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      Inflammatory Bowel Disease: A Gastrointestinal Presentation of Congenital Plasminogen Deficiency

      case-report
      Author(s): , MD, FRCPC 1 , , MD, PhD, MSc, FRCPC 2 , , MD, MSc, FRCPC 1 ,
      Publication date (Electronic):
      Journal: ACG Case Reports Journal
      Publisher: Wolters Kluwer

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          ABSTRACT

          Plasminogen deficiency (PD) is a rare autosomal recessive disease that results in the formation of fibrin-rich pseudomembranes, which impair wound-healing capacity. We report a 21-year-old man with congenital PD-associated inflammatory bowel disease. After an episode of Clostridioides difficile infection, he developed chronic diarrhea. Colonoscopy revealed moderate friability and erythema of the colon. Histology showed fibrin deposits in the lamina propria of the colonic mucosa with surrounding inflammation and focal ulceration. He was treated with infliximab and achieved clinical remission. To our knowledge, this is the first reported case of colonic involvement of PD.

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          Structure and function of the plasminogen/plasmin system.

          Francis Castellino, Victoria A. Ploplis (2005)
          Activation of the fibrinolytic system is dependent on the conversion of the plasma zymogen, plasminogen (Pg), to the serine protease plasmin (Pm) by the physiological activators urokinase-type Pg activator (uPA) or tissue-type plasminogen activator (tPA). The primary in vivo function of Pm is to regulate vascular patency by degrading fibrin-containing thrombi. However, the identification of Pg/Pm receptors and the ability of Pm to degrade other matrix proteins have implicated Pm in other functions involving degradation of protein barriers, thereby mediating cell migration, an important event in a number of normal e.g., embryogenesis, wound healing, angiogenesis, and pathological, e.g., tumor growth and dissemination, processes. Prior to the development of Pg-deficient mice, much of the evidence for its role in other biological events was based on indirect studies. With the development and characterization of these mice, and ability to apply challenges utilizing a number of animal models that mimic the human condition, a clearer delineation of Pg/Pm function has evolved and has contributed to an understanding of mechanisms associated with a number of pathophysiological events.
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            Molecular and clinical spectrum of type I plasminogen deficiency: A series of 50 patients.

            Reinhard Schneppenheim, Idil Kurtulus, Stefan Seregard … (2006)
            Severe type I plasminogen (PLG) deficiency has been causally linked to a rare chronic inflammatory disease of the mucous membranes that may be life threatening. Here we report clinical manifestations, PLG plasma levels, and molecular genetic status of the PLG gene of 50 patients. The most common clinical manifestations among these patients were ligneous conjunctivitis (80%) and ligneous gingivitis (34%), followed by less common manifestations such as ligneous vaginitis (8%), and involvement of the respiratory tract (16%), the ears (14%), or the gastrointestinal tract (2%). Four patients showed congenital occlusive hydrocephalus, 2 with Dandy-Walker malformation of cerebellum. Venous thrombosis was not observed. In all patients, plasma PLG levels were markedly reduced. In 38 patients, distinct mutations in the PLG gene were identified. The most common genetic alteration was a K19E mutation found in 34% of patients. Transient in vitro expression of PLG mutants R134K, delK212, R216H, P285T, P285A, T319_N320insN, and R776H in transfected COS-7 cells revealed significantly impaired secretion and increased degradation of PLG. These results demonstrate impaired secretion of mutant PLG proteins as a common molecular pathomechanism in type I PLG deficiency.
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              Plasminogen deficiency.

              Sanjay Mehta, Lee A. Shapiro (2008)
              Plasminogen deficiency has emerged as a well-recognized disorder in which reduced levels of plasminogen lead to the development of pseudo membranes on mucosal surfaces, with subsequent end-organ damage of the affected tissue. Ligneous conjunctivitis is the most recognizable, well-documented, and common presentation of the clinical syndromes associated with plasminogen deficiency, although numerous other organs have been reported to be affected. Interestingly, while plasminogen deficiency was initially believed to be related to development of venous thromboembolic disease, more recent data suggest that decreased plasminogen levels may not, in and of themselves, increase the risk of thrombosis. Two types of plasminogen deficiency have been described in the literature. Type I represents a quantitative deficiency and type II a qualitative deficiency. It appears that hypoplasminogenaemia (type I deficiency) is the type most associated with pseudomembrane disease. A variety of genetic mutations has been identified recently and is reported to lead to these disorders. These defects have been identified in diverse populations, with no specific ethnic predilection. However, this disorder may have increased prevalence in areas and communities where consanguinity is more common. Despite the fact that the characteristic lesions are now better recognized and plasminogen levels are accurately and easily measured, adequate treatment of the clinical manifestations of this disorder is lacking. For ligneous conjunctivitis, a plasminogen concentrate formulated into an ophthalmologic preparation has been found to be an effective local therapy. Unfortunately, no plasminogen concentrate is currently available commercially for either systemic or local therapy.
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                Author and article information

                Contributors
                Aducio Thiesen
                Karen I. Kroeker
                Journal
                Journal ID (nlm-ta): ACG Case Rep J
                Journal ID (iso-abbrev): ACG Case Rep J
                Journal ID (coden): ACGCRJ
                Journal ID (pmc): ACGCRJ
                Journal ID (publisher-id): AC9
                Title: ACG Case Reports Journal
                Publisher: Wolters Kluwer (Maryland, MD )
                ISSN (Electronic): 2326-3253
                Publication date Collection: May 2021
                Publication date (Electronic): 02 June 2021
                Volume: 8
                Issue: 5
                Electronic Location Identifier: e00613
                Affiliations
                [1 ]Division of Gastroenterology, University of Alberta, Edmonton, Alberta, Canada
                [2 ]Department of Laboratory Medicine and Pathology, University of Alberta Hospital, University of Alberta, Edmonton, Alberta, Canada
                Author notes
                Correspondence: Karen I. Kroeker, MD, MSc, FRCPC ( kkroeker@ 123456ualberta.ca ).
                Author information
                http://orcid.org/0000-0001-6267-9934
                Article
                Publisher ID: ACGCR-20-1387 Accession ID: 00045
                DOI: 10.14309/crj.0000000000000613
                PMC ID: 8174548
                PubMed ID: 34095331
                SO-VID: 14a91e20-30e5-457d-849b-dc8f18dc753b
                Copyright © © 2021 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of The American College of Gastroenterology.
                License:

                This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.

                History
                Date received : 01 December 2020
                Date accepted : 02 March 2021
                Categories
                Subject: Case Report
                Subject: Inflammatory Bowel Disease
                Custom metadata
                OPEN-ACCESS TRUE

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