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Interstitial lung disease in primary immunodeficiency: Towards a brighter future

The result's identifiers

  • Result code in IS VaVaI

    <a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00216208%3A11130%2F20%3A10410910" target="_blank" >RIV/00216208:11130/20:10410910 - isvavai.cz</a>

  • Alternative codes found

    RIV/00064203:_____/20:10410910

  • Result on the web

    <a href="https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=VCCslb_P_j" target="_blank" >https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=VCCslb_P_j</a>

  • DOI - Digital Object Identifier

    <a href="http://dx.doi.org/10.1183/13993003.00089-2020" target="_blank" >10.1183/13993003.00089-2020</a>

Alternative languages

  • Result language

    angličtina

  • Original language name

    Interstitial lung disease in primary immunodeficiency: Towards a brighter future

  • Original language description

    Lung disease is a frequent clinical manifestation in people living with primary immunodeficiency diseases, the most prevalent of which are common variable immunodeficiency disorders (CVID). CVID is primarily characterised by antibody deficiency, but recent definitions and diagnostic criteria recognise a much more complex pattern of immunological defects. CVID can be classified into two major clinical phenotypes. One group experiences infection as the only major clinical manifestation, whilst the other present a variety of lymphoproliferative, inflammatory and/or autoimmune complications. The most frequent consequences in the lung of CVID are acute infections, and secondary airway complications of infection, such as bronchiectasis. However, up to 15% of patients with CVID develop an interstitial lung disease. Infections and bronchiectasis are primarily driven by antibody deficiency, but CVID associated interstitial lung disease (CVID-ILD) is best considered part of a systemic immune dysregulatory process such that people with CVID-ILD often have splenomegaly, lymphadenopathy and autoimmune cytopenias. With an EU population of 747 million, we estimate there are up to 30 000 people living with CVID in Europe, and thus 4500 with CVID-ILD. Whilst people with &quot;infection only&quot; CVID can now expect a near normal life expectancy, those with systemic immune dysregulation including CVID-ILD often have a much more complicated course. CVID-ILD increases morbidity and mortality in CVID, although the outcome is now recognised to be more variable than originally reported.

  • Czech name

  • Czech description

Classification

  • Type

    J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database

  • CEP classification

  • OECD FORD branch

    30203 - Respiratory systems

Result continuities

  • Project

  • Continuities

    I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Others

  • Publication year

    2020

  • Confidentiality

    S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Data specific for result type

  • Name of the periodical

    European Respiratory Journal

  • ISSN

    0903-1936

  • e-ISSN

  • Volume of the periodical

    55

  • Issue of the periodical within the volume

    4

  • Country of publishing house

    CH - SWITZERLAND

  • Number of pages

    4

  • Pages from-to

    2000089

  • UT code for WoS article

    000531099900040

  • EID of the result in the Scopus database

    2-s2.0-85082736663