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Two Series of Familial Cases With Unclassified Interstitial Pneumonia With Fibrosis

Identifieur interne : 000487 ( Pmc/Checkpoint ); précédent : 000486; suivant : 000488

Two Series of Familial Cases With Unclassified Interstitial Pneumonia With Fibrosis

Auteurs : Eun Lee [Corée du Sud] ; Ju-Hee Seo [Corée du Sud] ; Hyoung-Young Kim [Corée du Sud] ; Jinho Yu [Corée du Sud] ; Jin Woo Song [Corée du Sud] ; Young Soo Park [Corée du Sud] ; Se-Jin Jang [Corée du Sud] ; Kyung-Hyun Do [Corée du Sud] ; Jiwon Kwon [Corée du Sud] ; Sung-Woo Park [Corée du Sud] ; Jeong-Hwan Park [Corée du Sud] ; Soo-Jong Hong [Corée du Sud]

Source :

RBID : PMC:3378931

Abstract

Several children presenting with mild symptoms of respiratory tract infection were diagnosed with unclassified interstitial pneumonia with fibrosis. Their clinical and radiological findings were similar to those of acute interstitial pneumonia, but there were some differences in the pathological findings. Unclassified interstitial pneumonia with fibrosis is characterized by histological findings of centrilobular distribution of alveolar damage and bronchiolar destruction with bronchiolar obliteration. This report describes two different series of familial cases of unclassified interstitial pneumonia with fibrosis, which developed almost simultaneously in the spring. Some of the individual cases showed rapidly progressive respiratory failure of unknown cause, with comparable clinical courses and similar radiological and pathological features, including lung fibrosis. Each family member was affected almost simultaneously in the spring, different kinds of viruses were detected in two patients, and all members were negative for bacterial infection, environmental and occupational agents, drugs, and radiation. These findings implicate a viral infection and/or processes related to a viral infection, such as an exaggerated or altered immune response, or an unknown inhaled environmental agent in the pathogenesis of unclassified interstitial pneumonia with fibrosis.


Url:
DOI: 10.4168/aair.2012.4.4.240
PubMed: 22754718
PubMed Central: 3378931


Affiliations:


