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W B Storch

Publications and source records attributed to W B Storch.

16 recordsLinked to original sources

Autoimmune hepatitides--update 2004.

This update reports is primarily made to update papers published in 2002 and 2003. A selection had to be realized since a search of the literature yielded more than 1600 journal articles. The prevailing opinion of an international group is especially emphasized. Autoimmune hepatitides (AIH)--as the name already suggests it--are no disease entity. We prefer to classify them under the terms of typical and atypical autoimmune hepatitides. Typical autoimmune hepatitis can be subdivided into a type 1 (with antibodies against nuclear material and/or smooth muscle) and a type 2 (with antibodies against endoplasmic reticulum). In the case of atypical autoimmune hepatitides, the detected autoantibodies would have to be stated. The essential feature of an autoimmune hepatitis is the presence of various high-titer autoantibodies circulating in serum, especially such of the IgG class. Special mention is made of the very rare liver ribosomal antibodies (LRA), which are mainly directed against 80S liver ribosomes and point to a so-called virus-associated autoimmune hepatitis. Also discussed are a genetic disposition, which is still speculative in some points, and the existence of so-called autogenes (physiological, pathological/pathogenic and so-called regulator genes). Various noxae, e.g. viruses, might also be "autogenous". Further research in the field of the autoimmune hepatitides (and also autoimmunity and autoimmune diseases as such) is therefore likely to concentrate on viruses and genes and their interrelations.

Animals↗

So-called "overlap syndromes" in hepatology: combinations or associations of concurrent autoimmune liver and other diseases.

Based on present knowledge, the so-called "overlap syndromes" consisting of autoimmune liver diseases do not constitute entities in their own right. Likewise, no generally accepted marker such as an autoantibody is so far known. It would, therefore, be more correct to speak of combinations or associations of two or more concurrent diseases. Sixteen such combinations are considered here.

Autoantibodies↗

Serum antibodies in celiac disease.

Celiac disease is characterized by small intestinal damage which often leads to global malabsorption. For diagnosis an intestinal biopsy to demonstrate the mucosal changes is mandatory. However, serological tests are useful additional tools to confirm the diagnosis and to monitor therapy. These serological tests, which are based on the detection of antibodies against gliadin and autoantibodies against components of the extracellular matrix, are described in this article.

Autoantibodies↗

Identification of an autoimmune enteropathy-related 75-kilodalton antigen.

BACKGROUND & AIMS: We have previously reported a 75-kilodalton autoantigen specific to X-linked autoimmune enteropathy (AIE) associated with tubulonephropathy. The aim of this study was to identify the autoantigen. METHODS: Complementary DNA (cDNA) clones were isolated by immunoscreening a human duodenal cDNA-expression library with serum from a patient with AIE. RESULTS: cDNA encoding the 75-kilodalton antigen (AIE-75) was identified. The composite nucleotide sequence of the cDNA for AIE-75 was 2214 base pairs long and encoded 552 amino acids. The genomic sequence of AIE-75 was found in Sequence DataBank, which consisted of 21 exons and was located on the chromosome 11p14.3. Recombinant AIE-75 specifically reacted with sera from 3 of 4 unrelated patients with AIE but not with 58 control sera. AIE-75 was predominantly distributed in the epithelial cells of the luminal surface and the upper half of the crypts of the intestine and in the proximal renal tubulus. Similarity searches revealed that the AIE-75 cDNA sequence was an authentic form of several colon cancer-related cDNAs of unknown function. The deduced amino acid sequence contained 3 conserved PSD-95/Dlg/ZO-1 (PDZ) domains. CONCLUSIONS: AIE-75 is a PDZ domain-containing protein expressed in the differentiated epithelial cells of the intestine and kidney and may be involved in protein-protein interaction. The identification of the autoantigen may prove useful in the approach to the pathogenesis of this poorly understood disease.

Amino Acid Sequence↗

[Viral genesis and autoimmunity of chronic hepatitis. Suggestion for a dynamically descriptive nomenclature without verification of etiology].

