C1 Inhibitor Deficiency and Angioedema of the Small Intestine Masquerading as Crohn’s Disease

Burak, Kelly W.; May, Gary R.

doi:10.1155/2000/414107

Cited by 9 publications

(4 citation statements)

References 8 publications

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“…HAE attacks are characterized by localized inflammation that occurs in subcutaneous and submucosal sites at the extremities, the trachea, or the intestine with little evidence of systemic illness. Acute episodes often include diarrhoea and abdominal pain, with swelling of the bowel wall or abdominal cavity (Burak and May, 2000). The angioedema is believed to be attributable to uncontrolled production of anaphylatoxin complement products, bradykinin and thrombin, which increase vascular permeability (Caliezi et al.…”

Section: Discussionmentioning

confidence: 99%

StcE, a metalloprotease secreted by Escherichia coli O157:H7, specifically cleaves C1 esterase inhibitor

Lathem

Grys

Witowski

et al. 2002

Molecular Microbiology

163

187

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SummaryEscherichia coli O157:H7 causes diarrhoea, haemorrhagic colitis, and the haemolytic uraemic syndrome. We have identified a protein of previously unknown function encoded on the pO157 virulence plasmid of E. coli O157:H7, which is the first described protease that specifically cleaves C1 esterase inhibitor (C1-INH), a member of the serine protease inhibitor family. The protein, named StcE for secreted protease of C1 esterase inhibitor from EHEC (formerly Tagn) Factor XIa, and kallikrein of the coagulation and the inflammation systems respectively. Additionally, C1-INH competes with Factor B for binding to C3b to inhibit the activation of the alternative complement pathway (Jiang et al., 2001). Circulating C1-INH has a M r of 105 kDa, with post-translational glycosylations accounting for nearly half of its mass. Most glycosylations occur in the N-terminal 100 amino acids that are unique to this serpin. Desialylation does not affect the in vitro inhibitory activity of C1-INH, but reduces its circulating half-life in rabbits from >24 h to 3-5 min (Minta, 1981). C1-INH interacts with its target proteases to form large SDS-insoluble complexes that are subsequently removed from circulation (reviewed in Caliezi et al., 2000). Also, data suggest that surfaceassociated C1-INH protects cells from proinflammatory events at their surface (Schmaier et al., 1989;Schmaier et al., 1993;Caliezi et al., 2000).In this article, we describe the identification and characterization of a zinc metalloprotease produced by E. coli O157:H7 that cleaves C1-INH. This protein, termed StcE, is encoded by a gene on pO157, secreted via the etp type II secretion pathway, is positively regulated by Ler, and is highly specific for its substrate. Results Lysates of E. coli carrying pO157 aggregate cultured human T cell linesTo find novel E. coli O157:H7 virulence factors, we looked for cytopathic effects in Jurkat cells, a human T cell lymphoma line, treated with lysates of STEC strains EDL933 and EDL933cu, E. coli K-12 strain C600, C600 carrying pO157 (WAM2035), enteropathogenic E. coli strain E2348/69, and the mouse pathogen Citrobacter rodentium strain DBS100. Lysates of wild-type and transformed E. coli containing pO157 aggregated Jurkat cells (Fig. 1A); lysates of E. coli without pO157 (Fig. 1B) or other bacteria able to induce the A/E phenotype, such as E2348/69 and DBS100, did not. Lysates of EDL933, but not EDL933cu, aggregated MOLT-4 cells ( Fig. 1C and D) but not HL-60, U937, or Raji cells (data not shown), suggesting T cell-lineage specificity for this effect. Identification and cloning of stcETo localize the gene(s) on pO157 responsible for this unusual phenotype, we subjected pO157 to mutagenesis using a minitransposon. The location of the transposon insertion of one mutant whose lysate was unable to aggregate Jurkat cells (WAM2553) was determined to be position 23 772 of pO157 (position based on GenBank accession no. #AF074613). The ORF into which the transposon inserted was designated L7031 (tagA) (Burland et al., 1998), and is...