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PMC:3378931

Le document en format XML

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<name sortKey="Song, Jin Woo" sort="Song, Jin Woo" uniqKey="Song J" first="Jin Woo" last="Song">Jin Woo Song</name>
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<name sortKey="Hong, Soo Jong" sort="Hong, Soo Jong" uniqKey="Hong S" first="Soo-Jong" last="Hong">Soo-Jong Hong</name>
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<title level="j">Allergy, Asthma & Immunology Research</title>
<idno type="ISSN">2092-7355</idno>
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<p>Several children presenting with mild symptoms of respiratory tract infection were diagnosed with unclassified interstitial pneumonia with fibrosis. Their clinical and radiological findings were similar to those of acute interstitial pneumonia, but there were some differences in the pathological findings. Unclassified interstitial pneumonia with fibrosis is characterized by histological findings of centrilobular distribution of alveolar damage and bronchiolar destruction with bronchiolar obliteration. This report describes two different series of familial cases of unclassified interstitial pneumonia with fibrosis, which developed almost simultaneously in the spring. Some of the individual cases showed rapidly progressive respiratory failure of unknown cause, with comparable clinical courses and similar radiological and pathological features, including lung fibrosis. Each family member was affected almost simultaneously in the spring, different kinds of viruses were detected in two patients, and all members were negative for bacterial infection, environmental and occupational agents, drugs, and radiation. These findings implicate a viral infection and/or processes related to a viral infection, such as an exaggerated or altered immune response, or an unknown inhaled environmental agent in the pathogenesis of unclassified interstitial pneumonia with fibrosis.</p>
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</TEI>
<pmc article-type="case-report">
<pmc-dir>properties open_access</pmc-dir>
<front>
<journal-meta>
<journal-id journal-id-type="nlm-ta">Allergy Asthma Immunol Res</journal-id>
<journal-id journal-id-type="iso-abbrev">Allergy Asthma Immunol Res</journal-id>
<journal-id journal-id-type="publisher-id">AAIR</journal-id>
<journal-title-group>
<journal-title>Allergy, Asthma & Immunology Research</journal-title>
</journal-title-group>
<issn pub-type="ppub">2092-7355</issn>
<issn pub-type="epub">2092-7363</issn>
<publisher>
<publisher-name>The Korean Academy of Asthma, Allergy and Clinical Immunology; The Korean Academy of Pediatric Allergy and Respiratory Disease</publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id pub-id-type="pmid">22754718</article-id>
<article-id pub-id-type="pmc">3378931</article-id>
<article-id pub-id-type="doi">10.4168/aair.2012.4.4.240</article-id>
<article-categories>
<subj-group subj-group-type="heading">
<subject>Case Report</subject>
</subj-group>
</article-categories>
<title-group>
<article-title>Two Series of Familial Cases With Unclassified Interstitial Pneumonia With Fibrosis</article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname>Lee</surname>
<given-names>Eun</given-names>
</name>
<xref ref-type="aff" rid="A1">1</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Seo</surname>
<given-names>Ju-Hee</given-names>
</name>
<xref ref-type="aff" rid="A1">1</xref>
<xref ref-type="aff" rid="A2">2</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Kim</surname>
<given-names>Hyoung-Young</given-names>
</name>
<xref ref-type="aff" rid="A1">1</xref>
<xref ref-type="aff" rid="A2">2</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Yu</surname>
<given-names>Jinho</given-names>
</name>
<xref ref-type="aff" rid="A1">1</xref>
<xref ref-type="aff" rid="A2">2</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Song</surname>
<given-names>Jin Woo</given-names>
</name>
<xref ref-type="aff" rid="A3">3</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Park</surname>
<given-names>Young Soo</given-names>
</name>
<xref ref-type="aff" rid="A4">4</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Jang</surname>
<given-names>Se-Jin</given-names>
</name>
<xref ref-type="aff" rid="A4">4</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Do</surname>
<given-names>Kyung-Hyun</given-names>
</name>