Today, viruses and autoimmunity phenomena are at the focus of interest in chronic hepatitis, not least with respect to the differing treatments, e.g. with interferon and immunosuppressants. Unfortunately, the last international nomenclature fails to take adequate account of the various forms of chronic hepatitis, of the fact that autoimmunity phenomena are predominantly physiological and also occur in the case of viral hepatitides, and also that the cause of autoimmune hepatitis is not clear. If we wish to avoid errors, we should employ a descriptive dynamic classification and not forget that the reclassification may be needed during the natural history of the disease. The indication for treatment should be critically and carefully weighed, and not established too hastily. During treatment--in particular with interferon--the autoimmunity phenomena should be monitored.

Biopsy↗

New autoantibodies and their antigens in autoimmune diseases.

Autoimmune diseases are caused by failure to distinguish between host and foreign (e.g. microbial) antigens. We do not know why autoimmune disease occurs and what the relevant pathogenic mechanisms are. Autoantibodies might be considered as diagnostic markers, e.g. as "witnesses" to or "messengers" of autoimmune disease. Therefore, older and newer autoantibodies, as well as results on their respective antigens, will be considered.

Animals↗

[Autoantibodies against podocytes of the kidney glomerulus].

Novel autoantibodies are described which react with renal podocytes. These antibodies were found in 9 patients (2 with renal disease, 2 with liver disease, 2 with rheumatic disease, 1 with achalasia, 1 with carcinoma of the pancreas and 1 with anemia). Colocalization studies with double immunofluorescence and confocal laser scanning microscopy using monoclonal antibodies against the 44 kD podocyte protein showed a similar fluorescence pattern for both antibodies. The target antigens remain unclear.

Adolescent↗

Autoantibodies to Auerbach's plexus in achalasia.

Achalasia is a motor disorder of the oesopagus characterized by decrease in ganglion cell density in Auerbach's plexus. The cause of the lesion is unknown. This is to repeat on the occurrence of autoimmune phenomena in patients with achalasia, in particular circulating antibodies against Auerbach's plexus and its possible meaning. IgG-antibodies against Auerbach's plexus were determined by standard indirect immunofluorescence. Antibodies to the cytoplasm of Auerbach's plexus were found in 37 of 58 patients with achalasia at variable stages of the disease (I-IV) with a disease duration ranging from 1 to 20 years but only in 4 out of 54 healthy controls (specificity 93%, sensitivity 64%, p < 0.0001), and in none of 12 patients with Hirschsprung's disease as well as 12 patients with cancer of oesophagus and only in one of 11 patients with peptic oesophagitis as well as in one of 13 patients with myasthenia gravis. The present observations suggest that autoimmunity to Auerbach's plexus plays a role in the pathogenesis of achalasia, the mechanism of action is unknown.

Adolescent↗

[Antibodies against Auerbach's plexus--studies using antisera with different fluorochromes].

Human sera containing autoantibodies against the plexus myentericus Auerbach were investigated to find the optimal conditions using double or triple immunofluorescence. FITC-, DTAF-, Texas-red-, tetramethylrhodamine-, Cy-3-, and AMCA-labeled antisera were used. The confirmation of the neuronal binding of the human antibody was achieved by the almost identical binding of monoclonal antibody against the 68 kD neurofilament protein. DTAF- and Cy-3-labeled antisera used as F(ab')2-fragments and absorbed repeatedly proved to be particularly suitable especially since they have a low fading effect.

Autoantibodies↗

Immunohistochemical demonstration of human circulating antibodies against lamina propria of gastric mucosa.

This paper describes a fluorescent pattern of human antibodies circulating in the serum of a 57 year old patient with lymphogranulomatosis. Following incubation with different FITC-labeled anti-human IgG sera including F(ab)2-fragments and with antibodies to human IgA, IgM, IgD, IgE as well as guinea pig serum and FITC-labeled anti-guinea pig serum (for proof the complement binding), it can be assumed that this is a non complement binding IgG antibody directed first of all against the rat gastric mucosal lamina propria (in the cardia and corpus and weaker in the pylorus region). The titre was 1:640. 1:80 diluted serum did not react with duodenum, jejunum, colon, tongue, oesophagus, parotid gland, trachea, lung, heart, diaphragm, liver, spleen, or kidneys. Among others, the species specificity and the antigen binding sides remain to establish.

Animals↗