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Section: Discussionmentioning

confidence: 99%

StcE, a metalloprotease secreted by Escherichia coli O157:H7, specifically cleaves C1 esterase inhibitor

Lathem

Grys

Witowski

et al. 2002

Molecular Microbiology

163

187

View full text Add to dashboard Cite

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“…A thorough literature review has revealed very few case reports of urticaria or angioedema associated with IBDs. These include cases of Hereditary angioedema associated with Crohn's disease [12,13] , angioedema of the small intestine masquerading as Crohn's disease [14,15] , and a single case of chronic urticaria without angioedema in a patient who was subsequently diagnosed with Crohn' s disease [16] . There has also been a case report of chronic urticaria and ulcerative colitis [17] .…”

Section: Discussionmentioning

confidence: 99%

Angioedema associated with Crohnʼs disease: Response to biologics

Habal

Huang

2011

Inflammatory Bowel Diseases

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A 46-year-old female patient with terminal ileum Crohn's disease and ankylosing spondylitis presented with recurrent angioedema and urticaria. Investigations ruled out hereditary angioedema, and environmental or food allergen triggers. She was diagnosed with chronic idiopathic urticaria with angioedema, and was treated with a trial of intravenous immunoglobulin immunotherapy, danazol, prednisone and hydroxyzine. Due to ongoing bowel and arthritic complaints, she was started on infliximab infusions and within 2 treatments, she had complete resolution of the angioedema and urticaria, as well as of the bowel and arthritic symptoms. Unfortunately she developed allergic reactions to the infliximab and was switched to another anti-tumor necrosis factor (TNF)-a agent, adalimumab. Since then, she has had no further angioedema or urticaria, and her Crohn's disease has been quiescent. This is the first known case report of chronic idiopathic urticaria with angioedema coexistent with Crohn's disease that was successfully treated with anti-TNF-a agents.

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“…Читайте нас на сайті: http://med expert.com.ua Крона [7,8], ангіоневротичний набряк тонкої кишки, що маскується під хворобу Крона [4,13], і окремий випадок хронічної кропив'ян ки без ангіоневротичного набряку в пацієнта, в якого згодом діагностували хворобу Крона [14]. Були також повідомлення про випадки хронічної кропив'янки і виразкового коліту [16].…”

Section: хроническая крапивница и воспалительные болезни кишечника -н...unclassified

Chronic urticaria and inflammatory bowel disease — is it worth looking further?

Оkhotnikova¹,

Romanchuk²,

Grishchenko³

2021

MPU

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Chronic urticaria is a common disease that also occurs against the background of inflammatory bowel disease. This case draws attention to the common pathophysiology between autoimmune and autoinflammatory diseases and the need for careful differential diagnosis and further research, which can significantly influence the choice of treatment tactics. Clinical case. An 9-year-old girl with chronic urticaria from birth and recurrent episodes of fever and conjunctivitis was examined at the pediatric ward of the National Children's Specialized Hospital «OKHMATDYT». On the basis of negative results of determination of serum level of immunoglobulins A to gliadin and endomysia celiac disease is excluded, and also by means of modern methods of allergodiagnostics (skin prick-tests, cold test, molecular allergodiagnostics, elimination dietodiagnostics) influence of various is excluded. The girl was diagnosed with chronic idiopathic urticaria with inflammatory bowel disease with trial treatment with ketatifen, bilastine and mesalazine. Due to complaints of recurrent rashes and fever on the background of this therapy, the anamnesis of the disease was re-analyzed and the material was sent for genetic sequencing, which allowed to change the diagnosis to auto-inflammatory disease and prescribed therapy — daily administration of anakinra. Since then, she no longer has recurrent febrile fever or urticaria, and the level of markers of inflammation has returned to normal. Conclusions. This clinical example is interesting for a complex diagnostic search, when a combination of chronic urticaria and inflammatory bowel disease was observed without effect on therapy, which forced to reconsider the diagnosis, which turned out to be even rarer and was finally confirmed by genome sequencing. But despite the fact that the diagnosis was different, it is interesting that on the background of taking mesalazine the girl's condition still improved slightly: became less likely to bother urticarial rash and fever on the background of the rash, which may indicate some common pathogenetic features of inflammation in intestines and chronic idiopathic urticaria, which has an indisputable scientific and practical interest. The research was carried out in accordance with the principles of the Helsinki declaration. The informed consent of the patient was obtained for conducting the studies. No conflict of interest was declared by the authors. Key words: urticaria, inflammatory bowel disease, autoinflammatory diseases, interleukinE1, Biologics, anakinra.

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C1 Inhibitor Deficiency and Angioedema of the Small Intestine Masquerading as Crohn’s Disease

Cited by 9 publications

References 8 publications

StcE, a metalloprotease secreted by Escherichia coli O157:H7, specifically cleaves C1 esterase inhibitor

StcE, a metalloprotease secreted by Escherichia coli O157:H7, specifically cleaves C1 esterase inhibitor

Angioedema associated with Crohnʼs disease: Response to biologics

Chronic urticaria and inflammatory bowel disease — is it worth looking further?

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