<xref ref-type="aff" rid="A5">5</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Kwon</surname>
<given-names>Jiwon</given-names>
</name>
<xref ref-type="aff" rid="A6">6</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Park</surname>
<given-names>Sung-woo</given-names>
</name>
<xref ref-type="aff" rid="A7">7</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Park</surname>
<given-names>Jeong-hwan</given-names>
</name>
<xref ref-type="aff" rid="A7">7</xref>
</contrib>
<contrib contrib-type="author" corresp="yes">
<name>
<surname>Hong</surname>
<given-names>Soo-Jong</given-names>
</name>
<xref ref-type="aff" rid="A1">1</xref>
<xref ref-type="aff" rid="A2">2</xref>
</contrib>
</contrib-group>
<aff id="A1">
<label>1</label>
Department of Pediatrics, Asan Medical Center Children's Hospital, Seoul, Korea.</aff>
<aff id="A2">
<label>2</label>
Childhood Asthma Atopy Center, Asan Medical Center Children's Hospital, Seoul, Korea.</aff>
<aff id="A3">
<label>3</label>
Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.</aff>
<aff id="A4">
<label>4</label>
Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.</aff>
<aff id="A5">
<label>5</label>
Department of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.</aff>
<aff id="A6">
<label>6</label>
Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, Korea.</aff>
<aff id="A7">
<label>7</label>
Department of Internal Medicine, Soonchunhyang University Bucheon Hospital, Bucheon, Korea.</aff>
<author-notes>
<corresp>Correspondence to: Soo-Jong Hong, MD, PhD, Department of Pediatrics, Childhood Asthma Atopy Center, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, 43-gil Olympic-ro, Songpa-gu, Seoul 138-736, Korea. Tel: +82-2-3010-3379; Fax: +82-2-473-3725;
<email>sjhong@amc.seoul.kr</email>
</corresp>
</author-notes>
<pub-date pub-type="ppub">
<month>7</month>
<year>2012</year>
</pub-date>
<pub-date pub-type="epub">
<day>09</day>
<month>3</month>
<year>2012</year>
</pub-date>
<volume>4</volume>
<issue>4</issue>
<fpage>240</fpage>
<lpage>244</lpage>
<history>
<date date-type="received">
<day>03</day>
<month>6</month>
<year>2011</year>
</date>
<date date-type="rev-recd">
<day>28</day>
<month>10</month>
<year>2011</year>
</date>
<date date-type="accepted">
<day>02</day>
<month>12</month>
<year>2011</year>
</date>
</history>
<permissions>
<copyright-statement>Copyright © 2012 The Korean Academy of Asthma, Allergy and Clinical Immunology • The Korean Academy of Pediatric Allergy and Respiratory Disease</copyright-statement>
<copyright-year>2012</copyright-year>
<license license-type="open-access" xlink:href="http://creativecommons.org/licenses/by-nc/3.0/">
<license-p>This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (
<ext-link ext-link-type="uri" xlink:href="http://creativecommons.org/licenses/by-nc/3.0/">http://creativecommons.org/licenses/by-nc/3.0/</ext-link>
) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.</license-p>
</license>
</permissions>
<abstract>
<p>Several children presenting with mild symptoms of respiratory tract infection were diagnosed with unclassified interstitial pneumonia with fibrosis. Their clinical and radiological findings were similar to those of acute interstitial pneumonia, but there were some differences in the pathological findings. Unclassified interstitial pneumonia with fibrosis is characterized by histological findings of centrilobular distribution of alveolar damage and bronchiolar destruction with bronchiolar obliteration. This report describes two different series of familial cases of unclassified interstitial pneumonia with fibrosis, which developed almost simultaneously in the spring. Some of the individual cases showed rapidly progressive respiratory failure of unknown cause, with comparable clinical courses and similar radiological and pathological features, including lung fibrosis. Each family member was affected almost simultaneously in the spring, different kinds of viruses were detected in two patients, and all members were negative for bacterial infection, environmental and occupational agents, drugs, and radiation. These findings implicate a viral infection and/or processes related to a viral infection, such as an exaggerated or altered immune response, or an unknown inhaled environmental agent in the pathogenesis of unclassified interstitial pneumonia with fibrosis.</p>
</abstract>
<kwd-group>
<kwd>Interstitial pneumonia</kwd>
<kwd>family</kwd>
<kwd>fibrosis</kwd>
