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Acute exacerbation of fibrotic hypersensitivity pneumonitis: incidence and outcomes

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Abstract
Background: Patients with fibrotic hypersensitivity pneumonitis (HP) show variable clinical courses, and some experience rapid deterioration (RD), including acute exacerbation (AE). However, little is known about AE in fibrotic HP. Here, we retrospectively examined the incidence, risk factors, and outcomes of AE in fibrotic HP.

Methods: The incidence rates of AE were calculated in 101 patients with biopsy-proven HP. AE was defined as the worsening of dyspnoea within 30 days, with new bilateral lung infiltration and no evidence of infection or other causes of dyspnoea.

Results: During follow-up (median: 30 months), 18 (17.8%) patients experienced AE. The 1, 3, and 5 year incidence rates of AE were 6.0, 13.6, and 22.8%, respectively. Lower diffusing capacity of the lung for carbon monoxide (DLCO) and a radiologic usual interstitial pneumonia (UIP)-like pattern were risk factors for AE. In-hospital mortality after AE was 44.4%. Median survival from diagnosis was significantly shorter in patients with AE (26.0 months) than in those with no-AE RD (55.0 months; p = 0.008) or no RD (not reached; p < 0.001). AE remained a significant predictor of all-cause mortality (hazard ratio, 8.641; 95% confidence interval, 3.388-22.040; p < 0.001) after adjustment for age, body mass index, lung function, lymphocyte levels in bronchoalveolar lavage fluid, and the presence of a UIP-like pattern.

Conclusions: AE was not uncommon among patients with fibrotic HP and significantly affected prognosis. A lower DLCO value and radiologic UIP-like pattern at diagnosis were associated with the development AE in patients with fibrotic HP.
Author(s)
송진우채은진Jieun KangJooae ChoeYeon Joo Kim
Issued Date
2021
Type
Article
Keyword
Acute exacerbationComplications and side effectsFibrosisHypersensitivity pneumonitisIncidenceOutcomePatient outcomesStatistics
DOI
10.1186/s12931-021-01748-2
URI
https://oak.ulsan.ac.kr/handle/2021.oak/7045
https://ulsan-primo.hosted.exlibrisgroup.com/primo-explore/fulldisplay?docid=TN_cdi_doaj_primary_oai_doaj_org_article_d8320b83a789467292961917b0b894ed&amp;context=PC&amp;vid=ULSAN&amp;lang=ko_KR&amp;search_scope=default_scope&amp;adaptor=primo_central_multiple_fe&amp;tab=default_tab&amp;query=any,contains,Acute%20exacerbation%20of%20fibrotic%20hypersensitivity%20pneumonitis:%20incidence%20and%20outcomes&amp;offset=0&amp;pcAvailability=true
Publisher
RESPIRATORY RESEARCH
Location
영국
Language
영어
ISSN
1465-993X
Citation Volume
22
Citation Number
1
Citation Start Page
0
Citation End Page
0
Appears in Collections:
Medicine > Medicine
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