4
views
0
recommends
+1 Recommend
1 collections
    0
    shares
      • Record: found
      • Abstract: found
      • Article: found
      Is Open Access

      Manifestaciones laríngeas de la enfermedad relacionada con IgG4. Revisión sistemática Translated title: Laryngeal manifestations of IgG4-related disease. Systematic review

      review-article

      Read this article at

      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          RESUMEN: Introducción y objetivos: La enfermedad relacionada con IgG4 (ER-IgG4) es un trastorno sistémico autoinmune caracterizado por la infiltración tisular por células plasmáticas positivas para IgG4, aparición de fibrosis y disfunción orgánica. Puede dar afectación a muchos niveles del organismo. El objetivo de este trabajo es identificar las posibles manifestaciones de esta enfermedad a nivel laríngeo. Material y métodos: Se realizó una revisión sistemática de la literatura médica publicada entre 2003 y abril de 2021, en busca de los casos descritos de ER-IgG4 con afectación laríngea. La búsqueda se realizó a través de las bases de datos Pubmed, Web of Science y Scopus. Se seleccionaron 23 casos, todos ellos con demostración histológica de la enfermedad a ese nivel. Resultados: La mayoría de los pacientes eran varones, con edades comprendidas entre los 41 y los 60 años. Las lesiones a nivel de la laringe mayoritarimaente tenían un aspecto pseudotumor. La zona de aparición preferente de estas fue la supraglotis. Con tratamiento corticoideo los pacientes suelen evolucionar favorablemente. Sin embargo, gran parte de estos sufrió importantes retrasos en el diagnóstico, así como cirugías agresivas. Conclusiones: La afectación laríngea es infrecuente en la ER-IgG4. Se debe tener en mente esta entidad ante lesiones pseudotumorales con biopsias negativas para malingnidad, dado que un diagnóstico temprano y un tratamiento adecuado son efectivos para prevenir secuelas importantes a largo plazo.

          Translated abstract

          SUMMARY: Introduction and objectives: IgG4-Related disease (IgG4-RD) is a systemic autoimmune disorder characterized by tissue infiltration with IgG4-secreting plasma cells, fibrosis and organic disfunction. It can involve multiple sites. The objectives in this article are to identify the laryngeal involvement of this disease. Methods: A systematic review was conducted by collecting the literature published between 2003 and February 2021, seeking for case reports of IgG4-RD with laryngeal involvement. The bibliographic databases utilized were Pubmed, Web of Science and Scopus. 22 cases were selected, all of them including histological findings in the larynx. Results: Most of the patients were male, between 41 and 60 years old. The most common laryngeal manifestations were pseudotumoral neoformations. The supraglottis was the area were most of these lesions appeared. In this disease the standard treatment involves glucocorticoids, often with an optimal clinical response. However, a great part of the patients included in the review got a delayed diagnosis, some of them leading to aggressive surgical procedures. Conclusions: Laryngeal manifestations in the IgG4-Related disease are unusual. That's why it should be considered when considering the differential diagnosis of malignant tumors. This is an illness that needs to be suspected in some cases, to ensure a prompt diagnosis and a proper treatment, thus avoiding long-term sequelae.

          Related collections

          Most cited references35

          • Record: found
          • Abstract: found
          • Article: not found

          Consensus statement on the pathology of IgG4-related disease.

          IgG4-related disease is a newly recognized fibro-inflammatory condition characterized by several features: a tendency to form tumefactive lesions in multiple sites; a characteristic histopathological appearance; and-often but not always-elevated serum IgG4 concentrations. An international symposium on IgG4-related disease was held in Boston, MA, on 4-7 October 2011. The organizing committee comprising 35 IgG4-related disease experts from Japan, Korea, Hong Kong, the United Kingdom, Germany, Italy, Holland, Canada, and the United States, including the clinicians, pathologists, radiologists, and basic scientists. This group represents broad subspecialty expertise in pathology, rheumatology, gastroenterology, allergy, immunology, nephrology, pulmonary medicine, oncology, ophthalmology, and surgery. The histopathology of IgG4-related disease was a specific focus of the international symposium. The primary purpose of this statement is to provide practicing pathologists with a set of guidelines for the diagnosis of IgG4-related disease. The diagnosis of IgG4-related disease rests on the combined presence of the characteristic histopathological appearance and increased numbers of IgG4⁺ plasma cells. The critical histopathological features are a dense lymphoplasmacytic infiltrate, a storiform pattern of fibrosis, and obliterative phlebitis. We propose a terminology scheme for the diagnosis of IgG4-related disease that is based primarily on the morphological appearance on biopsy. Tissue IgG4 counts and IgG4:IgG ratios are secondary in importance. The guidelines proposed in this statement do not supplant careful clinicopathological correlation and sound clinical judgment. As the spectrum of this disease continues to expand, we advocate the use of strict criteria for accepting newly proposed entities or sites as components of the IgG4-related disease spectrum.
            Bookmark
            • Record: found
            • Abstract: found
            • Article: not found

            A new clinicopathological entity of IgG4-related autoimmune disease.