<kwd>respiratory tract infections</kwd>
<kwd>environment</kwd>
</kwd-group>
</article-meta>
</front>
<floats-group>
<fig id="F1" position="float">
<label>Fig. 1</label>
<caption>
<p>Chest radiography and biopsy specimens for Case 1. Chest radiography (A) on day 2 of the exacerbation of cough and dyspnea. Chest computed tomography (CT) scan (B) on admission revealed the presence of ground-glass attenuation with diffuse centrinodular opacity in both lungs. Pre-discharge chest radiography (C) and chest CT (D) showed a reduction in the extent of ground-glass opacities in both lungs. Biopsy specimens (E) obtained on day 14 of the exacerbation of symptoms. Alveolar pneumocyte hyperplasia (arrow) and interstitial fibroblastic proliferation are features consistent with the organizing phase of diffuse alveolar damage. Bronchioles are destructed and obliterated by fibroblastic proliferation (asterisk). (Hematoxylin and eosin staining, ×200 magnification)</p>
</caption>
<graphic xlink:href="aair-4-240-g001"></graphic>
</fig>
<fig id="F2" position="float">
<label>Fig. 2</label>
<caption>
<p>Findings for Case 3. Chest radiography (A) and chest computed tomography scan (B) revealed the presence of an extensive pneumomediastinum, a pneumothorax of the right lung, and diffuse ground-glass opacities and consolidation in both lungs. Follow-up chest radiography on day 72 of admission revealed an increased level of bilateral infiltrates (C). Histological section (D) obtained from a postmortem biopsy showed extensive interstitial fibrosis with hyperplastic type II pneumocytes and hyaline membrane formation, suggesting the fibrotic phase of diffuse alveolar damage. Bronchiolar destruction and mild bronchiolar obliteration are present. (Hematoxylin and eosin staining, ×400 magnification)</p>
</caption>
<graphic xlink:href="aair-4-240-g002"></graphic>
</fig>
<table-wrap id="T1" position="float">
<label>Table</label>
<caption>
<p>Summary of two familial case series</p>
</caption>
<graphic xlink:href="aair-4-240-i001"></graphic>
<table-wrap-foot>
<fn>
<p>DAD, diffuse alveolar damage; CPM, cyclophosphamide; IVIG, intravenous immune globulin; DOE, dyspnea on exertion; RSV, respiratory syncytial virus; Adm., admission.</p>
</fn>
</table-wrap-foot>
</table-wrap>
</floats-group>
</pmc>
<affiliations>
<list>
<country>
<li>Corée du Sud</li>
</country>
<region>
<li>Région capitale de Séoul</li>
</region>
<settlement>
<li>Séoul</li>
</settlement>
</list>
<tree>
<country name="Corée du Sud">
<region name="Région capitale de Séoul">
<name sortKey="Lee, Eun" sort="Lee, Eun" uniqKey="Lee E" first="Eun" last="Lee">Eun Lee</name>
</region>
<name sortKey="Do, Kyung Hyun" sort="Do, Kyung Hyun" uniqKey="Do K" first="Kyung-Hyun" last="Do">Kyung-Hyun Do</name>
<name sortKey="Hong, Soo Jong" sort="Hong, Soo Jong" uniqKey="Hong S" first="Soo-Jong" last="Hong">Soo-Jong Hong</name>
<name sortKey="Hong, Soo Jong" sort="Hong, Soo Jong" uniqKey="Hong S" first="Soo-Jong" last="Hong">Soo-Jong Hong</name>
<name sortKey="Jang, Se Jin" sort="Jang, Se Jin" uniqKey="Jang S" first="Se-Jin" last="Jang">Se-Jin Jang</name>
<name sortKey="Kim, Hyoung Young" sort="Kim, Hyoung Young" uniqKey="Kim H" first="Hyoung-Young" last="Kim">Hyoung-Young Kim</name>
<name sortKey="Kim, Hyoung Young" sort="Kim, Hyoung Young" uniqKey="Kim H" first="Hyoung-Young" last="Kim">Hyoung-Young Kim</name>
<name sortKey="Kwon, Jiwon" sort="Kwon, Jiwon" uniqKey="Kwon J" first="Jiwon" last="Kwon">Jiwon Kwon</name>
<name sortKey="Park, Jeong Hwan" sort="Park, Jeong Hwan" uniqKey="Park J" first="Jeong-Hwan" last="Park">Jeong-Hwan Park</name>
<name sortKey="Park, Sung Woo" sort="Park, Sung Woo" uniqKey="Park S" first="Sung-Woo" last="Park">Sung-Woo Park</name>
<name sortKey="Park, Young Soo" sort="Park, Young Soo" uniqKey="Park Y" first="Young Soo" last="Park">Young Soo Park</name>
<name sortKey="Seo, Ju Hee" sort="Seo, Ju Hee" uniqKey="Seo J" first="Ju-Hee" last="Seo">Ju-Hee Seo</name>
<name sortKey="Seo, Ju Hee" sort="Seo, Ju Hee" uniqKey="Seo J" first="Ju-Hee" last="Seo">Ju-Hee Seo</name>
<name sortKey="Song, Jin Woo" sort="Song, Jin Woo" uniqKey="Song J" first="Jin Woo" last="Song">Jin Woo Song</name>
<name sortKey="Yu, Jinho" sort="Yu, Jinho" uniqKey="Yu J" first="Jinho" last="Yu">Jinho Yu</name>
<name sortKey="Yu, Jinho" sort="Yu, Jinho" uniqKey="Yu J" first="Jinho" last="Yu">Jinho Yu</name>
</country>
</tree>
</affiliations>
</record>

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