Abstract：
:Limited information is available regarding interstitial lung disease (ILD) in Erdheim⁻Chester disease (ECD), a rare multisystemic non-Langerhans cell histiocytosis. Sixty-two biopsy-confirmed ECD patients were divided into those with no ILD (19.5%), minimal ILD (32%), mild ILD (29%), and moderate/severe ILD (19.5%), based on computed tomography (CT) findings. Dyspnea affected at least half of the patients with mild or moderate/severe ILD. Diffusion capacity was significantly reduced in ECD patients with minimal ILD. Disease severity was inversely correlated with pulmonary function measurements; no correlation with BRAF V600E mutation status was seen. Reticulations and ground-glass opacities were the predominant findings on CT images. Automated CT scores were significantly higher in patients with moderate/severe ILD, compared to those in other groups. Immunostaining of lung biopsies was consistent with ECD. Histopathology findings included subpleural and septal fibrosis, with areas of interspersed normal lung, diffuse interstitial fibrosis, histiocytes with foamy cytoplasm embedded in fibrosis, lymphoid aggregates, and focal type II alveolar cell hyperplasia. In conclusion, ILD of varying severity may affect a high proportion of ECD patients. Histopathology features of ILD in ECD can mimic interstitial fibrosis patterns observed in idiopathic ILD.
journal_name
J Clin Medjournal_title
Journal of clinical medicineauthors
Haroutunian SG,O'Brien KJ,Estrada-Veras JI,Yao J,Boyd LC,Mathur K,Gahl WA,Mirmomen SM,Malayeri AA,Kleiner DE,Jaffe ES,Gochuico BRdoi
10.3390/jcm7090243subject
Has Abstractpub_date
2018-08-28 00:00:00issue
9issn
2077-0383pii
jcm7090243journal_volume
7pub_type
杂志文章abstract：:The type of fixation used in primary total knee replacement (TKR) may influence the risk of prosthetic joint infection (PJI). We conducted a systematic review and meta-analysis to assess published evidence linking type of fixation (cemented, uncemented, or hybrid) with the risk of PJI following primary TKR. Randomised...
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更新日期：2019-03-23 00:00:00
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更新日期：2019-02-13 00:00:00
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pub_type： 社论
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更新日期：2018-03-29 00:00:00
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更新日期：2018-09-25 00:00:00
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更新日期：2019-06-10 00:00:00
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