            Autoimmune pancreatitis (AIP) is occasionally associated with other autoimmune diseases. To investigate the pathophysiology of AIP, we immunohistochemically examined the pancreas and other organs in eight patients with AIP, and in controls, using anti-CD4-T and CD8-T cell subsets, as well as IgG4 antibodies. In AIP patients, severe or moderate infiltration of IgG4-positive plasma cells associated with CD4- or CD8-positive T lymphocytes was detected in the peripancreatic tissue (6/6), bile duct (8/8), gallbladder (8/8), portal area of the liver (3/3), gastric mucosa (5/7), colonic mucosa (2/2), salivary glands (1/2), lymph nodes (6/6), and bone marrow (2/2), as well as in the pancreas (8/8). There were few IgG4-positive plasma cells at the same sites in controls. These results suggest that AIP is not simply pancreatitis but that it is a pancreatic lesion involved in IgG4-related systemic disease with extensive organ involvement. We propose a new clinicopathological entity, of a systemic IgG4-related autoimmune disease in which AIP and its associated diseases might be involved. Autoimmune pancreatitis (AIP) is occasionally associated with other autoimmune diseases.
              Bookmark
              • Record: found
              • Abstract: found
              • Article: not found

              High serum IgG4 concentrations in patients with sclerosing pancreatitis.

              Sclerosing pancreatitis is a unique form of pancreatitis that is characterized by irregular narrowing of the main pancreatic duct, lymphoplasmacytic inflammation of the pancreas, and hypergammaglobulinemia and that responds to glucocorticoid treatment. Preliminary studies suggested that serum IgG4 concentrations are elevated in this disease but not in other diseases of the pancreas or biliary tract. We measured serum IgG4 concentrations using single radial immunodiffusion and an enzyme-linked immunosorbent assay in 20 patients with sclerosing pancreatitis, 20 age- and sex-matched normal subjects, and 154 patients with pancreatic cancer, ordinary chronic pancreatitis, primary biliary cirrhosis, primary sclerosing cholangitis, or Sjögren's syndrome. Serum concentrations of immune complexes and the IgG4 subclass of immune complexes were determined by means of an enzyme-linked immunosorbent assay with monoclonal rheumatoid factor. The median serum IgG4 concentration in the patients with sclerosing pancreatitis was 663 mg per deciliter (5th and 95th percentiles, 136 and 1150), as compared with 51 mg per deciliter (5th and 95th percentiles, 15 and 128) in normal subjects (P<0.001). The serum IgG4 concentrations in the other groups of patients were similar to those in the normal subjects. In patients with sclerosing pancreatitis, serum concentrations of immune complexes and the IgG4 subclass of immune complexes were significantly higher before glucocorticoid therapy than after four weeks of such therapy. Glucocorticoid therapy induced clinical remissions and significantly decreased serum concentrations of IgG4, immune complexes, and the IgG4 subclass of immune complexes. Patients with sclerosing pancreatitis have high serum IgG4 concentrations, providing a useful means of distinguishing this disorder from other diseases of the pancreas or biliary tract.
                Bookmark

                Author and article information

                Journal
                orl
                Revista ORL
                Rev. ORL
                Ediciones Universidad de Salamanca (Salamanca, Salamanca, Spain )
                2444-7986
                March 2022
                : 13
                : 1
                : 41-53
                Affiliations
                [1] Santander Cantabria orgnameUniversidad de Cantabria orgdiv1Hospital Universitario Marqués de Valdecilla orgdiv2IDIVAL (Instituto de Investigación Sanitaria Valdecilla) Spain
                Article
                S2444-79862022000100005 S2444-7986(22)01300100005
                10.14201/orl.27445
                c0ac6275-7445-4fb3-b010-bbb5b6f982ba

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

                History
                : 12 December 2021
                : 03 November 2021
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 35, Pages: 13
                Product

                SciELO Spain

                Categories
                Artículo de revisión

                dysphonia,supraglottis,larynx,Immunoglobulin Ig G4-Related disease,disfonía,supraglotis,laringe,Enfermedad relacionada con IgG4

                Comments

                Comment on this article