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Treating chronic diarrhea: A systematic review on Immunoproliferative Small Intestinal Disease (IPSID)

Immunoproliferative Small Intestinal Disease (IPSID) is a disease characterized by extra-nodal marginal zone B-cell lymphoma with villous atrophy in the small intestine, causing chronic intermittent non-bloody diarrhea. Although originally associated with the Mediterranean region, this disease is pr...

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Autores principales: Evangelista-Leite, Daniele, Affonso Madaloso, Breno, Shouta Yamashita, Bruno, Enrico Aloise, Francesco, Polito Verdasca, Lucas, Lopes de Mello, Murilo, Murata Hayashi, Renan, Zimberg Chehter, Ethel
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8284658/
https://www.ncbi.nlm.nih.gov/pubmed/34270561
http://dx.doi.org/10.1371/journal.pone.0253695
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author Evangelista-Leite, Daniele
Affonso Madaloso, Breno
Shouta Yamashita, Bruno
Enrico Aloise, Francesco
Polito Verdasca, Lucas
Lopes de Mello, Murilo
Murata Hayashi, Renan
Zimberg Chehter, Ethel
author_facet Evangelista-Leite, Daniele
Affonso Madaloso, Breno
Shouta Yamashita, Bruno
Enrico Aloise, Francesco
Polito Verdasca, Lucas
Lopes de Mello, Murilo
Murata Hayashi, Renan
Zimberg Chehter, Ethel
author_sort Evangelista-Leite, Daniele
collection PubMed
description Immunoproliferative Small Intestinal Disease (IPSID) is a disease characterized by extra-nodal marginal zone B-cell lymphoma with villous atrophy in the small intestine, causing chronic intermittent non-bloody diarrhea. Although originally associated with the Mediterranean region, this disease is present in many countries worldwide and may have been underreported due to its complicated diagnosis and scarce scientific literature, especially in regards to treatment. This study aims to review IPSID clinical features, therapeutic options, and treatment outcomes to help physicians identify and treat IPSID. Using PRISMA guidelines, a systematic review of articles was conducted on PubMed database with search terms including IPSID, therapy, treatment, and outcomes. Inclusion and exclusion criteria were used to select 33 English language articles published from the year 2000–2020 that included relevant clinical information about IPSID treatment. Data were extracted independently by at least two authors to reduce the introduction of potential bias. There were 22 case reports, 7 reviews, 1 research article, 1 prospective study, 1 letter to the editor and 1 memoriam in which 76 patients were identified. Epidemiological analysis showed a mean patient age of 32 years old, 2.4:1 mal to female ratio and heterogeneous ethnicities, with 16 Europeans (43.2%) and 12 Asians (32.4%). Chief symptoms included chronic diarrhea (53/76, 69.7%), weight loss (49/76, 64.4%), malabsorption (38/76, 50%), abdominal pain (32/76, 42.1%), and finger clubbing (24/76, 31.6%). Patients stratified into the early disease stage (Galian A) were treated with tetracycline antibiotics, corticosteroids, and non-pharmacological supplements with mostly with complete or partial remission. Late stages (Galian B or C), were treated mostly with anthracycline-based chemotherapy, and occasionally surgery, radiotherapy, or rituximab. This work offers a targeted approach to diagnosing and treating IPSID to aid physicians and serve as a treatment guideline recommendation for future public policies and clinical studies.
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spelling pubmed-82846582021-07-28 Treating chronic diarrhea: A systematic review on Immunoproliferative Small Intestinal Disease (IPSID) Evangelista-Leite, Daniele Affonso Madaloso, Breno Shouta Yamashita, Bruno Enrico Aloise, Francesco Polito Verdasca, Lucas Lopes de Mello, Murilo Murata Hayashi, Renan Zimberg Chehter, Ethel PLoS One Research Article Immunoproliferative Small Intestinal Disease (IPSID) is a disease characterized by extra-nodal marginal zone B-cell lymphoma with villous atrophy in the small intestine, causing chronic intermittent non-bloody diarrhea. Although originally associated with the Mediterranean region, this disease is present in many countries worldwide and may have been underreported due to its complicated diagnosis and scarce scientific literature, especially in regards to treatment. This study aims to review IPSID clinical features, therapeutic options, and treatment outcomes to help physicians identify and treat IPSID. Using PRISMA guidelines, a systematic review of articles was conducted on PubMed database with search terms including IPSID, therapy, treatment, and outcomes. Inclusion and exclusion criteria were used to select 33 English language articles published from the year 2000–2020 that included relevant clinical information about IPSID treatment. Data were extracted independently by at least two authors to reduce the introduction of potential bias. There were 22 case reports, 7 reviews, 1 research article, 1 prospective study, 1 letter to the editor and 1 memoriam in which 76 patients were identified. Epidemiological analysis showed a mean patient age of 32 years old, 2.4:1 mal to female ratio and heterogeneous ethnicities, with 16 Europeans (43.2%) and 12 Asians (32.4%). Chief symptoms included chronic diarrhea (53/76, 69.7%), weight loss (49/76, 64.4%), malabsorption (38/76, 50%), abdominal pain (32/76, 42.1%), and finger clubbing (24/76, 31.6%). Patients stratified into the early disease stage (Galian A) were treated with tetracycline antibiotics, corticosteroids, and non-pharmacological supplements with mostly with complete or partial remission. Late stages (Galian B or C), were treated mostly with anthracycline-based chemotherapy, and occasionally surgery, radiotherapy, or rituximab. This work offers a targeted approach to diagnosing and treating IPSID to aid physicians and serve as a treatment guideline recommendation for future public policies and clinical studies. Public Library of Science 2021-07-16 /pmc/articles/PMC8284658/ /pubmed/34270561 http://dx.doi.org/10.1371/journal.pone.0253695 Text en © 2021 Evangelista-Leite et al https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0/) , which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Research Article
Evangelista-Leite, Daniele
Affonso Madaloso, Breno
Shouta Yamashita, Bruno
Enrico Aloise, Francesco
Polito Verdasca, Lucas
Lopes de Mello, Murilo
Murata Hayashi, Renan
Zimberg Chehter, Ethel
Treating chronic diarrhea: A systematic review on Immunoproliferative Small Intestinal Disease (IPSID)
title Treating chronic diarrhea: A systematic review on Immunoproliferative Small Intestinal Disease (IPSID)
title_full Treating chronic diarrhea: A systematic review on Immunoproliferative Small Intestinal Disease (IPSID)
title_fullStr Treating chronic diarrhea: A systematic review on Immunoproliferative Small Intestinal Disease (IPSID)
title_full_unstemmed Treating chronic diarrhea: A systematic review on Immunoproliferative Small Intestinal Disease (IPSID)
title_short Treating chronic diarrhea: A systematic review on Immunoproliferative Small Intestinal Disease (IPSID)
title_sort treating chronic diarrhea: a systematic review on immunoproliferative small intestinal disease (ipsid)
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8284658/
https://www.ncbi.nlm.nih.gov/pubmed/34270561
http://dx.doi.org/10.1371/journal.pone.0253695
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