Role of autoimmunity in primary biliary cirrhosis

Primary biliary cirrhosis(PBC) is an autoimmune liver disease characterized by the presence of serum autoantibodies and chronic nonsuppurative destructive cholangitis.The pathogenesis of PBC involves environmental factors,genetic predisposition and loss of immune tolerance.In recent years,it has become univocally accepted that an inappropriately activated immune response is one of the most important factors in PBC.In this study,the role of autoimmunity in PBC is summarized and a feasible research orientation is recommended.

Nodular regenerative hyperplasia:Evolving concepts on underdiagnosed cause of portal hypertension

Nodular regenerative hyperplasia(NRH)is a rare liver condition characterized by a widespread benign transformation of the hepatic parenchyma into small regenerative nodules.NRH may lead to the development of non-cirrhotic portal hypertension.There are no published systematic population studies on NRH and our current knowledge is limited to case reports and case series.NRH may develop via autoimmune,hematological,infectious,neoplastic,or drug-related causes.The disease is usually asymptomatic,slowly or nonprogressive unless complications of portal hypertension develop.Accurate diagnosis is made by histopathology,which demonstrates diffuse micronodular transformation without fibrous septa.Lack of perinuclear collagen tissue distinguishes NRH from typical regenerative nodules in the cirrhotic liver.While the initial treatment is to address the underlying disease,ultimately the therapy is directed to the management of portal hypertension.The prognosis of NRH depends on both the severity of the underlying illness and the prevention of secondary complications of portal hypertension.In this review we detail the epidemiology,pathogenesis,diagnosis,management,and prognosis of NRH.

Primary Sjgren's Syndrome Accompanied by Intestinal Obstruction: a Case Report and Literature Review

SJOGREN＇S syndrome （SS） is a chronic inflammatory autoimmune disease characterized by the infiltration of lymphocytes and plasma cells in exocrine glands, especially salivary and lacrimal gland interstitium. The clinical manifestations of SS are complex. When the digestive system is involved,

B cell depletion in treating primary biliary cirrhosis:Pros and cons

Primary biliary cirrhosis (PBC) is a progressive autoim- mune liver disease of unknown etiology that affects almost exclusively women.Ursodeoxycholic acid (UDCA) is currently the only approved drug by Food and Drug Administration for patients with PBC.Although the precise pathogenesis of PBC remains unclear,it has been postulated that many cell populations,including B cells,are involved in the ongoing inflammatory process,which implicates,not surprisingly,a potential thera- peutic target of depleting B cell to treat this disorder.Rituximab is a chimeric anti-CD20 monoclonal antibody that has been approved for the treatment of lymphoma and some autoimmune diseases such as rheumatoid arthritis.Whether it is effective in the treatment of PBC has not been evaluated.Recently,Tsuda et al [1] demon- strated that B cell depletion with rituximab significantly reduced the number of anti-mitochondrial antibodies (AMA)-producing B cells,AMA titers,the plasma levels of immunoglobulins (IgA,IgM and IgG) as well as se- rum alkaline phosphatase,and it was well tolerated by all the treated patients with no serious adverse events.This observation provides a novel treatment option for the patients with PBC who have incomplete response to UDCA.

PRESENCE OF ANTILAMIN ANTIBODIES IN SERA OF PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS

In this study, we characterized specifically-stained sera from patients with systemic lupus erythematosus (SLE) which had been shown to display the homogeneous or peripheral region of nuclei by indirect immunofluorescence (IIF). By western blotting, we demonstrated that in some cases there was a correlation between the peripheral or homogenous. IIF staining of nuclei by sera from patients with SLE and the presence of autoantibodies to lamins. Here we first report the presence of 2. 2% anti-lamin autoantibodies in the sera among the 174 patients with SLE in China.

