mass index (BMI) were evaluated. Patients were assessed for body image [Body Dysmorphic Disorder Examination (BDDE)], self-esteem (Rosenberg Self-Esteem Scale), function [Health Assessment Questionnaire (HAQ)] and quality of life [Short Form-36 (SF-36)]. The RA group had a mean age of 51.6 years, BMI 26.01, and disease duration 12.2 years. Most participants were categorized in functional class I. The BDDE score of the RA group (51.8) was significantly higher than in the control group (22.6) (p < 0.001). Rosenberg Self-Esteem Scale, HAQ and some SF-36 subscales were worse in the RA group than in the control group. These scores had a direct correlation with body image scores (p < 0.001). Individuals with RA had a worse body image than individuals without this condition. Body image was directly correlated with self-esteem, function, and quality of life.     

A Rare Congenital Finding in an Elderly Lady—Scimitar Syndrome with Dextrocardia in a 79 Year Old

We present a case of a 79-year-old African-American woman with scimitar syndrome and associated dextrocardia. This case represents, to our knowledge, the oldest living patient described in the literature with scimitar syndrome.