Microscopic colitis

Microscopic colitis may be defined as a clinical syndrome, of unknown etiology, consisting of chronic watery diarrhea, with no alterations in the large bowel at the endoscopic and radiologic evaluation. Therefore, a definitive diagnosis is only possible by histological analysis. The epidemiological impact of this disease has become increasingly clear in the last years, with most data coming from Western countries. Microscopic colitis includes two histological subtypes [collagenous colitis (CC) and lymphocytic colitis (LC)] with no differences in clinical presentation and management. Collagenous colitis is characterized by a thickening of the subepithelial collagen layer that is absent in LC. The main feature of LC is an increase of the density of intra-epithelial lymphocytes in the surface epithelium. A number of pathogenetic theories have been proposed over the years, involving the role of luminal agents, autoimmunity, eosinophils, genetics (human leukocyte antigen), biliary acids, infections, alterations of pericryptal fibroblasts, and drug intake; drugs like ticlopidine, carbamazepine or ranitidine are especially associated with the development of LC, while CC is more frequently linked to cimetidine, non-steroidal antiinflammatory drugs and lansoprazole. Microscopic colitis typically presents as chronic or intermittent watery diarrhea, that may be accompanied by symptoms such as abdominal pain, weight loss and incontinence. Recent evidence has added new pharmacological options for the treatment of microscopic colitis:the role of steroidal therapy, especially oral budesonide, has gained relevance, as well as immunosuppressive agents such as azathioprine and 6-mercaptopurine. The use of anti-tumor necrosis factoragents, infliximab and adalimumab, constitutes a new, interesting tool for the treatment of microscopic colitis, but larger, adequately designed studies are needed to confirm existing data.

Presence of Alternaria in Scalp of Patients with Alopecia Areata： Triggering Factor or Coexistence？

AA （Alopecia areata） is the most frequent cause of inflammation-induced hair loss, affecting 0.1 to 0.2% of population worldwide. The development of organ-specific autoimmune reactions directed against anagen hair follicles seems to play a key role in the pathogenesis of alopecia areata. However, the triggering antigen（s） responsible for inducing autoimmune phenomena in these individuals remain unknown. Viral, bacterial or fungal pathogens have been implied as possible triggering factors of autoimmune reactions. The present study aims to identify the role of dematiaceous fungi in the pathogenesis of alopecia areata. 30 patients diagnosed clinically as alopecia areata and 30 normal age matched persons have undergone mycological examination. Mycology examination of the epidermal scrapings was done by DME （direct microscopic examination）, culture on SDA （sabouraud＇s dextrose agar） and imaging. There is significantly higher percentage of positive results for Alternaria species by culture on sabouraud＇s agar in patients group （20%） compared to controls （13.3%） P-value 〈 0.05. The possible role of Alternaria antigens （e.g. antigens involved in melanin synthesis） in triggering autoimmunity in alopecia areata still needs further research on a wider scale of cases.

An update on primary ovarian insufficiency

Primary ovarian insufficiency(POI) occurs in about 1% of female population under the age of 40,leading to reproductive problems,an earlier encounter with menopausal symptoms,and complicated diseases.There are three presumable mechanisms involved in the development of POI,namely apoptosis acceleration,follicular maturation blocking and premature follicle activation,through the following studied causes:(i) chromosomal abnormalities or gene mutations:mostly involve X chromosome,such as FMR1 premutation;more and more potentially causal genes have been screened recently;(ii) metabolic disorders such as classic galactosaemia and 17-OH deficiency;(iii) autoimmune mediated ovarian damage:observed alone or with some certain autoimmune disorders and syndromes;but the specificity and sensitivity of antibodies towards ovary are still questionable;(iv) iatrogenic:radiotherapy or chemotherapy used in cancer treatment,as well as pelvic surgery with potential threat to ovaries' blood supply can directly damage ovarian function;(v) virus infection such as HIV and mumps;(vi) toxins and other environmental/lifestyle factors:cigarette smoking,toxins(e.g.,4-vinylcyclohexene diepoxide),and other environmental factors are associated with the development of POI.The etiology of a majority of POI cases is not identified,and is believed to be multifactorial.Strategies to POI include hormone replacement and infertility treatment.Assisted conception with donated oocytes has been proven to achieve pregnancy in POI women.Embryo cryopreservation,ovarian tissue cryopreservation and oocyte cryopreservation have been used to preserve ovarian reserve in women undergoing cancer treatments.